혈전성 혈소판 감소성 자반증의 임상상을 보인 항사구체 기저막 항체질환 1 예

초록

Thrombotic thrombocytopenic purpura(TTP) was first described in 1925, however, the definite cause of TTP remained unknown. Further study revealed that the pathogenesis of thrombotic thrombocytopenia was related to autoimmune disease. Autoantibodies generated by vessel injury and other causes result in excessive platelet aggregation and consumption. Recently, many autoimmune diseases and conditions associated with vascular injury have been reported to be accompanied by TTP-like feature, such as microangiopathic hemolytic anemia and thrombocytopenia. Anti-glomerular basement membrane(GBM) mediated glomerulonephritis is also related to autoantibody against collagen IV fiber in GBM, and cha- racterizded by diffuse glomerular capillary injury. Although the relationship of these two diseases is not clearly defined, here we report a case of anti- GBM antibody disease accompanied by TTP-like feature in a 40-year old man admitted for dyspnea. The patient had hemolytic anemia, thrombocytopenia, and acute renal failure. Anti-GBM antibody was positive and crescent formation and linear deposition of IgG along glomerular basement membrane was seen in renal biopsy. Disease progressed with no clinical response despite of prompt treatment by steroid and cyclophosphamide pulse therapy and plasmapheresis.

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