[Anti-phospholipid antibody with recurrent venous thromboembolism and severe autoimmune thrombocytopenia].
A 51 year-old man with a history of deep venous thromboses and recurrent pulmonary embolism on long-term anticoagulant treatment was admitted to our department because of insidious onset thrombocytopenia. He had neither a history nor clinical signs of abnormal bleeding. On admission, the platelet count was reduced to 21 x 10(9)/l, platelet associated IgG was increased, and bone marrow specimens showed megakaryocytic hyperplasia. Platelet survival was slightly shortened with enhanced platelet sequestration in a normal size spleen. Laboratory evaluation after discontinuation of anticoagulant treatment revealed persisting prolongation of both the prothrombin time and the activated partial thromboplastin time which could be attributed to the presence of a lupus-type circulating anticoagulant. Further relevant laboratory findings included an elevated titer of IgG anti-cardiolipin antibodies and a reduced euglobulin clot lysis activity after venous occlusion due to increased plasminogen activator inhibitor activity. In recent years, it has become apparent that a striking correlation exists between the presence of antibodies to phospholipids and thromboembolic disease and immune thrombocytopenia respectively. The present case report on the association of these autoantibodies with both, recurrent venous thromboembolism and severe thrombocytopenia, supports the hypothesis that anti-phospholipid antibodies may play a crucial part in the pathogenesis of these clinical conditions. A reduced vascular fibrinolytic capacity may be involved in the thrombophilic state induced by anti-phospholipid antibodies.
Kienast
J
J
Medizinische Klinik A, Westfälischen Wilhelms-Universität Münster.
Ostermann
H
H
Stenzinger
W
W
Kötter
E M
EM
van de Loo
J
J
D001323:Autoantibodies / Q000032:analysis*; D001327:Autoimmune Diseases / Q000276:immunology*; D001779:Blood Coagulation Factors / Q000032:analysis / Q000276:immunology; D001780:Blood Coagulation Tests; D001792:Blood Platelets / Q000276:immunology; D002308:Cardiolipins / Q000276:immunology; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D007075:Immunoglobulin M / Q000032:analysis; D016682:Lupus Coagulation Inhibitor; D008297:Male; D008875:Middle Aged; D010743:Phospholipids / Q000276:immunology*; D010976:Platelet Count; D011655:Pulmonary Embolism / Q000276:immunology; D012008:Recurrence; D013921:Thrombocytopenia / Q000276:immunology*; D013924:Thrombophlebitis / Q000276:immunology*
D001323:Autoantibodies; D001779:Blood Coagulation Factors; D002308:Cardiolipins; D007074:Immunoglobulin G; D007075:Immunoglobulin M; D016682:Lupus Coagulation Inhibitor; D010743:Phospholipids
Anti-Phospholipid Antikörper mit rezidivierenden venösen Thromboembolien und schwerer Autoimmunthrombozytopenie.