抄録
A case of thrombotic thrombocytopenic purpura (TTP) treated sucessfuly by plasmapheresis, anti-platelet drugs and pred nisolone is reported.
A 33 years old housewife was diagnosed TTP because of microangiopathic hemolytic anemia, thrombocytopenia, proteinuria, microhematuria, coma, tonic convulsion and high fever. LDH was 3916 IU/L and all isozyme types were equally elevated. Serologically, anti-nuclear antibody was weakly positive, but other autoantibodies and immune complex were negative. Plasma 6-keto-PGF1α level was as low as 36 pg/ml and TXB2 was 280 pg/ml.
Gum biopsy revealed fibrin thrombus in the lumens of the submucosal capillaries and deposition of hyaline-like substances in the subendothelial regions without any activities of fibrinogen, IgG, A, M or complement in those regions by PAP method.
Treatment was started with pred nisolone 100 mg, Aspirin 1 g, dipyridamole 20 mg, urokinase 24,000 U. Plasmapheresis of 3,000 ml by Hemonetics PEX was done. Platelet count increased rapidly and patient recovered fully without any sequelae. Plasmapheresis is effective method to be chosen firstly in the treatment of TTP.
