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在患有IgG-Lambda多发性髓瘤的患者中,十二指肠的结晶缩性囊细胞瘤

Shunsuke Koga¹, Zhaohai Yang¹, Zahra Alipour¹

¹Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, PA, USA.

International journal of surgical pathology

|May 29, 2025

概括

结晶储存囊细胞症是一种罕见的疾病,涉及免疫球蛋白在囊细胞中的结晶积累,在患有多发性骨髓瘤的患者中被确定. 这个案例突出了它的发生在十二指肠,一个不寻常的地方.

科学领域:

组织病理学组织病理学
免疫组织化学免疫组织化学
胃肠病学胃肠病学

背景情况:

晶体储存囊细胞症是一种罕见的疾病,其特点是免疫球蛋白晶体在囊细胞内积累.
它经常与血细胞疾病有关,特别是多发性骨髓瘤.
十二指肠是这种情况的不常见部位.

研究的目的:

报告一个十二指肠内结晶储存细胞瘤病例.
在此背景下讨论诊断挑战和差异诊断.
为了强调与潜在的血细胞失分症的关联.

主要方法:

十二指肠活检的组织病理学检查.
定期酸-Schiff (PAS) 染色以识别细胞内物质.
针对CD68和免疫球蛋白轻链 (lambda) 的免疫组织化学染色.

主要成果:

十二指肠活组织检查显示了囊细胞瘤与丰富的细胞质含有PAS阳性,抗扩散物质.
免疫组织化学检测证实了CD68阳性和乳腺细胞内兰巴光链限制.
这些发现支持了结晶储存细胞瘤的诊断,排除了其他疾病,如惠普尔病或兰沉积.

结论:

结晶储存囊细胞瘤可以在十二指肠表现出来,这是一个非典型的位置.
识别至关重要,特别是在有血细胞疾病病史的患者中,如多发性骨髓瘤.
为了准确的诊断,全面的组织病理学和免疫组织化学评估至关重要.

关键词:

活组织活检十二指甲的十二指甲.质细胞形成的原因是histiocytosis 多发性骨髓瘤是多发性骨髓瘤的一种.

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