Factors Associated with Survival and Epidemiology of Gastrointestinal Neuroendocrine Tumors in the US Department of Veteran Affairs

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Introduction

Rectal carcinoid tumors are rare but the second most common carcinoid in the gastrointestinal tract. They are usually found incidentally during endoscopic or rectal examination. They do not often produce carcinoid syndrome like manifestations although they may manifest as rectal bleeding. Rectal carcinoid patients also have a higher morbidity for other cancers such as stomach, small intestine, or secondary lung cancer.

Methods

We retrospectively explored factors associated with survival in Veterans with rectal carcinoid tumors over a ten-year period from 2007-2017 using the National Veterans Affairs Cancer Cube Registry using specific histological ICD-03 coding. We identified 1110 cases of rectal carcinoid. Chi-squared tests were used for statistical analysis.

Results

Regarding age distribution in our cohort, there were 2.61% of patients ages 40-50 group, 14.0% in the 50-60 age group, 41.5% in the 60-70 age group, and 40.7% above ages 70. There was a higher proportion of rectal cancer in stage 1 compared to other stages (86.3%). The majority of diagnoses occur after age 50 (89.8%). A higher proportion of rectal carcinoid was identified in the 60-70 years category compared to <60 and >70 years old. In the general VA population, there are 80.2% White and 12.8% Black patients. We found a higher proportion of rectal carcinoid in Black patients (47.8%) over White patients (42.8%, p=0.02), which differs significantly from the racial makeup of the VA population (12.8% Black vs 80.3% White). Looking at survival time based on diagnosis, it is notable that 82.7% of individuals survive longer than 5 years when the diagnosis is made in ages 50-60 when compared to 68.7% when the diagnosis is made between ages 60-70 (p<0.001).

 

Conclusions

Our data is consistent with the SEER data in that the incidence and prevalence of rectal carcinoid are higher in Black patients compared to White patients. Further analysis into reasons for this racial disparity may prove beneficial to our understanding of this malignancy in the Veteran population. Further research is needed to determine whether diagnosis at a younger age offers a survival advantage in rectal carcinoid.

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Introduction

Rectal carcinoid tumors are rare but the second most common carcinoid in the gastrointestinal tract. They are usually found incidentally during endoscopic or rectal examination. They do not often produce carcinoid syndrome like manifestations although they may manifest as rectal bleeding. Rectal carcinoid patients also have a higher morbidity for other cancers such as stomach, small intestine, or secondary lung cancer.

Methods

We retrospectively explored factors associated with survival in Veterans with rectal carcinoid tumors over a ten-year period from 2007-2017 using the National Veterans Affairs Cancer Cube Registry using specific histological ICD-03 coding. We identified 1110 cases of rectal carcinoid. Chi-squared tests were used for statistical analysis.

Results

Regarding age distribution in our cohort, there were 2.61% of patients ages 40-50 group, 14.0% in the 50-60 age group, 41.5% in the 60-70 age group, and 40.7% above ages 70. There was a higher proportion of rectal cancer in stage 1 compared to other stages (86.3%). The majority of diagnoses occur after age 50 (89.8%). A higher proportion of rectal carcinoid was identified in the 60-70 years category compared to <60 and >70 years old. In the general VA population, there are 80.2% White and 12.8% Black patients. We found a higher proportion of rectal carcinoid in Black patients (47.8%) over White patients (42.8%, p=0.02), which differs significantly from the racial makeup of the VA population (12.8% Black vs 80.3% White). Looking at survival time based on diagnosis, it is notable that 82.7% of individuals survive longer than 5 years when the diagnosis is made in ages 50-60 when compared to 68.7% when the diagnosis is made between ages 60-70 (p<0.001).

 

Conclusions

Our data is consistent with the SEER data in that the incidence and prevalence of rectal carcinoid are higher in Black patients compared to White patients. Further analysis into reasons for this racial disparity may prove beneficial to our understanding of this malignancy in the Veteran population. Further research is needed to determine whether diagnosis at a younger age offers a survival advantage in rectal carcinoid.

Introduction

Rectal carcinoid tumors are rare but the second most common carcinoid in the gastrointestinal tract. They are usually found incidentally during endoscopic or rectal examination. They do not often produce carcinoid syndrome like manifestations although they may manifest as rectal bleeding. Rectal carcinoid patients also have a higher morbidity for other cancers such as stomach, small intestine, or secondary lung cancer.

Methods

We retrospectively explored factors associated with survival in Veterans with rectal carcinoid tumors over a ten-year period from 2007-2017 using the National Veterans Affairs Cancer Cube Registry using specific histological ICD-03 coding. We identified 1110 cases of rectal carcinoid. Chi-squared tests were used for statistical analysis.

Results

Regarding age distribution in our cohort, there were 2.61% of patients ages 40-50 group, 14.0% in the 50-60 age group, 41.5% in the 60-70 age group, and 40.7% above ages 70. There was a higher proportion of rectal cancer in stage 1 compared to other stages (86.3%). The majority of diagnoses occur after age 50 (89.8%). A higher proportion of rectal carcinoid was identified in the 60-70 years category compared to <60 and >70 years old. In the general VA population, there are 80.2% White and 12.8% Black patients. We found a higher proportion of rectal carcinoid in Black patients (47.8%) over White patients (42.8%, p=0.02), which differs significantly from the racial makeup of the VA population (12.8% Black vs 80.3% White). Looking at survival time based on diagnosis, it is notable that 82.7% of individuals survive longer than 5 years when the diagnosis is made in ages 50-60 when compared to 68.7% when the diagnosis is made between ages 60-70 (p<0.001).

 

Conclusions

Our data is consistent with the SEER data in that the incidence and prevalence of rectal carcinoid are higher in Black patients compared to White patients. Further analysis into reasons for this racial disparity may prove beneficial to our understanding of this malignancy in the Veteran population. Further research is needed to determine whether diagnosis at a younger age offers a survival advantage in rectal carcinoid.

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Double Hit: Epstein-Barr Virus Causing Infectious Mononucleosis Followed by Hemolytic Uremic Syndrome

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Introduction

Epstein-Barr virus (EBV) is a herpes virus that commonly causes infectious mononucleosis (IM) and linked to different hematological conditions. Here we present a case of EBV-triggered Hemolytic Uremic Syndrome (HUS) with pulmonary involvement.

Case Presentation

A 20-year-old male presented with fever, thrombocytopenia, and splenomegaly. Acute EBV serology was positive. Creatinine and hemoglobin were normal. He was diagnosed with IM. platelet count improved within 3 weeks. 4 weeks later, he returned with severe hemoptysis. Hgb 6.8g/dL, platelet 133,000/uL, lactate dehydrogenase 969u/L, creatinine 21mg/dL, and schistocytes on peripheral smear. Chest computed tomography showed bilateral opacities consistent with diffuse alveolar hemorrhage (DAH). Emergent hemodialysis and plasmapheresis were started. Infectious work up was negative. Autoimmune work up was also negative (anti-neutrophil cytoplasmic, anti-basement membrane antibodies, ANA). Aadamts13 activity was 62% (normal ~66%) ruling out thrombotic thrombocytopenic purpura (TTP). Kidney biopsy revealed thrombotic microangiopathic process. The patient was eventually diagnosed with HUS and treated with Eculizumab. 4 months later his renal function has partially recovered and no longer needs hemodialysis.

Discussion

HUS is a rare entity that is known to be triggered by different underlying pathologies. However, its link to EBV remains unclear. Literature review has revealed only two cases of EBV-triggered HUS, even though almost 90-95% of adults are EBV-seropositive. What unique about our case is the patient initially presented with documented IM, and HUS happened a month later. This raises the theory that HUS could be a sequela of the infection, rather than an effect of acute viral phase and this is the first case to report such correlation. The other unique thing is pulmonary involvement in HUS. With consultation with pulmonary service, we believe our patient had DAH based on clinical and radiographic findings. To our knowledge this is the first case to show this association.

 

Conclusion

EBV is a common virus with high seropositivity among world’s population. Its link to HUS remains unclear and needs more investigation. Providers should recognize HUS as a complication of EBV infection, either in the acute phase or as a sequela. Adolescents are at higher risk for such complication since IM is common in this population.

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Introduction

Epstein-Barr virus (EBV) is a herpes virus that commonly causes infectious mononucleosis (IM) and linked to different hematological conditions. Here we present a case of EBV-triggered Hemolytic Uremic Syndrome (HUS) with pulmonary involvement.

Case Presentation

A 20-year-old male presented with fever, thrombocytopenia, and splenomegaly. Acute EBV serology was positive. Creatinine and hemoglobin were normal. He was diagnosed with IM. platelet count improved within 3 weeks. 4 weeks later, he returned with severe hemoptysis. Hgb 6.8g/dL, platelet 133,000/uL, lactate dehydrogenase 969u/L, creatinine 21mg/dL, and schistocytes on peripheral smear. Chest computed tomography showed bilateral opacities consistent with diffuse alveolar hemorrhage (DAH). Emergent hemodialysis and plasmapheresis were started. Infectious work up was negative. Autoimmune work up was also negative (anti-neutrophil cytoplasmic, anti-basement membrane antibodies, ANA). Aadamts13 activity was 62% (normal ~66%) ruling out thrombotic thrombocytopenic purpura (TTP). Kidney biopsy revealed thrombotic microangiopathic process. The patient was eventually diagnosed with HUS and treated with Eculizumab. 4 months later his renal function has partially recovered and no longer needs hemodialysis.

Discussion

HUS is a rare entity that is known to be triggered by different underlying pathologies. However, its link to EBV remains unclear. Literature review has revealed only two cases of EBV-triggered HUS, even though almost 90-95% of adults are EBV-seropositive. What unique about our case is the patient initially presented with documented IM, and HUS happened a month later. This raises the theory that HUS could be a sequela of the infection, rather than an effect of acute viral phase and this is the first case to report such correlation. The other unique thing is pulmonary involvement in HUS. With consultation with pulmonary service, we believe our patient had DAH based on clinical and radiographic findings. To our knowledge this is the first case to show this association.

 

Conclusion

EBV is a common virus with high seropositivity among world’s population. Its link to HUS remains unclear and needs more investigation. Providers should recognize HUS as a complication of EBV infection, either in the acute phase or as a sequela. Adolescents are at higher risk for such complication since IM is common in this population.

Introduction

Epstein-Barr virus (EBV) is a herpes virus that commonly causes infectious mononucleosis (IM) and linked to different hematological conditions. Here we present a case of EBV-triggered Hemolytic Uremic Syndrome (HUS) with pulmonary involvement.

Case Presentation

A 20-year-old male presented with fever, thrombocytopenia, and splenomegaly. Acute EBV serology was positive. Creatinine and hemoglobin were normal. He was diagnosed with IM. platelet count improved within 3 weeks. 4 weeks later, he returned with severe hemoptysis. Hgb 6.8g/dL, platelet 133,000/uL, lactate dehydrogenase 969u/L, creatinine 21mg/dL, and schistocytes on peripheral smear. Chest computed tomography showed bilateral opacities consistent with diffuse alveolar hemorrhage (DAH). Emergent hemodialysis and plasmapheresis were started. Infectious work up was negative. Autoimmune work up was also negative (anti-neutrophil cytoplasmic, anti-basement membrane antibodies, ANA). Aadamts13 activity was 62% (normal ~66%) ruling out thrombotic thrombocytopenic purpura (TTP). Kidney biopsy revealed thrombotic microangiopathic process. The patient was eventually diagnosed with HUS and treated with Eculizumab. 4 months later his renal function has partially recovered and no longer needs hemodialysis.

Discussion

HUS is a rare entity that is known to be triggered by different underlying pathologies. However, its link to EBV remains unclear. Literature review has revealed only two cases of EBV-triggered HUS, even though almost 90-95% of adults are EBV-seropositive. What unique about our case is the patient initially presented with documented IM, and HUS happened a month later. This raises the theory that HUS could be a sequela of the infection, rather than an effect of acute viral phase and this is the first case to report such correlation. The other unique thing is pulmonary involvement in HUS. With consultation with pulmonary service, we believe our patient had DAH based on clinical and radiographic findings. To our knowledge this is the first case to show this association.

 

Conclusion

EBV is a common virus with high seropositivity among world’s population. Its link to HUS remains unclear and needs more investigation. Providers should recognize HUS as a complication of EBV infection, either in the acute phase or as a sequela. Adolescents are at higher risk for such complication since IM is common in this population.

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Survival Analysis of Untreated Early-Stage Non-Small Cell Lung Cancer (NSCLC) in a Veteran Population

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Introduction

Veterans with early-stage NSCLC who do not receive any form of treatment have been shown to have a worse overall survival compared to those who receive treatment. Factors that may influence the decision to administer treatment including age, performance status (PS), comorbidities, and racial disparity have not been assessed on a national level in recent years.

Methods

Data for 31,966 veterans diagnosed with early-stage (0, I) NSCLC between 2003-2017 was obtained from the Cancer cube registry (VACCR). IRB approval was obtained.

Results

Patients were divided into treatment (26,833/31,966, 83.16%) and no-treatment group (3096/31966, 9.68%). Of the no-treatment group, 3004 patients were stage I and 92 were stage 0 whereas in the treatment group, the distribution was 26,584 and 249 respectively. Gender, race, and histology distribution were comparable between the two. Patients with poor PS (defined as ECOG III and IV) received less treatment with any modality compared to those with good PS (ECOG I and II) (15.07% in no treatment group vs 4.03% in treatment group, p<0.05). The treatment group had a better 5-year overall survival (OS) as compared to no-treatment group (43.1% vs 14.7%, p<0.05). Regardless of treatment, patients above the age of 60 (41% vs 13.4%, p<0.05) and those with poor PS (19.6% vs 5.8%, p<0.05) had worse 5-year survival, with the effect being greater in the treatment group. Adenocarcinoma had a better 5-year survival compared to squamous cell carcinoma (SCC) in both groups (49.56% vs 39.1% p<0.05). There was no clinically significant OS difference in terms of race (Caucasian or African American) or tumor location (upper, middle, or lower lobe) in between the two groups. Our study was limited by lack of patient- level data including smoking status or reason why no treatment was given.

 

Conclusion

Patients with early-stage NSCLC who receive no treatment based on poor PS have a worse overall survival compared to the patients that receive treatment. Further investigation is required to assess what other criteria are used to decide treatment eligibility and whether these patients would be candidates for immunotherapy or targeted therapy in the future.

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Introduction

Veterans with early-stage NSCLC who do not receive any form of treatment have been shown to have a worse overall survival compared to those who receive treatment. Factors that may influence the decision to administer treatment including age, performance status (PS), comorbidities, and racial disparity have not been assessed on a national level in recent years.

Methods

Data for 31,966 veterans diagnosed with early-stage (0, I) NSCLC between 2003-2017 was obtained from the Cancer cube registry (VACCR). IRB approval was obtained.

Results

Patients were divided into treatment (26,833/31,966, 83.16%) and no-treatment group (3096/31966, 9.68%). Of the no-treatment group, 3004 patients were stage I and 92 were stage 0 whereas in the treatment group, the distribution was 26,584 and 249 respectively. Gender, race, and histology distribution were comparable between the two. Patients with poor PS (defined as ECOG III and IV) received less treatment with any modality compared to those with good PS (ECOG I and II) (15.07% in no treatment group vs 4.03% in treatment group, p<0.05). The treatment group had a better 5-year overall survival (OS) as compared to no-treatment group (43.1% vs 14.7%, p<0.05). Regardless of treatment, patients above the age of 60 (41% vs 13.4%, p<0.05) and those with poor PS (19.6% vs 5.8%, p<0.05) had worse 5-year survival, with the effect being greater in the treatment group. Adenocarcinoma had a better 5-year survival compared to squamous cell carcinoma (SCC) in both groups (49.56% vs 39.1% p<0.05). There was no clinically significant OS difference in terms of race (Caucasian or African American) or tumor location (upper, middle, or lower lobe) in between the two groups. Our study was limited by lack of patient- level data including smoking status or reason why no treatment was given.

 

Conclusion

Patients with early-stage NSCLC who receive no treatment based on poor PS have a worse overall survival compared to the patients that receive treatment. Further investigation is required to assess what other criteria are used to decide treatment eligibility and whether these patients would be candidates for immunotherapy or targeted therapy in the future.

Introduction

Veterans with early-stage NSCLC who do not receive any form of treatment have been shown to have a worse overall survival compared to those who receive treatment. Factors that may influence the decision to administer treatment including age, performance status (PS), comorbidities, and racial disparity have not been assessed on a national level in recent years.

Methods

Data for 31,966 veterans diagnosed with early-stage (0, I) NSCLC between 2003-2017 was obtained from the Cancer cube registry (VACCR). IRB approval was obtained.

Results

Patients were divided into treatment (26,833/31,966, 83.16%) and no-treatment group (3096/31966, 9.68%). Of the no-treatment group, 3004 patients were stage I and 92 were stage 0 whereas in the treatment group, the distribution was 26,584 and 249 respectively. Gender, race, and histology distribution were comparable between the two. Patients with poor PS (defined as ECOG III and IV) received less treatment with any modality compared to those with good PS (ECOG I and II) (15.07% in no treatment group vs 4.03% in treatment group, p<0.05). The treatment group had a better 5-year overall survival (OS) as compared to no-treatment group (43.1% vs 14.7%, p<0.05). Regardless of treatment, patients above the age of 60 (41% vs 13.4%, p<0.05) and those with poor PS (19.6% vs 5.8%, p<0.05) had worse 5-year survival, with the effect being greater in the treatment group. Adenocarcinoma had a better 5-year survival compared to squamous cell carcinoma (SCC) in both groups (49.56% vs 39.1% p<0.05). There was no clinically significant OS difference in terms of race (Caucasian or African American) or tumor location (upper, middle, or lower lobe) in between the two groups. Our study was limited by lack of patient- level data including smoking status or reason why no treatment was given.

 

Conclusion

Patients with early-stage NSCLC who receive no treatment based on poor PS have a worse overall survival compared to the patients that receive treatment. Further investigation is required to assess what other criteria are used to decide treatment eligibility and whether these patients would be candidates for immunotherapy or targeted therapy in the future.

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Cerebral Venous Thrombosis, an Extremely Rare Complication of Iron Deficiency Anemia

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INTRODUCTION: Cerebral venous thrombosis (CVT) is a rare type of stroke and can be challenging to diagnose. It is seen in most commonly young females and has been linked to thrombophilia, pregnancy, and contraceptive pills. Here we present a rare case of CVT in a young female with iron deficiency anemia.

CASE REPORT: A 19-year-old female patient presented with severe headache, CT scan of the head on admission showed acute superior sagittal sinus thrombosis which was confirmed with CT venogram and MRI of the brain. The patient had intact neurologic exam upon admission. She was started on heparin and admitted for monitoring. Later on she developed expressive aphasia and right sided weakness. She ultimately underwent catheter directed thrombolysis. Follow up CT and MRI scans showed significant decrease in clot burden, and the patient’s neurologic function started to improve.

Her initial labs were significant for thrombocytosis with platelet count 840,000/μL, and microcytic anemia with hemoglobin 9.6 g/dL and MCV 79 fL. She had low serum ferritin and iron levels with high total iron binding capacity consistent with iron deficiency anemia. An extensive hypercoagulable work up was done including antithrombin, protein C and S, factor V Leiden mutation, prothrombin gene mutation, hyperhomocysteinemia, antiphospholipid antibodies, anti-nuclear antibodies which all came back negative. Given her high platelet count, a myeloproliferative disorder was entertained however testing of mutations JAK2V617F, CALR, MPL, and BCR-ABL was negative. She also had a bone marrow biopsy that revealed normal bone marrow. The patient had no prior personal or family history of venous thrombosis, she was not taking any hormonal mediation and pregnancy test was negative. She did report menorrhagia for couple of months prior to admission.

CONCLUSION: After ruling out genetic prothrombotic states, autoimmune disease, and bone marrow disorders. We determined this was a case of cerebral venous thrombosis secondary to reactive thrombocytosis in setting of untreated iron deficiency and menorrhagia. The patient was started on iron supplements with improvement in her iron and hemoglobin levels, and subsequent decrease in her platelet count to normal values. She continued anticoagulation with rivaroxaban for 3-6 months period.

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INTRODUCTION: Cerebral venous thrombosis (CVT) is a rare type of stroke and can be challenging to diagnose. It is seen in most commonly young females and has been linked to thrombophilia, pregnancy, and contraceptive pills. Here we present a rare case of CVT in a young female with iron deficiency anemia.

CASE REPORT: A 19-year-old female patient presented with severe headache, CT scan of the head on admission showed acute superior sagittal sinus thrombosis which was confirmed with CT venogram and MRI of the brain. The patient had intact neurologic exam upon admission. She was started on heparin and admitted for monitoring. Later on she developed expressive aphasia and right sided weakness. She ultimately underwent catheter directed thrombolysis. Follow up CT and MRI scans showed significant decrease in clot burden, and the patient’s neurologic function started to improve.

Her initial labs were significant for thrombocytosis with platelet count 840,000/μL, and microcytic anemia with hemoglobin 9.6 g/dL and MCV 79 fL. She had low serum ferritin and iron levels with high total iron binding capacity consistent with iron deficiency anemia. An extensive hypercoagulable work up was done including antithrombin, protein C and S, factor V Leiden mutation, prothrombin gene mutation, hyperhomocysteinemia, antiphospholipid antibodies, anti-nuclear antibodies which all came back negative. Given her high platelet count, a myeloproliferative disorder was entertained however testing of mutations JAK2V617F, CALR, MPL, and BCR-ABL was negative. She also had a bone marrow biopsy that revealed normal bone marrow. The patient had no prior personal or family history of venous thrombosis, she was not taking any hormonal mediation and pregnancy test was negative. She did report menorrhagia for couple of months prior to admission.

CONCLUSION: After ruling out genetic prothrombotic states, autoimmune disease, and bone marrow disorders. We determined this was a case of cerebral venous thrombosis secondary to reactive thrombocytosis in setting of untreated iron deficiency and menorrhagia. The patient was started on iron supplements with improvement in her iron and hemoglobin levels, and subsequent decrease in her platelet count to normal values. She continued anticoagulation with rivaroxaban for 3-6 months period.

INTRODUCTION: Cerebral venous thrombosis (CVT) is a rare type of stroke and can be challenging to diagnose. It is seen in most commonly young females and has been linked to thrombophilia, pregnancy, and contraceptive pills. Here we present a rare case of CVT in a young female with iron deficiency anemia.

CASE REPORT: A 19-year-old female patient presented with severe headache, CT scan of the head on admission showed acute superior sagittal sinus thrombosis which was confirmed with CT venogram and MRI of the brain. The patient had intact neurologic exam upon admission. She was started on heparin and admitted for monitoring. Later on she developed expressive aphasia and right sided weakness. She ultimately underwent catheter directed thrombolysis. Follow up CT and MRI scans showed significant decrease in clot burden, and the patient’s neurologic function started to improve.

Her initial labs were significant for thrombocytosis with platelet count 840,000/μL, and microcytic anemia with hemoglobin 9.6 g/dL and MCV 79 fL. She had low serum ferritin and iron levels with high total iron binding capacity consistent with iron deficiency anemia. An extensive hypercoagulable work up was done including antithrombin, protein C and S, factor V Leiden mutation, prothrombin gene mutation, hyperhomocysteinemia, antiphospholipid antibodies, anti-nuclear antibodies which all came back negative. Given her high platelet count, a myeloproliferative disorder was entertained however testing of mutations JAK2V617F, CALR, MPL, and BCR-ABL was negative. She also had a bone marrow biopsy that revealed normal bone marrow. The patient had no prior personal or family history of venous thrombosis, she was not taking any hormonal mediation and pregnancy test was negative. She did report menorrhagia for couple of months prior to admission.

CONCLUSION: After ruling out genetic prothrombotic states, autoimmune disease, and bone marrow disorders. We determined this was a case of cerebral venous thrombosis secondary to reactive thrombocytosis in setting of untreated iron deficiency and menorrhagia. The patient was started on iron supplements with improvement in her iron and hemoglobin levels, and subsequent decrease in her platelet count to normal values. She continued anticoagulation with rivaroxaban for 3-6 months period.

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