重链沉积病：形态学、免疫荧光及超微结构评估
Heavy-chain deposition disease: a morphological, immunofluorescence and ultrastructural assessment.
作者信息
Rane Swapnil, Rana Seema, Mudrabettu Chetan, Jha Vivekananda, Joshi Kusum
机构信息
Department of Histopathology , Postgraduate Institute of Medical Education and Research , Chandigarh , India.
Department of Nephrology , Postgraduate Institute of Medical Education and Research , Chandigarh , India.
出版信息
Clin Kidney J. 2012 Oct;5(5):383-9. doi: 10.1093/ckj/sfs062.
Heavy-chain deposition disease (HCDD) is the least common of the monoclonal immunoglobulin deposition diseases with only 24 reported cases in English literature, including the present case. The rarity of this disease merits its documentation. We present a case of HCDD from our archival material, who presented with rapidly progressive renal failure and nephrotic syndrome and was found to have nodular glomerulosclerosis on renal biopsy which on immunofluorescence and electron microscopy confirmed HCDD of immunoglobulin G1 type without any light-chain deposition. We also present an in-depth literature review on HCDD.
重链沉积病（HCDD）是单克隆免疫球蛋白沉积病中最罕见的一种，英文文献中仅报道了24例病例，包括本病例。这种疾病的罕见性值得记录。我们从存档资料中报告一例HCDD，患者表现为快速进展性肾衰竭和肾病综合征，肾活检发现有结节性肾小球硬化，免疫荧光和电子显微镜检查证实为免疫球蛋白G1型HCDD，无轻链沉积。我们还对HCDD进行了深入的文献综述。
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