[Severe coagulopathy due to inhibitors of several clotting factors in IgM paraproteinaemia (author's transl)].
A 62-year-old man presented with an IgM paraproteinaemia and then suddenly developed an haemophilia-like haemorrhagic diathesis. Clotting tests revealed markedly prolonged Quick prothrombin times (more than 2 min) and much diminished factor II, V, VII, and X activities. Cross-mixing experiments indicated the presence of a circulating inhibitor, which specifically blocked the activity of these factors. But the exact mechanism remains uncertain, no inhibitor of this type having been previously reported. In addition to the clotting defect there was a probably monoclonal immunoglobulin IgM. Complete remission occurred and has now persisted for 18 months, after immuno-suppressive treatment with azathioprine and prednisone. It is suggested that the inhibitory capacity is confined to the abnormal IgM, due to a primary immunological disorder. A review of similar reported cases emphasizes the importance of immunosuppressive treatment in this disorder.
D001379:Azathioprine / Q000627:therapeutic use; D005165:Factor V / Q000037:antagonists & inhibitors; D005167:Factor VII / Q000037:antagonists & inhibitors; D006474:Hemorrhagic Disorders / Q000188:drug therapy / Q000276:immunology*; D006801:Humans; D007075:Immunoglobulin M / Q000032:analysis*; D008297:Male; D008875:Middle Aged; D010265:Paraproteinemias / Q000188:drug therapy / Q000276:immunology*; D011241:Prednisone / Q000627:therapeutic use; D011516:Prothrombin / Q000037:antagonists & inhibitors; D012075:Remission, Spontaneous
D007075:Immunoglobulin M; D005165:Factor V; D005167:Factor VII; D011516:Prothrombin; D001379:Azathioprine; D011241:Prednisone
Schwere Koagulopathie infolge Hemmkörper gegen mehrere Gerinnungsfaktoren bei IgM-Paraproteinämie