Bansal Nalini, Puri Pankaj, Nagpal Nishant, Naithani Rahul, Gupta Rahul
Histopathology, Fortis Escorts Heart Institute, New Delhi, IND.
Gastroenterology and Hepatology, Fortis Escorts Heart Institute, New Delhi, IND.
Cureus. 2021 May 13;13(5):e15009. doi: 10.7759/cureus.15009.
Crystal-storing histiocytosis (CSH) is a rare tissue phenomenon that is usually associated with lympho-proliferative diseases. The disease is characterized by prominent collections of macrophages with abundant eosinophilic cytoplasm and fibrillary cytoplasmic inclusions. The inclusions appear as linear crystals within the macrophages which are usually kappa restricted. The disease usually involves lungs, lymph nodes, bone marrow, thymus and spleen with rare involvement of the gastrointestinal tract. We report a rare case of lambda-restricted CSH of the stomach. The diagnosis of CSH triggered further hematological evaluation. The patient was later diagnosed to have diffuse large B-cell lymphoma involving lymph nodes and bone marrow. He received chemotherapy for the same and is on regular follow up. The index case highlights the need to identify CSH of stomach prompting evaluation for hematological malignancies and to increase its awareness among clinicians and pathologists.
晶体储存组织细胞增多症(CSH)是一种罕见的组织现象,通常与淋巴增殖性疾病相关。该疾病的特征是巨噬细胞显著聚集,伴有丰富的嗜酸性细胞质和纤维状细胞质内含物。这些内含物在巨噬细胞内表现为线性晶体,通常为κ限制型。该疾病通常累及肺、淋巴结、骨髓、胸腺和脾脏,胃肠道受累罕见。我们报告一例罕见的胃λ限制型CSH病例。CSH的诊断引发了进一步的血液学评估。该患者后来被诊断为弥漫性大B细胞淋巴瘤,累及淋巴结和骨髓。他接受了相应的化疗,目前正在定期随访。该病例强调了识别胃CSH以促使对血液系统恶性肿瘤进行评估的必要性,并提高临床医生和病理学家对其的认识。