Acquired Platelet Dysfunction as a Cause of Bleeding Diathesis: Two Case Studies

Abstract

The aims are to highlight the importance of recognizing that clinically significant platelet dysfunction can develop in the background of known causes of bleeding disorders. In this paper, we discuss two patients who developed acquired platelet dysfunction due to eosinophilia with underlying mild Hemophilia A and IgA paraproteinemia that complicated a clinical picture of acquired von Willebrand disease.Case 1: An 8-yr-old boy with known mild haemophilia A presented with abdominal pain and gross haematuria that partially response to Factor VIII concentrate and cryoprecipitate. Tests revealed significant peripheral eosinophilia (AEC 3200/mm 3 ), prolonged bleeding time >15seconds, FVIIIc 31%, normal vWF: Ag and RICOF assays. Functional platelet studies were abnormal and a diagnosis of Acquired Platelet Dysfunction with Eosinophilia (APDE) was made. His symptoms resolved with standard treatment of eosinophilia with anti-histamines, diethylcarbamazine and albendazole.Case 2: A 57-year-old lady was admitted with malena following commencement of oral prednisolone for recently diagnosed Sjogrens syndrome. UGI showed multiple gastric erosions with no response to standard treatment. She was a known diabetic and treated for carcinoma breast 4years ago. A year before this admission, she had her first bleeding episode with epistaxis following septoplasty (APTT prolonged at 40s, not investigated). She finally responded to fresh frozen plasma and cryoprecipitate. Investigations showed APTT 55sec, protein electrophoresis/immune fixation-small spike with IgA lambda and normal SFLC ratio. The mucosal bleeds with partial response to cryoprecipitate and FFP was suggestive of aVWD. Functional platelet studies were abnormal and a diagnosis of paraprotein associated acquired platelet dysfunction was made. She responded to rituximab with reduction in IgA levels and no further bleeds and remains on bortezomib. Identification of acquired platelet dysfunction and knowledge of how that may affect bleeding risk with existing complex autoimmune/hematological disorders are important in optimizing patient care.