Soma Jun, Sato Kozo, Sakuma Tsutomu, Saito Hirotaka, Sato Hiroshi, Sato Toshinobu, Abbas Acha, Aucouturier Pierre
Department of Nephrology, Iwate Prefectural Central Hospital, Morioka, Japan.
Am J Kidney Dis. 2004 Jan;43(1):E10-6. doi: 10.1053/j.ajkd.2003.09.024.
The authors describe a 54-year-old woman presenting with proteinuria, hematuria, and hypocomplementemia whose renal biopsy results showed diffuse increase in mesangial matrix and nodular formations in several glomeruli with the deposition of immunoglobulin gamma3-heavy-chain and complement components C1q and C3 in the glomeruli and on the tubular basement membranes, without associated light-chain deposits. Staining for the constant domains of gamma-heavy-chain showed a deletion of the first constant domain (CH1). These findings were consistent with those of gamma-heavy-chain deposition disease (gamma-HCDD). The patient was treated monthly with melphalan and prednisolone although a bone marrow aspirate did not show findings suggestive of plasmacytoma. Six courses of melphalan and prednisolone therapy resulted in a marked reduction of urinary protein excretion and marked rise of complement levels. The current case is the fourth HCDD patient reported featuring gamma3-heavy-chain deposition who showed severe hypocomplementemia and responded to chemotherapy with improved renal parameters and complement levels. A review of previously reported cases of HCDD showed that some but not all HCDD cases were associated with hypocomplementemia. The authors also discuss here the relationship of HCDD to hypocomplementemia.
作者描述了一名54岁女性，表现为蛋白尿、血尿和低补体血症，其肾活检结果显示系膜基质弥漫性增加，多个肾小球出现结节状形成，免疫球蛋白γ3重链及补体成分C1q和C3沉积于肾小球及肾小管基底膜，无相关轻链沉积。γ重链恒定区染色显示第一个恒定区（CH1）缺失。这些发现与γ重链沉积病（γ-HCDD）相符。尽管骨髓穿刺未显示提示浆细胞瘤的结果，但该患者每月接受美法仑和泼尼松龙治疗。六个疗程的美法仑和泼尼松龙治疗使尿蛋白排泄显著减少，补体水平显著升高。该病例是第四例报道的以γ3重链沉积为特征的HCDD患者，表现为严重低补体血症，对化疗有反应，肾脏参数和补体水平得到改善。对先前报道的HCDD病例进行回顾发现，部分但并非所有HCDD病例都与低补体血症有关。作者在此还讨论了HCDD与低补体血症的关系。
