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Resident PGY 1
Henry Ford Macomb
Acquired Hemophilia A manifests as an autoimmune condition characterized by spontaneous synthesis of IgG against Factor VIII (FVIII) occurring in 1 in 1,500,000 cases annually with an approximate 9-22% mortality1,2.A 72-year-old-male with recent hospitalization from ESBL UTI and DVT, returned to the hospital with weakness, fatigue, exertional dyspnea and diffuse ecchymosis while on Xarelto. Admission labs revealed an elevated PTT without prior personal or family history of coagulopathy. Subsequent testing exhibited non-corrective PTT mixing study of 199s, preliminary FVIII activity of 0.26% and FVIII inhibitor level of 112.0 Bestheda units (BU).Patient was treated with prednisone 1mg/kg and Cyclophosphamide 2mg/kg daily. With minimal change of FVIII activity/inhibitor levels after seven days, cyclophosphamide was subsequently increased to 3mg/kg daily; 800mg Rituximab weekly for four weeks and adjuvant IVIG 1g/kg for two days were added. While FVIII inhibitor levels decreased promptly after giving Rituximab, FVIII activity remained relatively stagnant throughout the treatment course until the inhibitor level breached 10BU. The FVIII inhibitor decreased from 112 to 1.6BU, FVIII activity increased from 0.26% to 17%, and PTT decreased from 199s to 38s over 35 days.Cyclophosphamide and steroids were insufficient, and Rituximab and IVIG were supplemented in the treatment regimen. The patient's levels improved only after the initiation of Rituximab and IVIG. Given this, further research should be conducted in the use of Rituximab and IVIG for the treatment of acquired Hemophilia A with Factor VIII inhibitor.
Siu, Jordan; Peruri, Adithya; Kerndt, Connor; Severson, Dawn; and Patel, Prashant, "Factor VIII Eradication in Acquired Hemophilia A" (2019). Case Reports. 95.