Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN, USA.
Am J Kidney Dis. 2011 Oct;58(4):621-5. doi: 10.1053/j.ajkd.2011.05.022.
Heavy chain deposition disease (HCDD) is a comparatively recently described entity characterized by glomerular and tubular basement membrane deposition of monoclonal heavy chains without associated light chains. To our knowledge, review of the literature shows only 24 previously reported cases of HCDD with unequivocal evidence of monoclonal heavy chain deposition in the kidney using immunofluorescence microscopic and electron microscopic studies. The predominant heavy chain subtype was γ. There has been a single case of μ HCDD and 2 previously reported cases of α HCDD. In this report, we describe 3 additional cases of α HCDD, all with a crescentic pattern of injury and one of which was associated with cutis laxa. We compare their clinicopathologic features with all previously reported cases of HCDD.
重链沉积病（HCDD）是一种相对较新描述的疾病，其特征是肾小球和肾小管基底膜沉积单克隆重链，而无相关的轻链。据我们所知，文献复习仅显示了 24 例以前报道的 HCDD 病例，这些病例在使用免疫荧光显微镜和电子显微镜研究时具有明确的肾脏单克隆重链沉积证据。主要的重链亚型是γ。有一例μ HCDD 和两例以前报道的α HCDD。在本报告中，我们描述了另外 3 例α HCDD，均具有新月体损伤模式，其中 1 例与皮肤松弛症有关。我们将它们的临床病理特征与以前报道的所有 HCDD 病例进行了比较。
