Acquired von Willebrand disease secondary to lymphoproliferative syndromes: 6 cases

OBJECTIVE: Acquired von Willebrand disease occurs in patients with or witho ut cutaneous and mucosal bleeding who have no personal or family history of the disease. The clinical course of these patients is poorly known due to the rarity of acquired von Willebrand factor (vWF) deficit. We conducted th is study to assess the clinical course of acquired vWF deficit secondary to lymphoproliferative syndromes. PATIENTS AND METHODS: We report the clinical course of acquired von Willebr and disease in 6 patients with monoclonal gammapathy of undetermined signif icance, multiple myeloma, chronic lymphoid leukemia, Wadenstom's macroglobu linemia, or lymphoma who were followed for 1 to 1 1 years. RESULTS: Acquired von Willebrand disease was suspected in nonthrombocytopen ic patients with a lymphoproliferative syndrome who developed a hemorrhagic syndrome. The VWF anomaly was symptomatic in 4 of 6 patients at diagnosis. Patients were given symptomatic treatment with VWF replacement therapy as needed and specific treatment for their lymphoproliferative syndrome. Admin istration of DDAVP was sufficient in 3 out of 4 patients to allow invasive procedures but was unable to control digestive ulcer bleeding that required infusion of factor VIII-VWF concentrate. For 2 patients, chemotherapy was initiated due to threatening massive hemorrhage. The result was spectacular The 4 other patients have been asymptomatic without treatment for 3, 5, 6 and 11 years during which time their lymphoproliferative syndrome has been quiescent. CONCLUSION: The clinical features and laboratory findings are similar in pa tients with congenital or acquired von Willebrand disease, but specific and etiologic chemotherapy is indicated for patients with acquired disease.