A Two Part Case Series on Acquired Hemophilia A

نویسندگان

چکیده

Abstract Introduction/Objective Acquired Hemophilia A is a rare condition with an incidence of 1 per 1.5 million year. The average age at presentation 74 years. It due to spontaneous production IgG autoantibodies that target endogenous factor viii. This disease should be considered when patient presents bleeding, abnormal prolongation activated partial thromboplastin time and /or no prior personal or family history bleeding. Autoimmune disorders malignancy are common associations. Pregnancy surgery presenting scenarios. Methods/Case Report Case 1: 71 year old female deep venous thrombosis, rheumatoid arthritis. Patient presented the emergency department hemoglobin 4.7 coagulation studies showed prolonged 119 seconds. Imaging revealed retroperitoneal mixing study correction time. was treated packed red blood cell transfusions until hemodynamically stable. Further VIII activity 3L inhibitor level 103 bethesda units. confirmed suspicion acquired associated patient’s arthritis as source lab values recombinant VIIa, novoseven, along continuous prednisone cyclophosphamide. 2: 82 male Kappa Monoclonal Gammopathy Undetermined Significance. gastrointestinal bleeding 4g/Dl hemoglobin. He received 5 units cells. Sigmoidoscopy rectal ulcer causing lower An advanced 1%, levels 17 started on initially, followed by However, continued. 6 VIIa required keep 7g/Dl. continued increased dosage discharged. Results (if Study enter NA) NA. Conclusion disease. suspected anytime there unprecedented and/or unexplained diagnosed panel, including inhibitor. Treatment includes immunosuppresants, intravenous immunoglobulins, VIIa.

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ژورنال

عنوان ژورنال: American Journal of Clinical Pathology

سال: 2022

ISSN: ['0002-9173', '1943-7722']

DOI: https://doi.org/10.1093/ajcp/aqac126.244