首页|消化道储晶体组织细胞增多症临床病理学分析

消化道储晶体组织细胞增多症临床病理学分析

扫码查看
目的 探讨发生于消化道的储晶体组织细胞增多症(CSH)的临床病理特点、鉴别诊断及与其相关的肿瘤与非肿瘤性病变.方法 对 1 例胃体黏膜储晶体组织细胞增多症活检标本的内镜表现、HE形态、免疫组化染色及基因重排检测进行观察分析,并复习和总结消化道原发储晶体组织细胞增多症相关文献.结果 本例内镜下表现为境界清楚的白色斑块,HE切片:胃黏膜固有层可见大量胞质内含有晶体状包涵体的细胞增生聚集,深部伴较多浆细胞及少量淋巴细胞浸润.免疫组化证实含晶体状包涵体的细胞为组织细胞,晶体状包涵体及周围浆细胞均存在κ轻链限制性表达,基因重排检测显示B细胞受体克隆性重排,证明晶体成分为组织细胞吞噬的免疫球蛋白,并且提示其与B细胞单克隆增生性病变相关.诊断为黏膜相关淋巴组织边缘区B细胞淋巴瘤伴浆样分化,伴储晶体组织细胞增多症.回顾了文献中报道的 21 例消化道原发储晶体组织细胞增多症,发现多数患者合并有B细胞淋巴瘤或单克隆淋巴/浆细胞增生性病变,少数病例与自身免疫性疾病或特殊用药史相关.结论 消化道储晶体组织细胞增多症罕见,鉴别诊断包括卢梭小体胃炎低黏附性癌、颗粒细胞瘤等病变,依据形态学及免疫组化染色,诊断并不困难.但该病变常合并淋巴造血系统肿瘤或其他非肿瘤性病变,需结合基因重排检测、临床表现、病史及其他辅助检查综合判断.
Clinicopathological features of crystal-storing histiocytosis in the digestive tract
Objective To investigate the clinical pathological features,differential diagnoses and the associated lesions of crystal-storing histiocytosis(CSH)of the digestive tract.Methods A case of crystal-storing histiocytosis developed in the stomach was reported and a literature review was performed.Results The lesion showed as a whitish plaque of mucosa of gastric body under the endoscope.Microscopically,the lamina propria of mucosa was markedly extensive with accumulation of histiocytes with abundant intracytoplasmic crystal inclusions,which showed restricted expression of kappa light chain,as well as the surrounding plasma cells.Molecular assay was performed and clonal gene rearrangement of Igκ was revealed.A diagnosis of MALT lymphoma with plasma differentiation,combined with CSH was made.Conclusion Crystal-storing histiocytosis of the digestive tract is rare and often associated with lymphoplasmacytic disorder.Once the diagnosis of CSH is made,checking for underlying neoplastic conditions by using histological,molecular and clinical methods is necessary.

Crystal-storing histiocytosisGene rearrangementDifferential diagnosisImmunohistochemistryIymphoma

杜强、薛丽燕

展开 >

国家癌症中心 国家肿瘤临床医学研究中心 中国医学科学院北京协和医学院肿瘤医院 病理科,北京 100021

储晶体组织细胞增多症 基因重排 鉴别诊断 免疫组化 淋巴瘤

2024

诊断病理学杂志
北京军区总医院

诊断病理学杂志

CSTPCD
影响因子:0.663
ISSN:1007-8096
年,卷(期):2024.31(12)