Pulmonary extranodal marginal zone lymphoma with massive crystal storing histiocytosis
Pulmonary extranodal marginal zone lymphoma with massive crystal storing histiocytosis
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DOI：
10.1097/01.pas.0000178093.99889.f7
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发表时间：
2006-02-01
影响因子：
5.6
通讯作者：
Tsikleas, G
中科院分区：
文献类型：
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作者：
Fairweather, PM;Williamson, R;Tsikleas, G
我们报告一例偶然发现的肺支气管粘膜相关淋巴组织淋巴瘤合并大量晶体储存组织细胞增多症的罕见病例，患者为69岁女性。肿瘤过程被大量含有结晶物质的上皮样细胞和纺锤状组织细胞所掩盖。组织化学染色显示胞浆内结晶，免疫过氧化物酶染色证实了肿胀细胞的组织细胞性质。电镜显示免疫球蛋白晶体的特征。免疫化学染色发现淋巴上皮病变，合并了潜在淋巴样肿瘤的怀疑。分子研究后来发现了一个单克隆b细胞群。患者无副蛋白血症的全身性证据。晶体储存组织细胞病是巨噬细胞聚集轻链或免疫球蛋白晶体包涵体的一种罕见现象。这种疾病可能是全身性的，也可能像本病例一样是局部性的。它通常与单克隆免疫球蛋白的过量产生有关，尽管有许多病例报告没有这种特征。综述了晶体储存组织细胞增多症。
We present an incidentally discovered, unusual case of pulmonary bronchial mucosa-associated lymphoid tissue lymphoma associated with massive crystal storing histiocytosis in a 69-year-old woman. The neoplastic process was masked by large numbers of epithelioid and spindled histiocytes containing crystalline material. Histochemical stains showed intracytoplasmic crystals and immunoperoxidase staining confirmed the histiocytic nature of the swollen cells. Electron microscopy demonstrated the characteristic appearance of immunoglobulin crystals. Lymphoepithelial lesions were identified on immunoltistochemical staining, compounding suspicions of an underlying lymphoid neoplasm. Molecular studies later revealed a monoclonal B-cell population. The patient had no systemic evidence of a paraproteinemia. Crystal storing histiocytosis is a rare phenomenon in which macrophages accumulate light chain or immunoglobulin crystalline inclusions. The disease may be systemic, or localized as in this case. It is usually associated with the excess production of a monoclonal immunoglobulin, although there have been many case reports without this feature. An over-view of crystal storing histiocytosis is given.