Hematology

INTRODUCTION

We present an unusual case of autoimmune hemolytic anemia (AIHA) associated with Kikuchi-Fujimoto Disease (KFD) and systemic lupus erythematosus (SLE) that resolved with steroid therapy.

CASE PRESENTATION

A 25-year-old female with no medical history presented with 6 weeks of high fevers, syncope, and 10-lb weight loss. Exam revealed generalized lymphadenopathy (LAD) and tiny malar papules. Labs showed IgG and IgM Coombs-positivity, hemoglobin of 5 g/dL, hyperbilirubinemia, low haptoglobin, LDH >2000 IU/L, thrombocytopenia, and leukopenia. Cryoglobulins were absent. Hemophagocytic lymphohistiocytosis (HLH) markers showed ferritin of 18,000 ng/mL, moderately elevated soluble IL-2 receptor, negative CD107, minimally elevated CXCL9, borderline transaminitis, and high-normal triglycerides. ANA was 1:1280, speckled, with high anti-RNP, high anti-Smith, and negative anti-dsDNA antibodies. CT confirmed LAD without organomegaly. A 4cm excised node reviewed at 2 institutions showed necrotizing lymphadenitis without granulomas, consistent with KFD. Flow cytometry and gene rearrangement assay showed no monoclonality. Bone marrow biopsy demonstrated erythroid hyperplasia, normal flow cytometry, and no hemophagocytosis. Infectious workup was unremarkable. Treatment was initiated with 50mg prednisone daily, weaned off over 5 months. 2 months post-initiation, the fevers resolved, hemoglobin increased, and LDH normalized. 3 months later, rheumatology service diagnosed SLE based on 2019 ACR/EULAR Criteria and initiated hydroxychloroquine. 9 months later, patient remains without recurrence.

DISCUSSION

KFD presents subacutely with LAD, fever, weight loss, and varying skin findings, often self-resolving. Diagnosis requires lymph node biopsy. Etiology is unclear, with infectious, neoplastic, and autoimmune mechanisms implicated. Studies suggest up to 15% of patients have SLE.

CONCLUSIONS

This case is a rare combination of AIHA, KFD, and SLE successfully treated with steroids and, later, hydroxychloroquine. It calls for vigilance for KFD in patients with LAD and AIHA. A successful treatment strategy could include highdose steroids. The presentation may mimic lymphoma and HLH, which must be ruled out with careful pathologic and lab evaluation. To our knowledge, this is the 3rd reported case of KFD with AIHA, and 2nd case of concomitant SLE, KFD, and AIHA. The only similar patient was treated with methylprednisolone and cyclophosphamide and did not have longer-term follow-up.

INTRODUCTION

We present an unusual case of autoimmune hemolytic anemia (AIHA) associated with Kikuchi-Fujimoto Disease (KFD) and systemic lupus erythematosus (SLE) that resolved with steroid therapy.

CASE PRESENTATION

A 25-year-old female with no medical history presented with 6 weeks of high fevers, syncope, and 10-lb weight loss. Exam revealed generalized lymphadenopathy (LAD) and tiny malar papules. Labs showed IgG and IgM Coombs-positivity, hemoglobin of 5 g/dL, hyperbilirubinemia, low haptoglobin, LDH >2000 IU/L, thrombocytopenia, and leukopenia. Cryoglobulins were absent. Hemophagocytic lymphohistiocytosis (HLH) markers showed ferritin of 18,000 ng/mL, moderately elevated soluble IL-2 receptor, negative CD107, minimally elevated CXCL9, borderline transaminitis, and high-normal triglycerides. ANA was 1:1280, speckled, with high anti-RNP, high anti-Smith, and negative anti-dsDNA antibodies. CT confirmed LAD without organomegaly. A 4cm excised node reviewed at 2 institutions showed necrotizing lymphadenitis without granulomas, consistent with KFD. Flow cytometry and gene rearrangement assay showed no monoclonality. Bone marrow biopsy demonstrated erythroid hyperplasia, normal flow cytometry, and no hemophagocytosis. Infectious workup was unremarkable. Treatment was initiated with 50mg prednisone daily, weaned off over 5 months. 2 months post-initiation, the fevers resolved, hemoglobin increased, and LDH normalized. 3 months later, rheumatology service diagnosed SLE based on 2019 ACR/EULAR Criteria and initiated hydroxychloroquine. 9 months later, patient remains without recurrence.

DISCUSSION

KFD presents subacutely with LAD, fever, weight loss, and varying skin findings, often self-resolving. Diagnosis requires lymph node biopsy. Etiology is unclear, with infectious, neoplastic, and autoimmune mechanisms implicated. Studies suggest up to 15% of patients have SLE.

CONCLUSIONS

This case is a rare combination of AIHA, KFD, and SLE successfully treated with steroids and, later, hydroxychloroquine. It calls for vigilance for KFD in patients with LAD and AIHA. A successful treatment strategy could include highdose steroids. The presentation may mimic lymphoma and HLH, which must be ruled out with careful pathologic and lab evaluation. To our knowledge, this is the 3rd reported case of KFD with AIHA, and 2nd case of concomitant SLE, KFD, and AIHA. The only similar patient was treated with methylprednisolone and cyclophosphamide and did not have longer-term follow-up.

INTRODUCTION

We present an unusual case of autoimmune hemolytic anemia (AIHA) associated with Kikuchi-Fujimoto Disease (KFD) and systemic lupus erythematosus (SLE) that resolved with steroid therapy.

CASE PRESENTATION

A 25-year-old female with no medical history presented with 6 weeks of high fevers, syncope, and 10-lb weight loss. Exam revealed generalized lymphadenopathy (LAD) and tiny malar papules. Labs showed IgG and IgM Coombs-positivity, hemoglobin of 5 g/dL, hyperbilirubinemia, low haptoglobin, LDH >2000 IU/L, thrombocytopenia, and leukopenia. Cryoglobulins were absent. Hemophagocytic lymphohistiocytosis (HLH) markers showed ferritin of 18,000 ng/mL, moderately elevated soluble IL-2 receptor, negative CD107, minimally elevated CXCL9, borderline transaminitis, and high-normal triglycerides. ANA was 1:1280, speckled, with high anti-RNP, high anti-Smith, and negative anti-dsDNA antibodies. CT confirmed LAD without organomegaly. A 4cm excised node reviewed at 2 institutions showed necrotizing lymphadenitis without granulomas, consistent with KFD. Flow cytometry and gene rearrangement assay showed no monoclonality. Bone marrow biopsy demonstrated erythroid hyperplasia, normal flow cytometry, and no hemophagocytosis. Infectious workup was unremarkable. Treatment was initiated with 50mg prednisone daily, weaned off over 5 months. 2 months post-initiation, the fevers resolved, hemoglobin increased, and LDH normalized. 3 months later, rheumatology service diagnosed SLE based on 2019 ACR/EULAR Criteria and initiated hydroxychloroquine. 9 months later, patient remains without recurrence.

DISCUSSION

KFD presents subacutely with LAD, fever, weight loss, and varying skin findings, often self-resolving. Diagnosis requires lymph node biopsy. Etiology is unclear, with infectious, neoplastic, and autoimmune mechanisms implicated. Studies suggest up to 15% of patients have SLE.

CONCLUSIONS

This case is a rare combination of AIHA, KFD, and SLE successfully treated with steroids and, later, hydroxychloroquine. It calls for vigilance for KFD in patients with LAD and AIHA. A successful treatment strategy could include highdose steroids. The presentation may mimic lymphoma and HLH, which must be ruled out with careful pathologic and lab evaluation. To our knowledge, this is the 3rd reported case of KFD with AIHA, and 2nd case of concomitant SLE, KFD, and AIHA. The only similar patient was treated with methylprednisolone and cyclophosphamide and did not have longer-term follow-up.

BACKGROUND

The safety and efficacy of biosimilars are carefully reviewed by the Food and Drug Administration (FDA) to ensure the biosimilar meets the high standards for approval. However, safety concerns from infusion nursing staff prompted a review of rituximab-PVVR and rituximab for any new trends in National VA, primary literature, and facility adverse events.

METHODS

Utilizing the VA ADERS (Veteran’s Affairs Adverse Drug Event Reporting System), data was analyzed from 01/01/21 thru 04/01/23. No clear trends were identified to support an increased reaction rate for Rituximab-PVVR or Rituximab. A total of 104 Rituximab product (both parent and biosimilar products) adverse reactions were reported nationally. Of those reported, about half 56 ADEs (54%) were specifically to Rituximab-PVVR.

RESULTS

Reviewing our facility specific VA ADERS data, Birmingham VA reported 7 ADEs. Similarly other sites reported a range of 0 to 13 Rituximab product ADEs. The total number of unique patients to receive a rituximab product in the Birmingham VA since 2021 is 106, resulting in an overall incidence rate of 6.6%.

DISCUSSION

Based on the recent publication, Safety of switching between rituximab biosimilars in onco-hematology “adverse events were similar, in terms of seriousness and frequency, to those described in the literature, providing further support to the clinical safety of biosimilars.” This prospective clinical trial published in 2021, reported grade 1 rituximab related infusion events in 7.1% of patients (n=83) which correlates closely to the reported incidence at our facility referenced above (6.6%). Our current pre-medications include acetaminophen, an antihistamine, and steroid 30 minutes prior to infusion. Although our interdisciplinary team deemed this appropriate, to improve and minimize infusion reaction symptoms, the following interventions were instituted including changing ORAL Diphenhydramine to intravenous Diphenhydramine 25mg IV and providing education to infusion nursing staff on the safety and efficacy of the rituximab and biosimilar products.

CONCLUSIONS

Following the intervention (04/07/23), 36 total unique patients received rituximab products with zero incidents reported. Although the results are limited, the data may suggest IV diphenhydramine reduces the severity of ADEs which may alter reporting or show a potential “nocebo” effect could be a factor with any rituximab infusion needing further evaluation.

BACKGROUND

The safety and efficacy of biosimilars are carefully reviewed by the Food and Drug Administration (FDA) to ensure the biosimilar meets the high standards for approval. However, safety concerns from infusion nursing staff prompted a review of rituximab-PVVR and rituximab for any new trends in National VA, primary literature, and facility adverse events.

METHODS

Utilizing the VA ADERS (Veteran’s Affairs Adverse Drug Event Reporting System), data was analyzed from 01/01/21 thru 04/01/23. No clear trends were identified to support an increased reaction rate for Rituximab-PVVR or Rituximab. A total of 104 Rituximab product (both parent and biosimilar products) adverse reactions were reported nationally. Of those reported, about half 56 ADEs (54%) were specifically to Rituximab-PVVR.

RESULTS

Reviewing our facility specific VA ADERS data, Birmingham VA reported 7 ADEs. Similarly other sites reported a range of 0 to 13 Rituximab product ADEs. The total number of unique patients to receive a rituximab product in the Birmingham VA since 2021 is 106, resulting in an overall incidence rate of 6.6%.

DISCUSSION

Based on the recent publication, Safety of switching between rituximab biosimilars in onco-hematology “adverse events were similar, in terms of seriousness and frequency, to those described in the literature, providing further support to the clinical safety of biosimilars.” This prospective clinical trial published in 2021, reported grade 1 rituximab related infusion events in 7.1% of patients (n=83) which correlates closely to the reported incidence at our facility referenced above (6.6%). Our current pre-medications include acetaminophen, an antihistamine, and steroid 30 minutes prior to infusion. Although our interdisciplinary team deemed this appropriate, to improve and minimize infusion reaction symptoms, the following interventions were instituted including changing ORAL Diphenhydramine to intravenous Diphenhydramine 25mg IV and providing education to infusion nursing staff on the safety and efficacy of the rituximab and biosimilar products.

CONCLUSIONS

Following the intervention (04/07/23), 36 total unique patients received rituximab products with zero incidents reported. Although the results are limited, the data may suggest IV diphenhydramine reduces the severity of ADEs which may alter reporting or show a potential “nocebo” effect could be a factor with any rituximab infusion needing further evaluation.

BACKGROUND

The safety and efficacy of biosimilars are carefully reviewed by the Food and Drug Administration (FDA) to ensure the biosimilar meets the high standards for approval. However, safety concerns from infusion nursing staff prompted a review of rituximab-PVVR and rituximab for any new trends in National VA, primary literature, and facility adverse events.

METHODS

Utilizing the VA ADERS (Veteran’s Affairs Adverse Drug Event Reporting System), data was analyzed from 01/01/21 thru 04/01/23. No clear trends were identified to support an increased reaction rate for Rituximab-PVVR or Rituximab. A total of 104 Rituximab product (both parent and biosimilar products) adverse reactions were reported nationally. Of those reported, about half 56 ADEs (54%) were specifically to Rituximab-PVVR.

RESULTS

Reviewing our facility specific VA ADERS data, Birmingham VA reported 7 ADEs. Similarly other sites reported a range of 0 to 13 Rituximab product ADEs. The total number of unique patients to receive a rituximab product in the Birmingham VA since 2021 is 106, resulting in an overall incidence rate of 6.6%.

DISCUSSION

Based on the recent publication, Safety of switching between rituximab biosimilars in onco-hematology “adverse events were similar, in terms of seriousness and frequency, to those described in the literature, providing further support to the clinical safety of biosimilars.” This prospective clinical trial published in 2021, reported grade 1 rituximab related infusion events in 7.1% of patients (n=83) which correlates closely to the reported incidence at our facility referenced above (6.6%). Our current pre-medications include acetaminophen, an antihistamine, and steroid 30 minutes prior to infusion. Although our interdisciplinary team deemed this appropriate, to improve and minimize infusion reaction symptoms, the following interventions were instituted including changing ORAL Diphenhydramine to intravenous Diphenhydramine 25mg IV and providing education to infusion nursing staff on the safety and efficacy of the rituximab and biosimilar products.

CONCLUSIONS

Following the intervention (04/07/23), 36 total unique patients received rituximab products with zero incidents reported. Although the results are limited, the data may suggest IV diphenhydramine reduces the severity of ADEs which may alter reporting or show a potential “nocebo” effect could be a factor with any rituximab infusion needing further evaluation.

BACKGROUND

The Pharmacogenomic Testing for Veterans (PHASER) program provides preemptive pharmacogenomic testing for Veterans nationally. Program implementation at the Madison VA began in the hematology and oncology (hem/onc) clinics. In these clinics, PHASER test results are reviewed by the hem/onc clinical pharmacist practitioner (CPP) who provides recommendations regarding therapy via an electronic health record note. The purpose of this retrospective chart review was to assess the impact of the CPP on medication management informed by pharmacogenomics.

METHODS

A retrospective chart review was completed for all Veterans enrolled in hem/onc services and offered PHASER testing between April 1, 2022 and November 1, 2022. The number and type of interventions recommended by the hem/onc CPP, acceptance of recommended interventions, and hem/onc CPP time spent were collected for all patients who accepted and completed PHASER testing. Interventions were categorized and descriptive statistics were used to summarize data.

RESULTS

Of the 98 patients reviewed by the CPP, 75 (77%) were prescribed a medication with potential pharmacogenomic implications. At least one actionable recommendation for medication therapy adjustment was identified for 40 (53%) of those patients based on their pharmacogenomic test results. The CPP spent an average of 12 minutes per patient review (range 5 to 30 minutes) and 100% of CPP recommendations were accepted.

CONCLUSIONS

The CPP efficiently reviewed pharmacogenomic test results and made meaningful recommendations for medication therapy adjustments. CPP recommendations were highly accepted in the hem/onc setting.

BACKGROUND

The Pharmacogenomic Testing for Veterans (PHASER) program provides preemptive pharmacogenomic testing for Veterans nationally. Program implementation at the Madison VA began in the hematology and oncology (hem/onc) clinics. In these clinics, PHASER test results are reviewed by the hem/onc clinical pharmacist practitioner (CPP) who provides recommendations regarding therapy via an electronic health record note. The purpose of this retrospective chart review was to assess the impact of the CPP on medication management informed by pharmacogenomics.

METHODS

A retrospective chart review was completed for all Veterans enrolled in hem/onc services and offered PHASER testing between April 1, 2022 and November 1, 2022. The number and type of interventions recommended by the hem/onc CPP, acceptance of recommended interventions, and hem/onc CPP time spent were collected for all patients who accepted and completed PHASER testing. Interventions were categorized and descriptive statistics were used to summarize data.

RESULTS

Of the 98 patients reviewed by the CPP, 75 (77%) were prescribed a medication with potential pharmacogenomic implications. At least one actionable recommendation for medication therapy adjustment was identified for 40 (53%) of those patients based on their pharmacogenomic test results. The CPP spent an average of 12 minutes per patient review (range 5 to 30 minutes) and 100% of CPP recommendations were accepted.

CONCLUSIONS

The CPP efficiently reviewed pharmacogenomic test results and made meaningful recommendations for medication therapy adjustments. CPP recommendations were highly accepted in the hem/onc setting.

BACKGROUND

The Pharmacogenomic Testing for Veterans (PHASER) program provides preemptive pharmacogenomic testing for Veterans nationally. Program implementation at the Madison VA began in the hematology and oncology (hem/onc) clinics. In these clinics, PHASER test results are reviewed by the hem/onc clinical pharmacist practitioner (CPP) who provides recommendations regarding therapy via an electronic health record note. The purpose of this retrospective chart review was to assess the impact of the CPP on medication management informed by pharmacogenomics.

METHODS

A retrospective chart review was completed for all Veterans enrolled in hem/onc services and offered PHASER testing between April 1, 2022 and November 1, 2022. The number and type of interventions recommended by the hem/onc CPP, acceptance of recommended interventions, and hem/onc CPP time spent were collected for all patients who accepted and completed PHASER testing. Interventions were categorized and descriptive statistics were used to summarize data.

RESULTS

Of the 98 patients reviewed by the CPP, 75 (77%) were prescribed a medication with potential pharmacogenomic implications. At least one actionable recommendation for medication therapy adjustment was identified for 40 (53%) of those patients based on their pharmacogenomic test results. The CPP spent an average of 12 minutes per patient review (range 5 to 30 minutes) and 100% of CPP recommendations were accepted.

CONCLUSIONS

The CPP efficiently reviewed pharmacogenomic test results and made meaningful recommendations for medication therapy adjustments. CPP recommendations were highly accepted in the hem/onc setting.

BACKGROUND

While the MISSION Act for community care has increased Veteran access to specialty services, this has caused considerable fragmentation of care and financial cost to U.S. taxpayers. The VA Salt Lake City Health Care System (VA SLCHCS) referral area spans 125,000 square miles, one of the largest geographic regions in the VA health care system. Numerous VA Community- Based Outpatient Clinics (CBOCs) have been established in central and southern Utah, eastern Nevada, and southern Idaho; however, these clinics do not currently provide specialty services.

DISCUSSION

In conjunction with the National Oncology Program’s Close to Me project team, we conducted a cost analysis to determine financial feasibility of providing low-risk oncology parenteral therapies at rural CBOCs. Based on FY22 DO Paid Claim PowerBI and Pyramid Analytics Reports, VA SLCHCS paid claims for Community Care Hematology/Oncology community services in excess of $5.7 million for 380 unique Veterans (approximately $15,060 per unique Veteran). Comparatively, Veterans received high quality oncology care through VA SLCHCS with an estimated average cost of care of $5,424 per unique Veteran. Cost of parenteral therapies was estimated via review of Community Care Paid Claims Reports for individual drug claim costs (based on Jcode), VA drug pricing data from the VA National Acquisition Center Catalog, and drug unit claims data. The unit price of VA-care and community care costs were calculated and drug cost at the VA versus non- VA was compared. By retaining or re-establishing Hematology/Oncology Veteran care within VA, we estimate cost savings of approximately $9,636 per unique Veteran.

CONCLUSIONS

By re-establishing oncology care within VA SLCHCS the facility could net a substantial cost savings while simultaneously making Veterans lives easier, reduce need for transportation to/from the main SLC VA site, decrease costs due to VA pricing contracts, lessen Veteran out-of-pocket costs, improve care coordination through use of one electronic medical record, and maintain Veteran care within VA SLCHCS. Additionally, VA SLCHCS oncology will help lead the effort to launch a system within the CBOC’s to deliver high-cost parental therapies that could benefit other medical specialties such as gastroenterology, dermatology, and rheumatology.

BACKGROUND

While the MISSION Act for community care has increased Veteran access to specialty services, this has caused considerable fragmentation of care and financial cost to U.S. taxpayers. The VA Salt Lake City Health Care System (VA SLCHCS) referral area spans 125,000 square miles, one of the largest geographic regions in the VA health care system. Numerous VA Community- Based Outpatient Clinics (CBOCs) have been established in central and southern Utah, eastern Nevada, and southern Idaho; however, these clinics do not currently provide specialty services.

DISCUSSION

In conjunction with the National Oncology Program’s Close to Me project team, we conducted a cost analysis to determine financial feasibility of providing low-risk oncology parenteral therapies at rural CBOCs. Based on FY22 DO Paid Claim PowerBI and Pyramid Analytics Reports, VA SLCHCS paid claims for Community Care Hematology/Oncology community services in excess of $5.7 million for 380 unique Veterans (approximately $15,060 per unique Veteran). Comparatively, Veterans received high quality oncology care through VA SLCHCS with an estimated average cost of care of $5,424 per unique Veteran. Cost of parenteral therapies was estimated via review of Community Care Paid Claims Reports for individual drug claim costs (based on Jcode), VA drug pricing data from the VA National Acquisition Center Catalog, and drug unit claims data. The unit price of VA-care and community care costs were calculated and drug cost at the VA versus non- VA was compared. By retaining or re-establishing Hematology/Oncology Veteran care within VA, we estimate cost savings of approximately $9,636 per unique Veteran.

CONCLUSIONS

By re-establishing oncology care within VA SLCHCS the facility could net a substantial cost savings while simultaneously making Veterans lives easier, reduce need for transportation to/from the main SLC VA site, decrease costs due to VA pricing contracts, lessen Veteran out-of-pocket costs, improve care coordination through use of one electronic medical record, and maintain Veteran care within VA SLCHCS. Additionally, VA SLCHCS oncology will help lead the effort to launch a system within the CBOC’s to deliver high-cost parental therapies that could benefit other medical specialties such as gastroenterology, dermatology, and rheumatology.

BACKGROUND

While the MISSION Act for community care has increased Veteran access to specialty services, this has caused considerable fragmentation of care and financial cost to U.S. taxpayers. The VA Salt Lake City Health Care System (VA SLCHCS) referral area spans 125,000 square miles, one of the largest geographic regions in the VA health care system. Numerous VA Community- Based Outpatient Clinics (CBOCs) have been established in central and southern Utah, eastern Nevada, and southern Idaho; however, these clinics do not currently provide specialty services.

DISCUSSION

In conjunction with the National Oncology Program’s Close to Me project team, we conducted a cost analysis to determine financial feasibility of providing low-risk oncology parenteral therapies at rural CBOCs. Based on FY22 DO Paid Claim PowerBI and Pyramid Analytics Reports, VA SLCHCS paid claims for Community Care Hematology/Oncology community services in excess of $5.7 million for 380 unique Veterans (approximately $15,060 per unique Veteran). Comparatively, Veterans received high quality oncology care through VA SLCHCS with an estimated average cost of care of $5,424 per unique Veteran. Cost of parenteral therapies was estimated via review of Community Care Paid Claims Reports for individual drug claim costs (based on Jcode), VA drug pricing data from the VA National Acquisition Center Catalog, and drug unit claims data. The unit price of VA-care and community care costs were calculated and drug cost at the VA versus non- VA was compared. By retaining or re-establishing Hematology/Oncology Veteran care within VA, we estimate cost savings of approximately $9,636 per unique Veteran.

CONCLUSIONS

By re-establishing oncology care within VA SLCHCS the facility could net a substantial cost savings while simultaneously making Veterans lives easier, reduce need for transportation to/from the main SLC VA site, decrease costs due to VA pricing contracts, lessen Veteran out-of-pocket costs, improve care coordination through use of one electronic medical record, and maintain Veteran care within VA SLCHCS. Additionally, VA SLCHCS oncology will help lead the effort to launch a system within the CBOC’s to deliver high-cost parental therapies that could benefit other medical specialties such as gastroenterology, dermatology, and rheumatology.

PURPOSE

To evaluate if low molecular weight iron dextran (LMWID) is a safe and effective alternative to iron sucrose for intravenous iron administration.

BACKGROUND

In recent years, intravenous iron administration has increased due to clinical indications and rapid iron repletion. Early IV iron formulations had safety concerns that precluded widespread use. High molecular weight iron dextran was removed from the US market in 2009 due to safety concerns. Since then, several new IV formulations including LMWID and iron sucrose have been approved with a favorable benefit risk profile. While recent evidence and guidelines indicate that LMWID and other iron formulations have comparable safety profiles, no head-to-head comparisons exist. Both iron sucrose and LMWID are used for the treatment of IDA in Veterans Affairs hospitals. Iron sucrose is given 200 mg weekly for 5 weeks, while LMWID is given as a single 1-gram dose over 3 hours. We conducted a retrospective crosssectional analysis to compare the safety and efficacy of IV LMWID to IV iron sucrose.

METHODS

We identified 129 patients (LMWID: n=29, iron sucrose: n=100) who received intravenous iron from 01/01/2022 to 03/03/2023. To match the sample size, we selected every 3rd patient from the iron sucrose group (n=33). We captured data on infusion-related reactions, history of asthma/inflammatory bowel disease/> 2 drug allergies, overall and ≥ 2 g/dL hemoglobin increase, and treatment cost. Descriptive statistics were used to describe the safety and efficacy parameters. An unpaired t-test was used to calculate statistical significance of the cost.

RESULTS

We found that 82.7% of the patients who received LMWID had an increase in hemoglobin vs. 60.6% in the iron sucrose group. 48.3% of patients in LMWID had ≥ 2 hemoglobin increases vs. 27.3% in the iron sucrose group. The cost for LMWID administration was $2016.10, compared to $2315.40 for administration of IV iron sucrose cost. Two-tailed p value < 0.0001 indicating the observed difference to be statistically significant. No infusion reactions were observed in both groups.

CONCLUSIONS

In this single center analysis, IV LMWID administered provided comparable safety, and improved effectiveness, and cost-effectiveness to iron sucrose.

PURPOSE

To evaluate if low molecular weight iron dextran (LMWID) is a safe and effective alternative to iron sucrose for intravenous iron administration.

BACKGROUND

In recent years, intravenous iron administration has increased due to clinical indications and rapid iron repletion. Early IV iron formulations had safety concerns that precluded widespread use. High molecular weight iron dextran was removed from the US market in 2009 due to safety concerns. Since then, several new IV formulations including LMWID and iron sucrose have been approved with a favorable benefit risk profile. While recent evidence and guidelines indicate that LMWID and other iron formulations have comparable safety profiles, no head-to-head comparisons exist. Both iron sucrose and LMWID are used for the treatment of IDA in Veterans Affairs hospitals. Iron sucrose is given 200 mg weekly for 5 weeks, while LMWID is given as a single 1-gram dose over 3 hours. We conducted a retrospective crosssectional analysis to compare the safety and efficacy of IV LMWID to IV iron sucrose.

METHODS

We identified 129 patients (LMWID: n=29, iron sucrose: n=100) who received intravenous iron from 01/01/2022 to 03/03/2023. To match the sample size, we selected every 3rd patient from the iron sucrose group (n=33). We captured data on infusion-related reactions, history of asthma/inflammatory bowel disease/> 2 drug allergies, overall and ≥ 2 g/dL hemoglobin increase, and treatment cost. Descriptive statistics were used to describe the safety and efficacy parameters. An unpaired t-test was used to calculate statistical significance of the cost.

RESULTS

We found that 82.7% of the patients who received LMWID had an increase in hemoglobin vs. 60.6% in the iron sucrose group. 48.3% of patients in LMWID had ≥ 2 hemoglobin increases vs. 27.3% in the iron sucrose group. The cost for LMWID administration was $2016.10, compared to $2315.40 for administration of IV iron sucrose cost. Two-tailed p value < 0.0001 indicating the observed difference to be statistically significant. No infusion reactions were observed in both groups.

CONCLUSIONS

In this single center analysis, IV LMWID administered provided comparable safety, and improved effectiveness, and cost-effectiveness to iron sucrose.

PURPOSE

To evaluate if low molecular weight iron dextran (LMWID) is a safe and effective alternative to iron sucrose for intravenous iron administration.

BACKGROUND

In recent years, intravenous iron administration has increased due to clinical indications and rapid iron repletion. Early IV iron formulations had safety concerns that precluded widespread use. High molecular weight iron dextran was removed from the US market in 2009 due to safety concerns. Since then, several new IV formulations including LMWID and iron sucrose have been approved with a favorable benefit risk profile. While recent evidence and guidelines indicate that LMWID and other iron formulations have comparable safety profiles, no head-to-head comparisons exist. Both iron sucrose and LMWID are used for the treatment of IDA in Veterans Affairs hospitals. Iron sucrose is given 200 mg weekly for 5 weeks, while LMWID is given as a single 1-gram dose over 3 hours. We conducted a retrospective crosssectional analysis to compare the safety and efficacy of IV LMWID to IV iron sucrose.

METHODS

We identified 129 patients (LMWID: n=29, iron sucrose: n=100) who received intravenous iron from 01/01/2022 to 03/03/2023. To match the sample size, we selected every 3rd patient from the iron sucrose group (n=33). We captured data on infusion-related reactions, history of asthma/inflammatory bowel disease/> 2 drug allergies, overall and ≥ 2 g/dL hemoglobin increase, and treatment cost. Descriptive statistics were used to describe the safety and efficacy parameters. An unpaired t-test was used to calculate statistical significance of the cost.

RESULTS

We found that 82.7% of the patients who received LMWID had an increase in hemoglobin vs. 60.6% in the iron sucrose group. 48.3% of patients in LMWID had ≥ 2 hemoglobin increases vs. 27.3% in the iron sucrose group. The cost for LMWID administration was $2016.10, compared to $2315.40 for administration of IV iron sucrose cost. Two-tailed p value < 0.0001 indicating the observed difference to be statistically significant. No infusion reactions were observed in both groups.

CONCLUSIONS

In this single center analysis, IV LMWID administered provided comparable safety, and improved effectiveness, and cost-effectiveness to iron sucrose.

PURPOSE

To improve the time to release of blood products for patients with severe or life-threatening bleeding.

BACKGROUND

Exsanguination, and the resultant coagulopathy, is the number one cause of trauma-related death. Massive transfusion protocols (MTP) improve mortality by shortening the time to transfusion and correcting coagulopathy. Many patients do not meet criteria for massive transfusion (> 10 units RBCs in 24 hours), yet present with clinical instability and require rapid release (RR) of uncrossmatched blood. A quality improvement initiative was performed to identify barriers to the MTP/RR protocol at a single institution.

METHODS/DATA

A multidisciplinary subcommittee was formed to evaluate the safety and efficacy of the current MTP/RR process. Timed mock-MTP/RR trials were conducted to identify areas of delay with a goal to achieve a blood to bedside (B2B) time of under 10 minutes.

RESULTS

Timed mock-MTP/RR trials were conducted, which revealed a baseline B2B time of approximately 30 minutes. We identified problems and categorized them in terms of ordering (phase 1) and processing (phase 2). We found significant delays in phase 1. Reasons for delay were varied and included difficulty logging into the computer, staff unavailable to place orders (involved in resuscitation efforts), orders entered incorrectly, etc. Once orders were received, the blood bank could process them quickly in phase 2. Using root cause analysis, we discovered a critical step was to remove the barrier of electronic ordering. For this, a new process was developed in which the blood bank could accept verbal orders to release uncrossmatched blood during a medical emergency. Over the course of one year, a new policy for MTP/RR was drafted, an education training video was recorded, informational flyers were printed, and training drills were conducted. A repeat mock-MTP/RR scenario was performed after the change showing the B2B time was reduced by 90% from pre-intervention values to under 3 minutes. Since implementation, no new safety signals have been received, and the staff have reported improved satisfaction with the MTP/RR process.

IMPLICATIONS

A critical piece of any MTP/RR is the immediate availability of blood. Allowing verbal orders for blood products reduced time to transfusion by 90%. Through multidisciplinary effort, safe and efficient release of uncrossmatched blood products for nontraumatic massive transfusion can be achieved.

PURPOSE

To improve the time to release of blood products for patients with severe or life-threatening bleeding.

BACKGROUND

Exsanguination, and the resultant coagulopathy, is the number one cause of trauma-related death. Massive transfusion protocols (MTP) improve mortality by shortening the time to transfusion and correcting coagulopathy. Many patients do not meet criteria for massive transfusion (> 10 units RBCs in 24 hours), yet present with clinical instability and require rapid release (RR) of uncrossmatched blood. A quality improvement initiative was performed to identify barriers to the MTP/RR protocol at a single institution.

METHODS/DATA

A multidisciplinary subcommittee was formed to evaluate the safety and efficacy of the current MTP/RR process. Timed mock-MTP/RR trials were conducted to identify areas of delay with a goal to achieve a blood to bedside (B2B) time of under 10 minutes.

RESULTS

Timed mock-MTP/RR trials were conducted, which revealed a baseline B2B time of approximately 30 minutes. We identified problems and categorized them in terms of ordering (phase 1) and processing (phase 2). We found significant delays in phase 1. Reasons for delay were varied and included difficulty logging into the computer, staff unavailable to place orders (involved in resuscitation efforts), orders entered incorrectly, etc. Once orders were received, the blood bank could process them quickly in phase 2. Using root cause analysis, we discovered a critical step was to remove the barrier of electronic ordering. For this, a new process was developed in which the blood bank could accept verbal orders to release uncrossmatched blood during a medical emergency. Over the course of one year, a new policy for MTP/RR was drafted, an education training video was recorded, informational flyers were printed, and training drills were conducted. A repeat mock-MTP/RR scenario was performed after the change showing the B2B time was reduced by 90% from pre-intervention values to under 3 minutes. Since implementation, no new safety signals have been received, and the staff have reported improved satisfaction with the MTP/RR process.

IMPLICATIONS

A critical piece of any MTP/RR is the immediate availability of blood. Allowing verbal orders for blood products reduced time to transfusion by 90%. Through multidisciplinary effort, safe and efficient release of uncrossmatched blood products for nontraumatic massive transfusion can be achieved.

PURPOSE

To improve the time to release of blood products for patients with severe or life-threatening bleeding.

BACKGROUND

Exsanguination, and the resultant coagulopathy, is the number one cause of trauma-related death. Massive transfusion protocols (MTP) improve mortality by shortening the time to transfusion and correcting coagulopathy. Many patients do not meet criteria for massive transfusion (> 10 units RBCs in 24 hours), yet present with clinical instability and require rapid release (RR) of uncrossmatched blood. A quality improvement initiative was performed to identify barriers to the MTP/RR protocol at a single institution.

METHODS/DATA

A multidisciplinary subcommittee was formed to evaluate the safety and efficacy of the current MTP/RR process. Timed mock-MTP/RR trials were conducted to identify areas of delay with a goal to achieve a blood to bedside (B2B) time of under 10 minutes.

RESULTS

Timed mock-MTP/RR trials were conducted, which revealed a baseline B2B time of approximately 30 minutes. We identified problems and categorized them in terms of ordering (phase 1) and processing (phase 2). We found significant delays in phase 1. Reasons for delay were varied and included difficulty logging into the computer, staff unavailable to place orders (involved in resuscitation efforts), orders entered incorrectly, etc. Once orders were received, the blood bank could process them quickly in phase 2. Using root cause analysis, we discovered a critical step was to remove the barrier of electronic ordering. For this, a new process was developed in which the blood bank could accept verbal orders to release uncrossmatched blood during a medical emergency. Over the course of one year, a new policy for MTP/RR was drafted, an education training video was recorded, informational flyers were printed, and training drills were conducted. A repeat mock-MTP/RR scenario was performed after the change showing the B2B time was reduced by 90% from pre-intervention values to under 3 minutes. Since implementation, no new safety signals have been received, and the staff have reported improved satisfaction with the MTP/RR process.

IMPLICATIONS

A critical piece of any MTP/RR is the immediate availability of blood. Allowing verbal orders for blood products reduced time to transfusion by 90%. Through multidisciplinary effort, safe and efficient release of uncrossmatched blood products for nontraumatic massive transfusion can be achieved.

PURPOSE

To improve germline genetic testing among Veterans with high risk, very high risk and metastatic prostate cancer.

BACKGROUND

During our Commission on Cancer survey in 2021, it was noted that the Detroit VA’s referrals for germline genetic testing and counseling were extremely low. In 2020, only 1 Veteran was referred for prostate germline genetic testing and counseling and only 8 Veterans were referred in 2021. It was felt that the need to refer Veterans outside of the Detroit VA may have contributed to these low numbers. Our Cancer Committee chose prostate cancer as a disease to focus on. We chose a timeline of one year to implement our process.

METHODS

We made testing and counseling locally accessible to Veterans and encouraged medical oncology providers to make it part of the care of Veterans with high risk, very high risk and metastatic prostate cancer. We sought the assistance of the VA’s National Precision Oncology Program and were able to secure financial and logistical support to perform germline molecular prostate panel testing at the Detroit VA. We were also able to identify a cancer genetic specialist at the Ann Arbor VA that would perform genetic counseling among this group of patients based on their test results. Our medical oncology providers identified Veterans meeting the criteria for testing. Education regarding germline testing, its benefits and implications were conducted with Veterans, and performed after obtaining their informed consent in collaboration with our pathology department. The specimen is then sent to a VA central laboratory for processing. Detroit VA providers are alerted by the local laboratory once results are available. Veterans are then referred to the genetic counseling specialist based on the results. Some of these counseling visits are done virtually for the Veteran’s convenience.

DATA ANALYSIS

A retrospective chart analysis was used to collect the data.

RESULTS

After the implementation of our initiative, 97 Veterans with high risk, very high risk or metastatic prostate cancer were educated on the benefits of germline genetic testing, 87 of whom agreed to be tested. As of 4/2/23, 48 tests have already been performed. Pathogenic variants were recorded on 2 Veterans so far. One was for BRCA2 and KDM6A, and the other was for ATM. Data collection and recording is on-going.

IMPLICATIONS

Improving accessibility and incorporating genetic testing and counseling in cancer care can improve their utilization.

PURPOSE

To improve germline genetic testing among Veterans with high risk, very high risk and metastatic prostate cancer.

BACKGROUND

During our Commission on Cancer survey in 2021, it was noted that the Detroit VA’s referrals for germline genetic testing and counseling were extremely low. In 2020, only 1 Veteran was referred for prostate germline genetic testing and counseling and only 8 Veterans were referred in 2021. It was felt that the need to refer Veterans outside of the Detroit VA may have contributed to these low numbers. Our Cancer Committee chose prostate cancer as a disease to focus on. We chose a timeline of one year to implement our process.

METHODS

We made testing and counseling locally accessible to Veterans and encouraged medical oncology providers to make it part of the care of Veterans with high risk, very high risk and metastatic prostate cancer. We sought the assistance of the VA’s National Precision Oncology Program and were able to secure financial and logistical support to perform germline molecular prostate panel testing at the Detroit VA. We were also able to identify a cancer genetic specialist at the Ann Arbor VA that would perform genetic counseling among this group of patients based on their test results. Our medical oncology providers identified Veterans meeting the criteria for testing. Education regarding germline testing, its benefits and implications were conducted with Veterans, and performed after obtaining their informed consent in collaboration with our pathology department. The specimen is then sent to a VA central laboratory for processing. Detroit VA providers are alerted by the local laboratory once results are available. Veterans are then referred to the genetic counseling specialist based on the results. Some of these counseling visits are done virtually for the Veteran’s convenience.

DATA ANALYSIS

A retrospective chart analysis was used to collect the data.

RESULTS

After the implementation of our initiative, 97 Veterans with high risk, very high risk or metastatic prostate cancer were educated on the benefits of germline genetic testing, 87 of whom agreed to be tested. As of 4/2/23, 48 tests have already been performed. Pathogenic variants were recorded on 2 Veterans so far. One was for BRCA2 and KDM6A, and the other was for ATM. Data collection and recording is on-going.

IMPLICATIONS

Improving accessibility and incorporating genetic testing and counseling in cancer care can improve their utilization.

PURPOSE

To improve germline genetic testing among Veterans with high risk, very high risk and metastatic prostate cancer.

BACKGROUND

During our Commission on Cancer survey in 2021, it was noted that the Detroit VA’s referrals for germline genetic testing and counseling were extremely low. In 2020, only 1 Veteran was referred for prostate germline genetic testing and counseling and only 8 Veterans were referred in 2021. It was felt that the need to refer Veterans outside of the Detroit VA may have contributed to these low numbers. Our Cancer Committee chose prostate cancer as a disease to focus on. We chose a timeline of one year to implement our process.

METHODS

We made testing and counseling locally accessible to Veterans and encouraged medical oncology providers to make it part of the care of Veterans with high risk, very high risk and metastatic prostate cancer. We sought the assistance of the VA’s National Precision Oncology Program and were able to secure financial and logistical support to perform germline molecular prostate panel testing at the Detroit VA. We were also able to identify a cancer genetic specialist at the Ann Arbor VA that would perform genetic counseling among this group of patients based on their test results. Our medical oncology providers identified Veterans meeting the criteria for testing. Education regarding germline testing, its benefits and implications were conducted with Veterans, and performed after obtaining their informed consent in collaboration with our pathology department. The specimen is then sent to a VA central laboratory for processing. Detroit VA providers are alerted by the local laboratory once results are available. Veterans are then referred to the genetic counseling specialist based on the results. Some of these counseling visits are done virtually for the Veteran’s convenience.

DATA ANALYSIS

A retrospective chart analysis was used to collect the data.

RESULTS

After the implementation of our initiative, 97 Veterans with high risk, very high risk or metastatic prostate cancer were educated on the benefits of germline genetic testing, 87 of whom agreed to be tested. As of 4/2/23, 48 tests have already been performed. Pathogenic variants were recorded on 2 Veterans so far. One was for BRCA2 and KDM6A, and the other was for ATM. Data collection and recording is on-going.

IMPLICATIONS

Improving accessibility and incorporating genetic testing and counseling in cancer care can improve their utilization.

A 45-year-old women presents for evaluation of fatigue. She has been tired for the past 6 months. She has had no problems with sleep and no other new symptoms. Her physical exam is unremarkable. Her Patient Health Questionnaire–9 score is 4. Lab results are as follows: hemoglobin, 13 g/dL; hematocrit, 39%; mean corpuscular volume, 90 fL; blood urea nitrogen, 10 mg/dL; Cr, 1.0 mg/dL; AST, 20 IU/L; ALT, 15 IU/L; ferritin, 35 mcg/mL; thyroid-stimulating hormone, 3.5 mIU/L.

What would you recommend?

A. Sertraline

B. Sleep study

C. Iron supplementation

Dr. Paauw is professor of medicine in the division of general internal medicine at the University of Washington, Seattle, and he serves as third-year medical student clerkship director at the University of Washington. Contact Dr. Paauw at [email protected].

References

1. Verdon F et al. BMJ. 2003 May 24;326(7399):1124. .

2. Houston BL et al. BMJ Open. 2018 Apr 5;8(4):e019240.

3. Almohanna HM et al. Dermatol Ther (Heidelb). 2019 Mar;9(1):51-70. .

4. Hard S. Acta Derm Venereol. 1963;43:562-9.

5. Kantor J et al. J Invest Dermatol. 2003 Nov;121(5):985-8. .

6. Allen RP et al. Sleep Med. 2018 Jan;41:27-44. .

A 45-year-old women presents for evaluation of fatigue. She has been tired for the past 6 months. She has had no problems with sleep and no other new symptoms. Her physical exam is unremarkable. Her Patient Health Questionnaire–9 score is 4. Lab results are as follows: hemoglobin, 13 g/dL; hematocrit, 39%; mean corpuscular volume, 90 fL; blood urea nitrogen, 10 mg/dL; Cr, 1.0 mg/dL; AST, 20 IU/L; ALT, 15 IU/L; ferritin, 35 mcg/mL; thyroid-stimulating hormone, 3.5 mIU/L.

What would you recommend?

A. Sertraline

B. Sleep study

C. Iron supplementation

Dr. Paauw is professor of medicine in the division of general internal medicine at the University of Washington, Seattle, and he serves as third-year medical student clerkship director at the University of Washington. Contact Dr. Paauw at [email protected].

References

1. Verdon F et al. BMJ. 2003 May 24;326(7399):1124. .

2. Houston BL et al. BMJ Open. 2018 Apr 5;8(4):e019240.

3. Almohanna HM et al. Dermatol Ther (Heidelb). 2019 Mar;9(1):51-70. .

4. Hard S. Acta Derm Venereol. 1963;43:562-9.

5. Kantor J et al. J Invest Dermatol. 2003 Nov;121(5):985-8. .

6. Allen RP et al. Sleep Med. 2018 Jan;41:27-44. .

A 45-year-old women presents for evaluation of fatigue. She has been tired for the past 6 months. She has had no problems with sleep and no other new symptoms. Her physical exam is unremarkable. Her Patient Health Questionnaire–9 score is 4. Lab results are as follows: hemoglobin, 13 g/dL; hematocrit, 39%; mean corpuscular volume, 90 fL; blood urea nitrogen, 10 mg/dL; Cr, 1.0 mg/dL; AST, 20 IU/L; ALT, 15 IU/L; ferritin, 35 mcg/mL; thyroid-stimulating hormone, 3.5 mIU/L.

What would you recommend?

A. Sertraline

B. Sleep study

C. Iron supplementation

Dr. Paauw is professor of medicine in the division of general internal medicine at the University of Washington, Seattle, and he serves as third-year medical student clerkship director at the University of Washington. Contact Dr. Paauw at [email protected].

References

1. Verdon F et al. BMJ. 2003 May 24;326(7399):1124. .

2. Houston BL et al. BMJ Open. 2018 Apr 5;8(4):e019240.

3. Almohanna HM et al. Dermatol Ther (Heidelb). 2019 Mar;9(1):51-70. .

4. Hard S. Acta Derm Venereol. 1963;43:562-9.

5. Kantor J et al. J Invest Dermatol. 2003 Nov;121(5):985-8. .

6. Allen RP et al. Sleep Med. 2018 Jan;41:27-44. .

A 55-YEAR-OLD WOMAN developed a small red papule on her left hand that, over the course of a week, progressed rapidly into an ulcerated nodule with accompanying swelling and pain. She reported concomitant fatigue, unintentional weight loss, and swollen axillary lymph nodes. Past medical history included rheumatoid arthritis.

A physical examination of her left hand revealed a tender, erythematous to violaceous nodule with ulceration and crust and surrounding diffuse erythema and edema ­(FIGURE). She also had several enlarged, nontender right axillary lymph nodes. Initial lab evaluation was significant for leukocytosis (13.8 K/uL) with increased neutrophils, lymphocytes, and eosinophils. Two punch biopsies were performed and the samples submitted for hematoxylin and eosin (H&E) staining and tissue culture.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

 

 

Diagnosis: Neutrophilic dermatosis of the dorsal hands

The results of H&E were consistent with neutrophilic dermatosis of the dorsal hands (NDDH). Tissue culture was negative for fungus, bacteria, and atypical mycobacteria, confirming the diagnosis.

NDDH often is mistaken for an infectious abscess and unsuccessfully treated with antimicrobial agents.

NDDH is a neutrophilic dermatosis and considered a localized variant of Sweet syndrome, manifesting on the dorsal hands as suppurative, erythematous to violaceous papules, plaques, or nodules that often undergo necrosis, blistering, and ulceration. The diagnosis can be made clinically, although a biopsy is usually performed for confirmation. It is characterized histologically by a dense dermal neutrophilic infiltrate along with dermal ­edema.¹

The pathogenesis of NDDH is not fully known.² It is often preceded by trauma and may be associated with recent infection (respiratory, gastrointestinal), inflammatory bowel disease, autoimmune disease (eg, rheumatoid arthritis), or malignancy.¹ The most common associated malignancies are hematologic, such as myelodysplastic syndrome, leukemia, or lymphoma, although solid tumors also can be seen.^1,3 Therefore, patients who receive a diagnosis of NDDH typically require further work-up to rule out these associated conditions. NDDH is a rare enough entity that incidence/prevalence data aren’t available or likely to be accurate.

The differential includes infection and neoplastic processes

NDDH often is mistaken for an infectious abscess and unsuccessfully treated with antimicrobial agents, such as those commonly used for staphylococcus and streptococcus skin and soft-tissue infections. Thus, wound or tissue culture may be considered to exclude infection from the differential diagnosis. In addition to infectious processes such as sporotrichosis or an atypical mycobacterial infection, the differential includes other neutrophilic dermatoses and neoplastic processes such as lymphoma or leukemia cutis.

Sporotrichosis is caused by Sporothrix schenckii and usually spreads proximally after entering through a wound or cut. Special stains on histology and culture are needed to make the diagnosis.

Continue to: Atypical mycobacterial infections

Atypical mycobacterial infections usually enter through an area of trauma and spread proximally after inoculation. Atypical mycobacterial infections can be diagnosed via biopsy with special stains, culture, and polymerase chain reaction of the tissue.

Neutrophilic dermatoses are a broad category of dermatoses that include NDDH, pyoderma gangrenosum, and Sweet syndrome. This category of dermatoses is differentiated by morphology and distribution of lesions.

Lymphoma can be primary cutaneous or secondary to a systemic lymphoma. A biopsy will show a collection of atypical lymphocytes.

Treatment begins with steroids

Treatment with topical (eg, 0.05% clobetasol ointment bid), intralesional (10 to 40 mg/mL triamcinolone acetonide), or systemic (eg, prednisone 0.5 to 1 mg/kg tapered over the course of 1-2 months) steroids is considered first-line therapy and often results in rapid clinical improvement. Agents such as dapsone (25 to 150 mg/d) and/or colchicine (0.6 mg bid to tid) may be used in recalcitrant cases or in patients for whom steroids are contra­indicated.²

Our patient’s NDDH was treated with prednisone (~1.0 mg/kg daily tapered over the course of 6 weeks). She was referred to Hematology/­Oncology for further work-up of her constitutional symptoms, lymphadenopathy,­ and leukocytosis. Ultimately, she received a diagnosis of concomitant chronic lymphocytic leukemia/small lymphocytic lymphoma. The patient required no immediate treatment for her indolent lymphoma and was advised that she would need to get blood work done on a regular basis and have annual check-ups.

A 55-YEAR-OLD WOMAN developed a small red papule on her left hand that, over the course of a week, progressed rapidly into an ulcerated nodule with accompanying swelling and pain. She reported concomitant fatigue, unintentional weight loss, and swollen axillary lymph nodes. Past medical history included rheumatoid arthritis.

A physical examination of her left hand revealed a tender, erythematous to violaceous nodule with ulceration and crust and surrounding diffuse erythema and edema ­(FIGURE). She also had several enlarged, nontender right axillary lymph nodes. Initial lab evaluation was significant for leukocytosis (13.8 K/uL) with increased neutrophils, lymphocytes, and eosinophils. Two punch biopsies were performed and the samples submitted for hematoxylin and eosin (H&E) staining and tissue culture.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

 

 

Diagnosis: Neutrophilic dermatosis of the dorsal hands

The results of H&E were consistent with neutrophilic dermatosis of the dorsal hands (NDDH). Tissue culture was negative for fungus, bacteria, and atypical mycobacteria, confirming the diagnosis.

NDDH often is mistaken for an infectious abscess and unsuccessfully treated with antimicrobial agents.

NDDH is a neutrophilic dermatosis and considered a localized variant of Sweet syndrome, manifesting on the dorsal hands as suppurative, erythematous to violaceous papules, plaques, or nodules that often undergo necrosis, blistering, and ulceration. The diagnosis can be made clinically, although a biopsy is usually performed for confirmation. It is characterized histologically by a dense dermal neutrophilic infiltrate along with dermal ­edema.¹

The pathogenesis of NDDH is not fully known.² It is often preceded by trauma and may be associated with recent infection (respiratory, gastrointestinal), inflammatory bowel disease, autoimmune disease (eg, rheumatoid arthritis), or malignancy.¹ The most common associated malignancies are hematologic, such as myelodysplastic syndrome, leukemia, or lymphoma, although solid tumors also can be seen.^1,3 Therefore, patients who receive a diagnosis of NDDH typically require further work-up to rule out these associated conditions. NDDH is a rare enough entity that incidence/prevalence data aren’t available or likely to be accurate.

The differential includes infection and neoplastic processes

NDDH often is mistaken for an infectious abscess and unsuccessfully treated with antimicrobial agents, such as those commonly used for staphylococcus and streptococcus skin and soft-tissue infections. Thus, wound or tissue culture may be considered to exclude infection from the differential diagnosis. In addition to infectious processes such as sporotrichosis or an atypical mycobacterial infection, the differential includes other neutrophilic dermatoses and neoplastic processes such as lymphoma or leukemia cutis.

Sporotrichosis is caused by Sporothrix schenckii and usually spreads proximally after entering through a wound or cut. Special stains on histology and culture are needed to make the diagnosis.

Continue to: Atypical mycobacterial infections

Atypical mycobacterial infections usually enter through an area of trauma and spread proximally after inoculation. Atypical mycobacterial infections can be diagnosed via biopsy with special stains, culture, and polymerase chain reaction of the tissue.

Neutrophilic dermatoses are a broad category of dermatoses that include NDDH, pyoderma gangrenosum, and Sweet syndrome. This category of dermatoses is differentiated by morphology and distribution of lesions.

Lymphoma can be primary cutaneous or secondary to a systemic lymphoma. A biopsy will show a collection of atypical lymphocytes.

Treatment begins with steroids

Treatment with topical (eg, 0.05% clobetasol ointment bid), intralesional (10 to 40 mg/mL triamcinolone acetonide), or systemic (eg, prednisone 0.5 to 1 mg/kg tapered over the course of 1-2 months) steroids is considered first-line therapy and often results in rapid clinical improvement. Agents such as dapsone (25 to 150 mg/d) and/or colchicine (0.6 mg bid to tid) may be used in recalcitrant cases or in patients for whom steroids are contra­indicated.²

Our patient’s NDDH was treated with prednisone (~1.0 mg/kg daily tapered over the course of 6 weeks). She was referred to Hematology/­Oncology for further work-up of her constitutional symptoms, lymphadenopathy,­ and leukocytosis. Ultimately, she received a diagnosis of concomitant chronic lymphocytic leukemia/small lymphocytic lymphoma. The patient required no immediate treatment for her indolent lymphoma and was advised that she would need to get blood work done on a regular basis and have annual check-ups.

A 55-YEAR-OLD WOMAN developed a small red papule on her left hand that, over the course of a week, progressed rapidly into an ulcerated nodule with accompanying swelling and pain. She reported concomitant fatigue, unintentional weight loss, and swollen axillary lymph nodes. Past medical history included rheumatoid arthritis.

A physical examination of her left hand revealed a tender, erythematous to violaceous nodule with ulceration and crust and surrounding diffuse erythema and edema ­(FIGURE). She also had several enlarged, nontender right axillary lymph nodes. Initial lab evaluation was significant for leukocytosis (13.8 K/uL) with increased neutrophils, lymphocytes, and eosinophils. Two punch biopsies were performed and the samples submitted for hematoxylin and eosin (H&E) staining and tissue culture.

WHAT IS YOUR DIAGNOSIS?
HOW WOULD YOU TREAT THIS PATIENT?

 

 

Diagnosis: Neutrophilic dermatosis of the dorsal hands

The results of H&E were consistent with neutrophilic dermatosis of the dorsal hands (NDDH). Tissue culture was negative for fungus, bacteria, and atypical mycobacteria, confirming the diagnosis.

NDDH often is mistaken for an infectious abscess and unsuccessfully treated with antimicrobial agents.

NDDH is a neutrophilic dermatosis and considered a localized variant of Sweet syndrome, manifesting on the dorsal hands as suppurative, erythematous to violaceous papules, plaques, or nodules that often undergo necrosis, blistering, and ulceration. The diagnosis can be made clinically, although a biopsy is usually performed for confirmation. It is characterized histologically by a dense dermal neutrophilic infiltrate along with dermal ­edema.¹

The pathogenesis of NDDH is not fully known.² It is often preceded by trauma and may be associated with recent infection (respiratory, gastrointestinal), inflammatory bowel disease, autoimmune disease (eg, rheumatoid arthritis), or malignancy.¹ The most common associated malignancies are hematologic, such as myelodysplastic syndrome, leukemia, or lymphoma, although solid tumors also can be seen.^1,3 Therefore, patients who receive a diagnosis of NDDH typically require further work-up to rule out these associated conditions. NDDH is a rare enough entity that incidence/prevalence data aren’t available or likely to be accurate.

The differential includes infection and neoplastic processes

NDDH often is mistaken for an infectious abscess and unsuccessfully treated with antimicrobial agents, such as those commonly used for staphylococcus and streptococcus skin and soft-tissue infections. Thus, wound or tissue culture may be considered to exclude infection from the differential diagnosis. In addition to infectious processes such as sporotrichosis or an atypical mycobacterial infection, the differential includes other neutrophilic dermatoses and neoplastic processes such as lymphoma or leukemia cutis.

Sporotrichosis is caused by Sporothrix schenckii and usually spreads proximally after entering through a wound or cut. Special stains on histology and culture are needed to make the diagnosis.

Continue to: Atypical mycobacterial infections

Atypical mycobacterial infections usually enter through an area of trauma and spread proximally after inoculation. Atypical mycobacterial infections can be diagnosed via biopsy with special stains, culture, and polymerase chain reaction of the tissue.

Neutrophilic dermatoses are a broad category of dermatoses that include NDDH, pyoderma gangrenosum, and Sweet syndrome. This category of dermatoses is differentiated by morphology and distribution of lesions.

Lymphoma can be primary cutaneous or secondary to a systemic lymphoma. A biopsy will show a collection of atypical lymphocytes.

Treatment begins with steroids

Treatment with topical (eg, 0.05% clobetasol ointment bid), intralesional (10 to 40 mg/mL triamcinolone acetonide), or systemic (eg, prednisone 0.5 to 1 mg/kg tapered over the course of 1-2 months) steroids is considered first-line therapy and often results in rapid clinical improvement. Agents such as dapsone (25 to 150 mg/d) and/or colchicine (0.6 mg bid to tid) may be used in recalcitrant cases or in patients for whom steroids are contra­indicated.²

Our patient’s NDDH was treated with prednisone (~1.0 mg/kg daily tapered over the course of 6 weeks). She was referred to Hematology/­Oncology for further work-up of her constitutional symptoms, lymphadenopathy,­ and leukocytosis. Ultimately, she received a diagnosis of concomitant chronic lymphocytic leukemia/small lymphocytic lymphoma. The patient required no immediate treatment for her indolent lymphoma and was advised that she would need to get blood work done on a regular basis and have annual check-ups.

Nearly 40% of girls and young women in the United States may have iron deficiency, which can lead to fatigue and increase the risk of many health problems, according to a new study. 

Researchers also found that 6 in every 100 of the girls and young women had extremely low iron levels, known as iron-deficiency anemia, which impacts the blood’s ability to carry oxygen throughout the body.

The findings suggest that current screening guidelines for iron levels in girls and women may be flawed, resulting in missed chances to get a simple blood test that can diagnose the easy-to-treat condition. Iron supplements are often prescribed as a treatment.

The study was published in JAMA and included 12 years of data for a total of nearly 3,500 girls and women aged 12-21 years.

In addition to shortness of breath and fatigue, other symptoms of iron deficiency anemia are: 

• Pale skin
• Cold hands and feet
• Feeling dizzy or lightheaded
• Unusual cravings for nonfood items such as ice, dirt, or paper.

The Cleveland Clinic says the most common causes of iron-deficiency anemia are those that involve blood loss, including heavy menstrual periods. The body gets iron from food, and not getting enough iron from food, as can happen from eating a vegan or vegetarian diet, can also lead to deficiency. 

In this latest study, researchers found that young women and girls’ likelihood to have iron deficiency or iron-deficiency anemia were significantly linked to race and ethnicity, poverty status, access to sufficient or quality food (also called food insecurity), and body mass index. Black and Hispanic girls and women were more likely to have iron level problems, compared with White girls and women. Black girls and women were four times more likely to have iron-deficiency anemia, compared with White girls and women.

The authors did not discuss potential causes and suggested further study is needed to identify risk factors of iron deficiency in girls and young women.

A version of this article originally appeared on WebMD.com.

Nearly 40% of girls and young women in the United States may have iron deficiency, which can lead to fatigue and increase the risk of many health problems, according to a new study. 

Researchers also found that 6 in every 100 of the girls and young women had extremely low iron levels, known as iron-deficiency anemia, which impacts the blood’s ability to carry oxygen throughout the body.

The findings suggest that current screening guidelines for iron levels in girls and women may be flawed, resulting in missed chances to get a simple blood test that can diagnose the easy-to-treat condition. Iron supplements are often prescribed as a treatment.

The study was published in JAMA and included 12 years of data for a total of nearly 3,500 girls and women aged 12-21 years.

In addition to shortness of breath and fatigue, other symptoms of iron deficiency anemia are: 

• Pale skin
• Cold hands and feet
• Feeling dizzy or lightheaded
• Unusual cravings for nonfood items such as ice, dirt, or paper.

The Cleveland Clinic says the most common causes of iron-deficiency anemia are those that involve blood loss, including heavy menstrual periods. The body gets iron from food, and not getting enough iron from food, as can happen from eating a vegan or vegetarian diet, can also lead to deficiency. 

In this latest study, researchers found that young women and girls’ likelihood to have iron deficiency or iron-deficiency anemia were significantly linked to race and ethnicity, poverty status, access to sufficient or quality food (also called food insecurity), and body mass index. Black and Hispanic girls and women were more likely to have iron level problems, compared with White girls and women. Black girls and women were four times more likely to have iron-deficiency anemia, compared with White girls and women.

The authors did not discuss potential causes and suggested further study is needed to identify risk factors of iron deficiency in girls and young women.

A version of this article originally appeared on WebMD.com.

Nearly 40% of girls and young women in the United States may have iron deficiency, which can lead to fatigue and increase the risk of many health problems, according to a new study. 

Researchers also found that 6 in every 100 of the girls and young women had extremely low iron levels, known as iron-deficiency anemia, which impacts the blood’s ability to carry oxygen throughout the body.

The findings suggest that current screening guidelines for iron levels in girls and women may be flawed, resulting in missed chances to get a simple blood test that can diagnose the easy-to-treat condition. Iron supplements are often prescribed as a treatment.

The study was published in JAMA and included 12 years of data for a total of nearly 3,500 girls and women aged 12-21 years.

In addition to shortness of breath and fatigue, other symptoms of iron deficiency anemia are: 

• Pale skin
• Cold hands and feet
• Feeling dizzy or lightheaded
• Unusual cravings for nonfood items such as ice, dirt, or paper.

The Cleveland Clinic says the most common causes of iron-deficiency anemia are those that involve blood loss, including heavy menstrual periods. The body gets iron from food, and not getting enough iron from food, as can happen from eating a vegan or vegetarian diet, can also lead to deficiency. 

In this latest study, researchers found that young women and girls’ likelihood to have iron deficiency or iron-deficiency anemia were significantly linked to race and ethnicity, poverty status, access to sufficient or quality food (also called food insecurity), and body mass index. Black and Hispanic girls and women were more likely to have iron level problems, compared with White girls and women. Black girls and women were four times more likely to have iron-deficiency anemia, compared with White girls and women.

The authors did not discuss potential causes and suggested further study is needed to identify risk factors of iron deficiency in girls and young women.

A version of this article originally appeared on WebMD.com.