ACQUIRED von WILLEBRAND DISEASE (AvWD) iN HAEMAtOLOGIC MALIGNANCIES

Abstract

Acquired von Willebrand disease (AvWD) is an acquired bleeding disorder which may suddenly become manifest in individuals, usually in the absence of a personal or family history of bleedings and frequently in association with monoclonal gammopathies, lymphoproliferative, myeloproliferative and autoimmune disorders. In a minority of the cases AvWD may develop in association witb drugs or solid tumors. Pathogenetic mechanisms involve autoantibodies directed against von Willebrand factor (vWF) resulting in a rapid clearance ofvWF from the circulation and/or release of •vWF from storage• sites; or precipitation of plasma vWF. Treatment options include-whenever possible-treatment of the underlying disorder or symptomatic treatment aimed at replacing the loss of vWF by either infusion of vWF-rich concentrates or administration of desmopressin (DDAVP). In related cases witl1 anti­ vWF antibodies, administration of high-dose intravenous gammaglobulin, plasma . exchange or extracorporeal immuno-adsorption may be successful. This study included 114 patients (73 males and 41 females) and 10 controls. The age of the patient group were ranging from 15-65 years while the age of the control group were ranging from 20-65 years. The patient group were presented to NCI •• complaining of a recent development of non­ thrombocytopenic mucocutaneous bleeding due to malignant haematological disorder. These patients were subdivided according to their diagnoses into: CML (34. cases), CLL (26 cases), NHL (25 cases), MM (19 cases), HD (6 cases) and MDS (4 cases). All patients and control groups were subjected to: Complete physical and clinical examination, laboratory work up including: BT, CBC, blood grouping, serum total protein, APTT, vWF: Ag, vWF: Ri Cof activity, F VIII:C and Multimer Analysis.