ISSN 2997-1036
International Journal of Hematology | Vol. 5, No. 9, September 2014 | pp. 65–72
DOI: 10.46882/2014/IJH/000057
Case Report
Title: Acquired Factor VIII inhibitor development in a patient with chronic lymphocytic leukemia: Successful eradication with rituximab
Names of Authors: K. L. Musa¹, M. N. Lawal², O. P. Dikko³
Authors’ Affiliations: ¹Department of Haematology, Federal Medical Centre, Katsina, Nigeria; ²Department of Internal Medicine, Bayero University, Kano, Nigeria; ³Department of Pathology, Ahmadu Bello University Teaching Hospital, Zaria, Nigeria
Abstract: Acquired hemophilia A is a rare, severe bleeding disorder caused by autoantibodies directed against coagulation Factor VIII, occasionally presenting as a paraneoplastic manifestation of B-cell malignancies. We report a 62-year-old male with stable Binet stage A chronic lymphocytic leukemia who presented with spontaneous, extensive soft tissue ecchymoses on his right thigh. Coagulation screens revealed an isolated, prolonged activated partial thromboplastin time of 84.5 seconds that failed to correct during a 1:1 mixing study with normal pooled plasma, confirming the presence of an inhibitor. Factor VIII activity was profoundly reduced (< 1.0%), and a Bethesda assay quantified a Factor VIII inhibitor titer of 24.5 Bethesda Units. Immunosuppressive therapy with high-dose prednisone failed to clear the inhibitor after 3 weeks. Subsequently, a targeted rituximab protocol (375 mg/m² weekly for 4 weeks) was initiated. Complete clinical resolution of bleeding and clearance of the inhibitor (0 BU) was achieved by week 6, alongside a normalization of Factor VIII activity (94.2%). This case highlights that acquired autoantibodies should be considered in lymphocytic leukemia patients with unprovoked bleeding, and demonstrates that rituximab provides rapid eradication of pathogenic clones.
Keywords: Acquired hemophilia A, Factor VIII inhibitor, chronic lymphocytic leukemia, rituximab, Bethesda assay
Manuscript Timeline: Received: June 18, 2014; Revised: July 28, 2014; Accepted: August 15, 2014; Published: September 17, 2014