Funabiki K, Kaneko S, Terajima M, Tomita H, Kawano Y, Tomino Y
Department of Medicine, Juntendo University School of Medicine, Hongo, Tokyo, Japan.
Am J Nephrol. 1998;18(3):247-50. doi: 10.1159/000013346.
This is the first reported case of multicentric Castleman's disease (MCD) associated with renal amyloidosis and pure red cell aplasia (PRCA). Inguinal lymph node biopsy showed follicular hyperplasia with prominent germinal centers and plasma cell proliferation in the interfollicular areas. Renal biopsy specimens revealed intraglomerular amyloid deposits, defined as AA amyloidosis. Since amyloid deposits were not present in the gastric and rectal mucosal tissues, his renal disorder was found to be an unusual secondary amyloidosis associated with MCD. Following treatment by plasma exchange, there was progressive deterioration of anemia due to PRCA detected by bone marrow aspiration. Subsequently he was successfully treated with steroid pulse therapy not only for anemia but also for renal function.
这是首例报告的与肾淀粉样变性和纯红细胞再生障碍性贫血(PRCA)相关的多中心Castleman病(MCD)病例。腹股沟淋巴结活检显示滤泡增生,生发中心突出,滤泡间区浆细胞增殖。肾活检标本显示肾小球内淀粉样沉积,诊断为AA型淀粉样变性。由于胃和直肠黏膜组织中未发现淀粉样沉积,发现他的肾脏疾病是一种与MCD相关的罕见继发性淀粉样变性。在进行血浆置换治疗后,骨髓穿刺检测发现因PRCA导致贫血进行性恶化。随后,他成功接受了类固醇脉冲疗法治疗,不仅改善了贫血,还改善了肾功能。