ACQUIRED WILLEBRANDS-SYNDROME ASSOCIATED WITH LYMPHOPROLIFERATIVE DISORDERS

INTRODUCTION: Willebrand's syndrome is rarely acquired. We report four cases associated with lymphoproliferative syndromes. CASE REPORTS: We observed four patients with lymphoid hemopathies who developed acquir ed Willebrand's syndrome. Two patients had Waldenstrom's disease (kapp a), one had a monoclonal gammapathy of undetermined signification (kap pa immunoglobulin M) and the fourth had chronic lymphoid leukemia with mast cell infiltration of the skin. Anti-vWFRCo antibodies were evide nced in only 1 case. Chemotherapy, used in 3 cases, improved hemostasi s in one patient, Intravenous immunoglobulins (1 patient) and desmopre ssin (2 patients) were ineffective. The pathogenic mechanisms and poss ible therapeutic approaches to acquired Willebrand's syndrome are disc ussed. DISCUSSION: Acquired Willebrand's syndrome rarely occurs in ass ociation with lymphoproliferative disorders appears to be uncommon but the frequency is probably underestimated because appropriate tests ar e not always performed, The diagnostic search is important however sin ce the hemostasis disorders due to acquired Willebrand's syndrome coul d be corrected if appropriate etiological treatment is given.