Objective: Acquired von Willebrand disease occurs in patients with or without 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 this 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 Willebrand disease in 6 patients with monoclonal gammapathy of undetermined significance, multiple myeloma, chronic lymphoid leukemia, Wadenstöm's macroglobulinemia, or lymphoma who were followed for 1 to 11 years.
Results: Acquired von Willebrand disease was suspected in non-thrombocytopenic 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. Administration 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 patients with congenital or acquired von Willebrand disease, but specific and etiologic chemotherapy is indicated for patients with acquired disease.