[Acquired Willebrand syndrome with lymphoproliferative disorders].
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.
Hamidou
M
M
Service de Médecine interne A, Hôtel-Dieu, Nantes.
Agard
C
C
Fressinaud
E
E
Fiks-Sigaud
M
M
Rapp
M J
MJ
Grolleau
J Y
JY
Harousseau
J L
JL
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
D000305:Adrenal Cortex Hormones; D007166:Immunosuppressive Agents
Syndrome de Willebrand acquis avec désordres lymphoprolifératifs.