title: [Acquired Willebrand syndrome with lymphoproliferative disorders].
abstract: BACKGROUND
  Willebrand's syndrome is rarely acquired. We report four cases associated with lymphoproliferative syndromes.
  
  
  METHODS
  We observed four patients with lymphoid hemopathies who developed acquired Willebrand's syndrome. Two patients had Waldenström's disease (kappa), one had a monoclonal gammapathy of undetermined signification (kappa immunoglobulin M) and the fourth had chronic lymphoid leukemia with mast cell infiltration of the skin. Anti-vWFRCo antibodies were evidenced in only 1 case. Chemotherapy, used in 3 cases, improved hemostasis in one patient. Intravenous immunoglobulins (1 patient) and desmopressin (2 patients) were ineffective. The pathogenic mechanisms and possible therapeutic approaches to acquired Willebrand's syndrome are discussed.
  
  
  CONCLUSIONS
  Acquired Willebrand's syndrome rarely occurs in association with lymphoproliferative disorders appears to be uncommon but the frequency is probably underestimated because appropriate tests are not always performed. The diagnostic search is important however since the hemostasis disorders due to acquired Willebrand's syndrome could be corrected if appropriate etiological treatment is given.
authors.affiliation: Service de Médecine interne A, Hôtel-Dieu, Nantes.
authors.forename: M
authors.initials: M
authors.lastname: Hamidou
authors.forename: C
authors.initials: C
authors.lastname: Agard
authors.forename: E
authors.initials: E
authors.lastname: Fressinaud
authors.forename: M
authors.initials: M
authors.lastname: Fiks-Sigaud
authors.forename: M J
authors.initials: MJ
authors.lastname: Rapp
authors.forename: J Y
authors.initials: JY
authors.lastname: Grolleau
authors.forename: J L
authors.initials: JL
authors.lastname: Harousseau
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000368:Aged; D002908:Chronic Disease; D004630:Emergencies; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008232:Lymphoproliferative Disorders / Q000209:etiology* / Q000276:immunology / Q000628:therapy; D008297:Male; D008875:Middle Aged; D014842:von Willebrand Diseases / Q000150:complications* / Q000276:immunology / Q000628:therapy
chemical_list: D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
vernacular_title: Syndrome de Willebrand acquis avec désordres lymphoprolifératifs.