[Association of acquired Willebrand's disease, monoclonal gammopathy and angiodysplasia of the small bowel: a rare indication of high-dose intravenous immunoglobulins].
Acquired von Willebrand's disease associated with a monoclonal gammopathy and angiodysplasia of the gut is a rare disorder. It is sometimes complicated by chronic intestinal bleeding and severe anemia, that is poorly responsive to usual treatments. We report such a new case that has been revealed by anemia, and characterised by the absence of the high-molecular weight multimers. The correction of the hemostasis defect and of anemia were related to the reappearance of the high-molecular weight multimers, that was achieved only after high-dose intravenous immunoglobulin courses. The perfusions were performed every 3 weeks for 2 years without loss of efficiency, that could be explained by the dissociation of immunoglobulin-von Willebrand's factor complex.
Sailler
L
L
Service de néphrologie, pavillon Sénac, CHU Purpan, Toulouse, France.
Ecoiffier
M
M
Cadroy
Y
Y
Couret
B
B
Sié
P
P
Mazurier
C
C
Arlet-Suau
E
E
Pourrat
J P
JP
D000368:Aged; D016888:Angiodysplasia / Q000150:complications* / Q000188:drug therapy; D006801:Humans; D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous / Q000008:administration & dosage / Q000627:therapeutic use*; D007421:Intestine, Small; D008297:Male; D010265:Paraproteinemias / Q000150:complications* / Q000188:drug therapy; D013997:Time Factors; D014842:von Willebrand Diseases / Q000150:complications* / Q000188:drug therapy
D007074:Immunoglobulin G; D016756:Immunoglobulins, Intravenous
Association maladie de Willebrand acquise--gammapathie monoclonale--angiodysplasie du grêle: une indication rare des immunoglobulines intraveineuses á forte dose.