PDF(Pubmed)
Abstract：
Crystal-storing histiocytosis (CSH) is a rare disorder characterized by the accumulation of non-neoplastic histiocytes that contain intracytoplasmic crystallized immunoglobulins. Although CSH can occur in various organs, gastric CSH is very rare. Therefore, diagnosing gastric CSH remains a challenge. Here, we present the case of a 69-year-old man with localized gastric CSH who presented with positive fecal occult blood for 2 days. Gastroscopy showed that there was a piece of irregular whitish focus in the big bend of the gastric antrum, which was soft and elastic. Histologically, the biopsied gastric mucosa showed chronic inflammation, mild activity with erosion, and numerous eosinophilic mononuclear cells containing fibrillary crystalloid inclusions in the lamina propria. Immunohistochemically, these crystal-containing cells were positive for CD68/PGM1 and Igk, which revealed that the cells were histiocytes harboring kappa light chain-restricted immunoglobulin crystals. Electron microscopic examination showed numerous high-electron-density particles in the cytoplasm of cells, with crystal structures of different sizes and shapes. This case highlights how immunohistochemistry can help with differential diagnosis and classification.
摘要：
储存晶体的组织细胞增多症（CSH）是一种罕见的疾病，其特征是包含胞浆内结晶免疫球蛋白的非肿瘤性组织细胞的积累。虽然CSH可以发生在不同的器官，胃CSH非常罕见。因此,诊断胃CSH仍然是一个挑战。这里,我们介绍了一例69岁的男性局限性胃部CSH患者，其粪便潜血阳性2天。胃镜检查显示胃窦大弯处有一片不规则的白色焦点，柔软有弹性。组织学上,活检的胃粘膜显示慢性炎症，轻度侵蚀活动，和许多嗜酸性单核细胞在固有层中含有原纤维晶体包裹体。免疫组织化学，这些含晶体的细胞对CD68/PGM1和Igk呈阳性，这表明这些细胞是含有κ轻链限制的免疫球蛋白晶体的组织细胞。电子显微镜检查显示细胞的细胞质中有许多高电子密度的颗粒，具有不同大小和形状的晶体结构。该病例强调了免疫组织化学如何帮助鉴别诊断和分类。
