혈전성 혈소판감소성 자반증으로 발현된 전신홍반루푸스 1예

Systemic Lupus Eiythematosus Presenting as Thromobotic Thrombocytopenic Puipura -A Case Report-

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening syndrome characterized by the classic pentad of clinical features that includes microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever, and renal dysfunction. Early diagnosis and utilization of plasmapheresis can improve the survival rates of patients with TTP. TTP rarely may be seen in association with autoimmune disease such as systemic lupus erythematous (SLE). We report here a 42-year-old female who was presented with severe digital gangrenes, microangiopathic hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever, and renal dysfunction. Her condition responded to combined therapy with high dose steroid, immunosuppressants, and plasmapheresis therapy.