A rare case of acquired hemophilia A associated with myelodysplastic syndrome

International Journal of Clinical and Experimental Medicine

Mihir Raval¹  Dinesh Bande¹  Rekha Kallamadi¹ 

关键词: Factor VIII;    acquired hemophilia A;    rFVIIa;    bethesda assay;    myelodysplastic syndrome;    aPTT;    factor VIII inhibitor;   

DOI  :  

学科分类：医学（综合）

来源: e-Century Publishing Corporation

【 摘 要 】

84 year old male with past medical history of myelodysplastic syndrome (MDS) presented with progressive subcutaneous and muscle bleed in the right forearm and arm. Workup revealed elevated activated partial thromboplastin time (aPTT) - 71.8 seconds (normal 23 - 32 seconds) which was persistently elevated after mixing study (37.1 seconds immediately and 51.1 seconds after 1 hour). Further laboratory work up revealed low factor VIII level (3%) and elevated factor VIII inhibitor by Bethesda assay (3 units/ml of blood). Acquired hemophilia A (AHA) diagnosis was established and patient was treated with recombinant factor VIIa (rFVlla) to control the bleeding and also prednisone for immunosuppression. Subsequent monitoring suggested reduction of factor VIII inhibitor - antibody levels to undetectable level in 3 days and increase of factor VIII level from 3% to 50% in 5 days. Despite of improvement in the laboratory values he continued to have progression of his bleeding which involved posterior chest wall and also left arm. Due to the progression of the condition and prior expressed wish family decided to stop the aggressive treatment and patient died nine days after the diagnosis. The case report describes a rare presentation of AHA in MDS (With bone marrow cytogenetics abnormality) patient with fatal outcome.

【 授权许可】

Unknown