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肾累及伴皮肤肾小球样血管瘤和浆细胞瘤的多中心性Castleman病

Renal involvement in multicentric Castleman disease with glomeruloid hemangioma of skin and plasmacytoma.

作者信息

Uthup Susan, Balachandran Krishna, Ammal Vimala Avadai, Abdul Salam Riyas, George Jacob, Nair Geetha M, Leela Manju

机构信息

Department of Nephrology, Medical College Thiruvananthapuram, Kerala, India.

出版信息

Am J Kidney Dis. 2006 Aug;48(2):e17-24. doi: 10.1053/j.ajkd.2006.04.089.

Abstract

A 47-year-old man presented with fever of unknown origin, generalized weakness, edema, and renal failure. He had left-sided pleural effusion, generalized lymphadenopathy, multiple nontender cutaneous nodules, hepatomegaly, renal failure, and hypergammaglobulinemia. Axillary lymph node biopsy showed findings consistent with Castleman disease of the hyaline vascular type associated with interfollicular plasmacytosis. A renal biopsy performed in view of proteinuria and acute renal failure showed hypercellular glomeruli with capillary loop thickening and double contours consistent with membranoproliferative glomerulonephritis. Skin nodule biopsy showed a glomeruloid hemangioma characterized by dermal proliferation of capillary loops in a nodular manner resembling a glomerulus. He experienced clinical and biochemical remission with steroids. Discontinuation of steroid therapy was associated with recurrence of renal failure, reappearance of nodules, lymphadenopathy, and appearance of bony lytic lesions. Biopsy of bony lytic lesions showed plasmacytoma. The patient achieved complete remission on treatment with steroids and cyclophosphamide and is free of symptoms at the end of 40 months of follow-up. To our knowledge, this is the first case report of the occurrence of membranoproliferative glomerulonephritis, glomeruloid hemangioma of the skin, and plasmacytoma in a patient with multicentric Castleman disease without human immunodeficiency virus infection.

摘要

一名47岁男性出现不明原因发热、全身乏力、水肿及肾衰竭。他有左侧胸腔积液、全身淋巴结肿大、多个无压痛的皮肤结节、肝肿大、肾衰竭及高球蛋白血症。腋窝淋巴结活检结果符合伴有滤泡间浆细胞增多症的透明血管型Castleman病。鉴于蛋白尿和急性肾衰竭进行的肾活检显示肾小球细胞增多,毛细血管袢增厚且有双轨征,符合膜增生性肾小球肾炎。皮肤结节活检显示为肾小球样血管瘤,其特征为真皮内毛细血管袢呈结节状增生,类似肾小球。他使用类固醇后临床和生化指标缓解。停用类固醇治疗后出现肾衰竭复发、结节再现、淋巴结肿大以及骨溶解性病变。骨溶解性病变活检显示为浆细胞瘤。该患者使用类固醇和环磷酰胺治疗后完全缓解,随访40个月结束时无症状。据我们所知,这是首例无人类免疫缺陷病毒感染的多中心Castleman病患者发生膜增生性肾小球肾炎、皮肤肾小球样血管瘤及浆细胞瘤的病例报告。

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