[Acquired von Willebrand's syndrome associated with malignant lymphoma].
The sudden development of multiple bleeding episodes in a 63-year-old patient with malignant lymphoma was shown to be due to the presence of an acquired von Willebrand syndrome, characterized by a prolonged bleeding time and low levels of the factors VIII: C, VIIR:Ag and VIII:Rcof. Crossed immunoelectrophoresis demonstrated diminution of the factor VIII portion with least anodic mobility. Mixing experiments of the patient's and normal plasma revealed a weak (T/2: 4.5 hours) inhibitory activity against the factors VIII:C, VIIIR:Ag and VIII:Rcof. After infusion of 800 units of cryoprecipitate there was rapid destruction (T/2: less than 1 hour) of infused factors VIII: C and VIIIR:Ag and no correction of the bleeding time. Chemotherapy resulted in disappearance of the lymphoma and the hemostatic defect, and its discontinuation in their reappearance. The weak inhibitory activity of the patient's plasma does not fully account for the factor VIII levels observed and other pathophysiological mechanisms, such as adsorption of factor VIII to the malignant lymphocytes, must be considered.
D000941:Antigens; D001791:Blood Platelet Disorders; D005167:Factor VII / Q000627:therapeutic use; D005169:Factor VIII / Q000378:metabolism; D006801:Humans; D008223:Lymphoma / Q000150:complications*; D008297:Male; D008875:Middle Aged; D010974:Platelet Aggregation; D014842:von Willebrand Diseases / Q000150:complications*
D000941:Antigens; D005167:Factor VII; D005169:Factor VIII
Syndrome de von Willebrand acquis associé à un lymphome malin.