Renal Pathology, Department of Pathology, Johns Hopkins University and Johns Hopkins Hospital, Baltimore, MD 21287, USA.
Renal Pathology, Department of Pathology, Johns Hopkins University and Johns Hopkins Hospital, Baltimore, MD 21287, USA.
Ann Diagn Pathol. 2019 Dec;43:151403. doi: 10.1016/j.anndiagpath.2019.151403. Epub 2019 Aug 29.
Crystal-storing histiocytosis (CSH) is a rare manifestation of monoclonal gammopathy in which histiocytes containing monoclonal proteins in their cytoplasm are found in various organs of the body including the kidney. Within the kidney, these monoclonal crystal-laden histiocytes have been described to occur in the interstitium (most commonly) or in the glomerular mesangium. CSH within glomerular capillary loops has rarely been reported. We describe three cases of CSH primarily affecting the glomerular capillaries and review the literature of CSH in general. Twenty cases of CSH involving the kidney are present in the literature; three describe CSH in glomeruli, only one of which showed histiocytes predominantly in glomerular capillary loops, while 15 had predominantly or solely interstitial CSH. Most cases involve IgG kappa crystals with only one case involving lambda light chain. Patients with CSH predominantly involving the glomerular capillaries showed a trend toward lower serum creatinine and proteinuria at presentation, and several patients with CSH lacked a definitive diagnosis of a monoclonal gammopathy at the time of diagnosis, emphasizing the role that kidney biopsy and particularly electron microscopy play in diagnosis of this entity.
晶体储存性组织细胞增生症（CSH）是单克隆丙种球蛋白病的一种罕见表现，其中在身体的各种器官（包括肾脏）中发现含有单克隆蛋白的组织细胞。在肾脏中，这些载有单克隆晶体的富含组织细胞已被描述为发生在间质（最常见）或肾小球系膜中。肾小球毛细血管袢内的 CSH 很少有报道。我们描述了三种主要影响肾小球毛细血管的 CSH，并复习了一般的 CSH 文献。文献中有 20 例涉及肾脏的 CSH，其中 3 例描述了肾小球中的 CSH，其中仅 1 例显示组织细胞主要位于肾小球毛细血管袢内，而 15 例主要或仅为间质 CSH。大多数病例涉及 IgG κ 晶体，仅有 1 例涉及 λ 轻链。主要累及肾小球毛细血管的 CSH 患者在发病时表现出血清肌酐和蛋白尿降低的趋势，并且一些 CSH 患者在诊断时缺乏明确的单克隆丙种球蛋白病诊断，这强调了肾活检，特别是电子显微镜在诊断该实体中的作用。
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