Hou Bo, Zhu Na, Yao Jiamei, Yu Juan, Zhang Lei, Tan Yunshan, Hou Yingyong, Ge Xiaowen
Department of Pathology, Zhongshan Hospital, Fudan University, Shanghai, China.
Ann Med Surg (Lond). 2025 Jun 10;87(7):4536-4542. doi: 10.1097/MS9.0000000000003367. eCollection 2025 Jul.
Crystal-storing histiocytosis (CSH) is a rare, non-neoplastic histiocytic proliferation that often complicates underlying lymphoproliferative or plasma cell disorders. The respiratory system is uncommonly involved in CSH. We present a local case of pulmonary CSH associated with marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT), along with eight additional cases identified from a literature review.
A 64-year-old woman was admitted to the hospital for coughing, expectoration, and asthma for more than 1 year. Chest CT revealed localized thickening of the tracheal wall, and the bronchoscopy examination showed neoplasms of the trachea and bronchus mucosa. The lesion was characterized by sheets of histiocytes with abundant eosinophilic cytoplasm and scattered aggregates of atypical lymphoid cells. Immunohistochemical stains and T/B-cell gene rearrangement studies supported the pathological diagnosis of CSH-associated MALT. The patient remained free from the disease after lesion resection via bronchoscopy, with further treatment of targeted therapy.
Pulmonary CSH-associated MALT can occur in patients of various ages and genders with non-specific clinical and imaging features. Histopathological examination is essential for differentiating CSH from similar conditions. The pathogenesis of CSH may relate to the overproduction of immunoglobulins, and the immunoglobulin types involved in the cases we described are predominantly kappa light chains.
Recognizing CSH is essential for starting a clinical evaluation to identify the underlying neoplasm or associated causes. A conclusive diagnosis relies on pathological examination. More detailed case reports are needed to clarify pathogenesis and improve diagnosis, treatment strategies, and patient outcomes.
晶体储存组织细胞增多症（CSH）是一种罕见的非肿瘤性组织细胞增殖性疾病，常使潜在的淋巴增殖性疾病或浆细胞疾病复杂化。呼吸系统较少累及CSH。我们报告一例与黏膜相关淋巴组织边缘区淋巴瘤（MALT）相关的肺部CSH病例，并通过文献回顾另外确定了8例。
一名64岁女性因咳嗽、咳痰和气喘1年余入院。胸部CT显示气管壁局限性增厚，支气管镜检查显示气管和支气管黏膜有肿物。病变特征为成片的组织细胞，胞质丰富嗜酸性，并有散在的非典型淋巴细胞聚集。免疫组化染色和T/B细胞基因重排研究支持CSH相关MALT的病理诊断。该患者经支气管镜切除病变后病情缓解，并接受了靶向治疗。
肺部CSH相关MALT可发生于各年龄和性别的患者，临床和影像学特征无特异性。组织病理学检查对于鉴别CSH与类似疾病至关重要。CSH的发病机制可能与免疫球蛋白过度产生有关，我们所描述病例中涉及的免疫球蛋白类型主要为κ轻链。
认识CSH对于启动临床评估以识别潜在肿瘤或相关病因至关重要。明确诊断依赖于病理检查。需要更详细的病例报告以阐明发病机制并改善诊断、治疗策略及患者预后。
