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논문분류 춘계학술대회 초록집
제목 IgA nephropathy Associated with Complement-mediated Thrombotic Microangiopathy Treated Successfully by Steroid therapy
저자 Ha Nee Jang* 1, Tae Won Lee1, Min Jeong Kim1, Yire Kim1, Kyunglan Moon1, Hyun Seop Cho1, 2, Se-Ho Chang1, 2, Eunjin Bae3, Dong Jun Park1, 2, 3, Hyun-Jung Kim1, 2
출판정보 2016; 2016(1):
키워드 comeplement-mediated TMA, hemolytic uremic syndrome, IgA nephropathy , methylprednisolone, nephrotic syndrome, thrombotic microangiopathy, thrombotic thrombocytopenic purpura, TMA
초록 Background: The primary thrombotic microangiopathy (TMA) syndrome includes thrombotic thrombocytopenic purpura (TTP), hemolytic uremic syndrome (HUS), comeplement-mediated TMA, drug-induced TMA and metabolism- or coagulation-mediated TMA by underlying cause. This systemic disorder is characterized by nonimmune microangiopathic hemolytic anemia (MAHA) and thrombocytopenia. Complement-mediated TMA can be caused by hereditary deficiency of regulatory proteins or acquired complement autoantibodies. IgA nephropathy (IgAN) that is the most common glomerulonephritis has been rarely reported with HUS, and the relationship between them is still unknown. We experienced the patient with complement-medicated TMA with IgAN and treated her successfully by steroid therapy. Methods: A 72-year-old woman was referred to our hospital due to azotemia aggravation, dyspepsia and gross hematuria. She denied taking any medications. Her laboratory findings revealed MAHA with elevated reticulocyte count, severe thrombocytopenia, high lactate dehydrogenase (LDH) and low haptoglobin. Her bone marrow examination revealed normocellular marrow with trilineage hematopoiesis. Her complement levels were decreased without vitamin B12 deficiency or ADAMTS 13 activity defect. Her renal pathologic findings were compatible with IgAN. But, the presenting clinical features were compatible to the characteristics of complement-associated TMA. Results: She was treated with oral methylprednisolone 1 mg/kg daily. Her abnormal renal and hematologic laboratory findings were restored to normal after 8 weeks. Conclusion: We treated her successfully without plasma exchange even though she presented complement-associated TMA with IgAN.
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