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Although it may seem that veterans would have a very low risk of bleeding disorders since they were medically cleared for military service, a hematologist/oncologist cautioned that veterans might indeed suffer from both inherited and noninherited forms of these conditions. At the virtual 2020 annual meeting of the Association of VA Hematology/Oncology (AVAHO) Bethany Samuelson Bannow, MD, of Oregon Health & Science University’s Knight Cancer Institute urged colleagues to understand the diagnosis and treatment of bleeding disorders.
“Most importantly, even though these are patients you probably don’t see on a regular basis, you are never alone,” since colleagues are available to help, she said. Samuelson Bannow treats patients at US Department of Veterans Affairs (VA) Portland Health Care System and used her presentation to focus on 4 types of bleeding disorders. A summary of her perspective and recommendations follows.
Acquired hemophilia: Watch for Infections
Acquired hemophilia affects only an estimated 1.3 to 1.5 in 1 million people, but VA physicians may see it more often since it affects an older population (median age is 78 years), Samuelson Bannow said. “I’ve seen about 4 cases in the last 2 years,” she said. “I’m not sure if we’re a magnet, but it does come up.”
The diagnosis is based on laboratory findings, and a lack of personal or family history of coagulopathy is key, she said. Twenty percent or more of patients older than 65 years die from the disorder, but bleeding usually isn’t the cause. Instead, patients tend to die from infections, she said.
Initial treatment must focus on stopping the bleeding, she said. The new drug porcine antihemophilic factor (recombinant)—Obizur—“is very helpful” and is Samuelson Bannow’s first choice, but it may not widely available at all VA medical centers. Recombinant FVIIa (NovoSeven) and activated prothrombin complex concentrate (Feiba) also are options.
“The goal is to overpower the clotting cascade and get that burst of thrombin generation that you need to get the bleeding under control. Titrate to the amount of bleeding the patient is having, and make sure you’re doing local control as well,” Samuelson Bannow said. She added that the 2 agents may not work depending on the patient. Neither is preferred and both may be appropriate. “There’s no real reason to pick one over the other beyond convenience and availability.”
There’s another “equally important component of management,” she said: Inhibitor eradication. “The only way to do this is with immune suppression. You’re going to have to suppress the immune system to get rid of the inhibitor. That’s why we see such high rates of death because we have to use heavy-hitter immunosuppressants.”
Treatment options include steroids and cyclophosphamide (a common first-line option), rituximab, calcineurin inhibitors, and mycophenolate mofetil. “Just be aware that there is an increased risk of infection with these agents,” she said. “You want to see a decrease in the titer of your inhibitor. This can take 3 or more weeks, and it can take longer for it to disappear entirely. Look for normalized factor VIII level and absent inhibitor.”
She added: “I tend to do a slow taper, one agent at a time, over the course of weeks. If you see a return of the inhibitor, you can ramp back up as needed. Continue to monitor for a year or more since patients are at high risk of recurrence.”
Acquired von Willebrand Syndrome: Fix the Associated Conditions
Acquired von Willebrand Syndrome is another rare bleeding disorder that may appear in veterans “since it’s associated with a lot of conditions that we see in the VA,” such as heart disorders, solid tumors, vascular malformations, and lymphoproliferative and myeloproliferative disorders, Samuelson Bannow said.
As with acquired hemophilia, the key is to focus on controlling the bleeding, she said. Then, she advised, focus on the associated condition: “Correct the underlying disorder: Treat the malignancy, address the [cardiac] shear stress, correct hypothyroidism, correct the cardiac defects.”
Inherited Hemophilia: Don’t Rule It Out
It’s possible to “sneak through” military medical screening with undiagnosed inherited bleeding disorders, Dr. Samuelson Bannow said. That’s because service members may have never had an injury that triggered abnormal bleeding, she said. “You can see how someone could slip into the military with a [low clotting factor level]. The classic presentation is going to be joint bleeds and abnormalities. These can be traumatic and spontaneous without any kind of an injury,” she said.
A general rule for these patients is to “replace what’s missing, and 100% is always normal.” The 100% refers to clotting factor level. She recommended reviewing 2013 guidelines for the treatment of the disorder.
Inherited von Willebrand Syndrome: Ask About Tonsillectomies
Inherited von Willebrand Syndrome is more common in women than it is in men. Bleeding “will primarily be mucocutaneous and trauma-induced,” said. Samuelson Bannow. She recommended asking patients if they have had a tonsillectomy and, if so, did they suffer from unusual bleeding. “If they did not have excessive bleeding, it’s a pretty good sign their hemostasis is good.”
She recommends a nasal spray drug called desmopressin (DDAVP, Stimate) for management. “It’s critical to know that you must test for efficacy first,” she said. “One of the most common mistakes that people make is that they may give patients DDAVP before surgery without knowing if it works or not. It doesn’t work for every patient.” She typically avoids this drug in patients aged > 65 years, or even > 60 years, due to increased thrombosis risk.
Recombinant von Willebrand factor (Vonvendi) is a “very helpful drug at patients at increased risk of thrombosis,” she said. However, she noted that the drug, at last check, is not on the VA’s formulary.
At the end of her presentation, Samuelson Bannow urged colleagues to contact specialized Hemophilia Treatment Centers (HTCs) near them: “Follow this link, find an HTC [Hemophilia Treatment Center] near you, and create a partnership.”
Samuelson Bannow reported no relevant disclosures.
Although it may seem that veterans would have a very low risk of bleeding disorders since they were medically cleared for military service, a hematologist/oncologist cautioned that veterans might indeed suffer from both inherited and noninherited forms of these conditions. At the virtual 2020 annual meeting of the Association of VA Hematology/Oncology (AVAHO) Bethany Samuelson Bannow, MD, of Oregon Health & Science University’s Knight Cancer Institute urged colleagues to understand the diagnosis and treatment of bleeding disorders.
“Most importantly, even though these are patients you probably don’t see on a regular basis, you are never alone,” since colleagues are available to help, she said. Samuelson Bannow treats patients at US Department of Veterans Affairs (VA) Portland Health Care System and used her presentation to focus on 4 types of bleeding disorders. A summary of her perspective and recommendations follows.
Acquired hemophilia: Watch for Infections
Acquired hemophilia affects only an estimated 1.3 to 1.5 in 1 million people, but VA physicians may see it more often since it affects an older population (median age is 78 years), Samuelson Bannow said. “I’ve seen about 4 cases in the last 2 years,” she said. “I’m not sure if we’re a magnet, but it does come up.”
The diagnosis is based on laboratory findings, and a lack of personal or family history of coagulopathy is key, she said. Twenty percent or more of patients older than 65 years die from the disorder, but bleeding usually isn’t the cause. Instead, patients tend to die from infections, she said.
Initial treatment must focus on stopping the bleeding, she said. The new drug porcine antihemophilic factor (recombinant)—Obizur—“is very helpful” and is Samuelson Bannow’s first choice, but it may not widely available at all VA medical centers. Recombinant FVIIa (NovoSeven) and activated prothrombin complex concentrate (Feiba) also are options.
“The goal is to overpower the clotting cascade and get that burst of thrombin generation that you need to get the bleeding under control. Titrate to the amount of bleeding the patient is having, and make sure you’re doing local control as well,” Samuelson Bannow said. She added that the 2 agents may not work depending on the patient. Neither is preferred and both may be appropriate. “There’s no real reason to pick one over the other beyond convenience and availability.”
There’s another “equally important component of management,” she said: Inhibitor eradication. “The only way to do this is with immune suppression. You’re going to have to suppress the immune system to get rid of the inhibitor. That’s why we see such high rates of death because we have to use heavy-hitter immunosuppressants.”
Treatment options include steroids and cyclophosphamide (a common first-line option), rituximab, calcineurin inhibitors, and mycophenolate mofetil. “Just be aware that there is an increased risk of infection with these agents,” she said. “You want to see a decrease in the titer of your inhibitor. This can take 3 or more weeks, and it can take longer for it to disappear entirely. Look for normalized factor VIII level and absent inhibitor.”
She added: “I tend to do a slow taper, one agent at a time, over the course of weeks. If you see a return of the inhibitor, you can ramp back up as needed. Continue to monitor for a year or more since patients are at high risk of recurrence.”
Acquired von Willebrand Syndrome: Fix the Associated Conditions
Acquired von Willebrand Syndrome is another rare bleeding disorder that may appear in veterans “since it’s associated with a lot of conditions that we see in the VA,” such as heart disorders, solid tumors, vascular malformations, and lymphoproliferative and myeloproliferative disorders, Samuelson Bannow said.
As with acquired hemophilia, the key is to focus on controlling the bleeding, she said. Then, she advised, focus on the associated condition: “Correct the underlying disorder: Treat the malignancy, address the [cardiac] shear stress, correct hypothyroidism, correct the cardiac defects.”
Inherited Hemophilia: Don’t Rule It Out
It’s possible to “sneak through” military medical screening with undiagnosed inherited bleeding disorders, Dr. Samuelson Bannow said. That’s because service members may have never had an injury that triggered abnormal bleeding, she said. “You can see how someone could slip into the military with a [low clotting factor level]. The classic presentation is going to be joint bleeds and abnormalities. These can be traumatic and spontaneous without any kind of an injury,” she said.
A general rule for these patients is to “replace what’s missing, and 100% is always normal.” The 100% refers to clotting factor level. She recommended reviewing 2013 guidelines for the treatment of the disorder.
Inherited von Willebrand Syndrome: Ask About Tonsillectomies
Inherited von Willebrand Syndrome is more common in women than it is in men. Bleeding “will primarily be mucocutaneous and trauma-induced,” said. Samuelson Bannow. She recommended asking patients if they have had a tonsillectomy and, if so, did they suffer from unusual bleeding. “If they did not have excessive bleeding, it’s a pretty good sign their hemostasis is good.”
She recommends a nasal spray drug called desmopressin (DDAVP, Stimate) for management. “It’s critical to know that you must test for efficacy first,” she said. “One of the most common mistakes that people make is that they may give patients DDAVP before surgery without knowing if it works or not. It doesn’t work for every patient.” She typically avoids this drug in patients aged > 65 years, or even > 60 years, due to increased thrombosis risk.
Recombinant von Willebrand factor (Vonvendi) is a “very helpful drug at patients at increased risk of thrombosis,” she said. However, she noted that the drug, at last check, is not on the VA’s formulary.
At the end of her presentation, Samuelson Bannow urged colleagues to contact specialized Hemophilia Treatment Centers (HTCs) near them: “Follow this link, find an HTC [Hemophilia Treatment Center] near you, and create a partnership.”
Samuelson Bannow reported no relevant disclosures.
Although it may seem that veterans would have a very low risk of bleeding disorders since they were medically cleared for military service, a hematologist/oncologist cautioned that veterans might indeed suffer from both inherited and noninherited forms of these conditions. At the virtual 2020 annual meeting of the Association of VA Hematology/Oncology (AVAHO) Bethany Samuelson Bannow, MD, of Oregon Health & Science University’s Knight Cancer Institute urged colleagues to understand the diagnosis and treatment of bleeding disorders.
“Most importantly, even though these are patients you probably don’t see on a regular basis, you are never alone,” since colleagues are available to help, she said. Samuelson Bannow treats patients at US Department of Veterans Affairs (VA) Portland Health Care System and used her presentation to focus on 4 types of bleeding disorders. A summary of her perspective and recommendations follows.
Acquired hemophilia: Watch for Infections
Acquired hemophilia affects only an estimated 1.3 to 1.5 in 1 million people, but VA physicians may see it more often since it affects an older population (median age is 78 years), Samuelson Bannow said. “I’ve seen about 4 cases in the last 2 years,” she said. “I’m not sure if we’re a magnet, but it does come up.”
The diagnosis is based on laboratory findings, and a lack of personal or family history of coagulopathy is key, she said. Twenty percent or more of patients older than 65 years die from the disorder, but bleeding usually isn’t the cause. Instead, patients tend to die from infections, she said.
Initial treatment must focus on stopping the bleeding, she said. The new drug porcine antihemophilic factor (recombinant)—Obizur—“is very helpful” and is Samuelson Bannow’s first choice, but it may not widely available at all VA medical centers. Recombinant FVIIa (NovoSeven) and activated prothrombin complex concentrate (Feiba) also are options.
“The goal is to overpower the clotting cascade and get that burst of thrombin generation that you need to get the bleeding under control. Titrate to the amount of bleeding the patient is having, and make sure you’re doing local control as well,” Samuelson Bannow said. She added that the 2 agents may not work depending on the patient. Neither is preferred and both may be appropriate. “There’s no real reason to pick one over the other beyond convenience and availability.”
There’s another “equally important component of management,” she said: Inhibitor eradication. “The only way to do this is with immune suppression. You’re going to have to suppress the immune system to get rid of the inhibitor. That’s why we see such high rates of death because we have to use heavy-hitter immunosuppressants.”
Treatment options include steroids and cyclophosphamide (a common first-line option), rituximab, calcineurin inhibitors, and mycophenolate mofetil. “Just be aware that there is an increased risk of infection with these agents,” she said. “You want to see a decrease in the titer of your inhibitor. This can take 3 or more weeks, and it can take longer for it to disappear entirely. Look for normalized factor VIII level and absent inhibitor.”
She added: “I tend to do a slow taper, one agent at a time, over the course of weeks. If you see a return of the inhibitor, you can ramp back up as needed. Continue to monitor for a year or more since patients are at high risk of recurrence.”
Acquired von Willebrand Syndrome: Fix the Associated Conditions
Acquired von Willebrand Syndrome is another rare bleeding disorder that may appear in veterans “since it’s associated with a lot of conditions that we see in the VA,” such as heart disorders, solid tumors, vascular malformations, and lymphoproliferative and myeloproliferative disorders, Samuelson Bannow said.
As with acquired hemophilia, the key is to focus on controlling the bleeding, she said. Then, she advised, focus on the associated condition: “Correct the underlying disorder: Treat the malignancy, address the [cardiac] shear stress, correct hypothyroidism, correct the cardiac defects.”
Inherited Hemophilia: Don’t Rule It Out
It’s possible to “sneak through” military medical screening with undiagnosed inherited bleeding disorders, Dr. Samuelson Bannow said. That’s because service members may have never had an injury that triggered abnormal bleeding, she said. “You can see how someone could slip into the military with a [low clotting factor level]. The classic presentation is going to be joint bleeds and abnormalities. These can be traumatic and spontaneous without any kind of an injury,” she said.
A general rule for these patients is to “replace what’s missing, and 100% is always normal.” The 100% refers to clotting factor level. She recommended reviewing 2013 guidelines for the treatment of the disorder.
Inherited von Willebrand Syndrome: Ask About Tonsillectomies
Inherited von Willebrand Syndrome is more common in women than it is in men. Bleeding “will primarily be mucocutaneous and trauma-induced,” said. Samuelson Bannow. She recommended asking patients if they have had a tonsillectomy and, if so, did they suffer from unusual bleeding. “If they did not have excessive bleeding, it’s a pretty good sign their hemostasis is good.”
She recommends a nasal spray drug called desmopressin (DDAVP, Stimate) for management. “It’s critical to know that you must test for efficacy first,” she said. “One of the most common mistakes that people make is that they may give patients DDAVP before surgery without knowing if it works or not. It doesn’t work for every patient.” She typically avoids this drug in patients aged > 65 years, or even > 60 years, due to increased thrombosis risk.
Recombinant von Willebrand factor (Vonvendi) is a “very helpful drug at patients at increased risk of thrombosis,” she said. However, she noted that the drug, at last check, is not on the VA’s formulary.
At the end of her presentation, Samuelson Bannow urged colleagues to contact specialized Hemophilia Treatment Centers (HTCs) near them: “Follow this link, find an HTC [Hemophilia Treatment Center] near you, and create a partnership.”
Samuelson Bannow reported no relevant disclosures.