Abstract Number: PB0774
Meeting: ISTH 2020 Congress
Background: Acquired haemophilia A (AHA) is a bleeding disorder characterized by autoantibodies against factor VIII. Half of patients present an underlying disease such as autoimmune disorders or cancer, particularly lymphoproliferative malignancies
Aims: To describe a patient with non Hodgkin indolent lymphoma who developed acquired AHA and platelet dysfunction.
Methods: FVIII:C was measured by one-stage clotting assay and inhibitor titer by Nijmegen-modified Bethesda assay. Platelet aggregation and ATP secretion were tested by lumi-light transmission aggregometry.
Results: A 67-year-old man was referred to our Internal Medicine Department for spontaneous bilateral gluteus hematomas and bruising. Laboratory data showed mild Coombs negative hemolytic anemia and prolonged activated partial thromboplastin time (ratio:1.8). Factor VIII:C resulted 10% and inhibitor titer 5 Bethesda units confirming diagnosis of AHA. Prednisone (1 mg/kg) was started. Because of severe bleeding tendency platelet aggregation and secretion were investigated and they were severely impaired. Platelet levels of ADP were reduced with high ATP/ADP ratio, supporting the diagnosis of acquired platelet δ-storage pool defect.
Computed tomography scan documented splenomegaly and bone marrow biopsy revealed a not further characterizable lymphoproliferative B cell disorder CD20+CD5-CD10-.
For bleeding symptoms factor rVIIa was introduced (100 U/Kg every 8 hours) together with rituximab 375 mg/m2 weekly x 4 weeks to treat both AHA and the associated lymphoma. Cyclophosphamide (2 mg/Kg) and tranexamic acid were added. Susoctocog alfa (a recombinant B-domain deleted porcine FVIII) was successfully administered (37 UI/Kg) to treat an episode of severe acute anemization. (Fig.1)
After four months AHA is in remission with progressive reduction of steroid and platelet function has also improved.
Conclusions: This rare and complex case underlines the need of multiple diagnostic and therapeutical approaches to AHA, due to the combination of coagulation and platelet dysfunction. Our case, suggest also the usefulness of Rituximab when used early in AHA secondary to lymphoproliferative disorders.
[The clinical course of patient: therapy and FVIII-related analysis ]
To cite this abstract in AMA style:Rossio R, Cassin R, Lecchi A, Noto A, Novembrino C, Mancuso ME, Ferrari B, Reda G, Peyvandi F. A Rare Case of Acquired Haemophilia A with Platelet Dysfunction Associated with Non Hodgkin Indolent Lymphoma [abstract]. Res Pract Thromb Haemost. 2020; 4 (Suppl 1). https://abstracts.isth.org/abstract/a-rare-case-of-acquired-haemophilia-a-with-platelet-dysfunction-associated-with-non-hodgkin-indolent-lymphoma/. Accessed September 24, 2023.
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