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Topics:
Pediatric Hematology/Oncology • Hemostasis • Hematology • Pediatric Hematology • Benign Hematology • Hemophilia
When do you stop trying to eradicate an acquired factor VIII inhibitor?
Patient with high titer acquired FVIII inhibitor with no causative etiology. Bleeding is well-controlled on emicizumab, but inhibitor has failed to respond to corticosteroids, rituximab, cyclophosphamide, and mycophenolate. With bleeding controlled, she is now developing more problems from immunosuppressive therapy than from the inhibitor.
Answer from: at Community Practice
First, to be clear, the answer to this question is not well studied in any population, and the clinical setting (i.e. age, titer - historical max or current), and the nature of the complications isn't detailed here. I presume "no causative etiology" includes "not postpartum, and not accompanied by (...
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Answer from: Medical Oncologist at Community Practice
Acquired factor VIII inhibitor causes a rare but life-threatening form of bleeding disorder. Treatment modalities have included the use of immunosuppressive drugs such as cyclophosphamide and corticosteroids, plasmapheresis, and I.V. immunoglobulin and rituximab.
I had two adult patients who could ...
Answer from: Medical Oncologist at Community Practice
I agree with Dr. @Neufeld that this is not well studied and that immunosuppressive therapy in an older patient is not always well tolerated. If the patient is not bleeding, then I would get rid of the more toxic immunosuppressive agents at least for a while.
However, if the inhibitor is still...