骨髓含铁血黄素沉积症：8 例临床病理研究及文献复习
Crystal-Storing Histiocytosis in Bone Marrow: A Clinicopathologic Study of Eight Cases and Review of the Literature.
机构信息
Department of Laboratory Medicine and Pathology, Division of Hematopathology, Mayo Clinic, Rochester, MN.
出版信息
Am J Clin Pathol. 2018 Jan 29;149(2):148-163. doi: 10.1093/ajcp/aqx150.
OBJECTIVES
We report the clinicopathologic characteristics of eight cases of crystal-storing histiocytosis (CSH) with bone marrow (BM) involvement (BM-CSH) and review CSH cases published in the English literature.
METHODS
We queried our pathology database for BM cases with CSH mentioned in the final diagnosis/comments from June 2011 to August 2016.
RESULTS
Eight cases of BM-CSH were identified. The underlying diagnoses consisted predominantly of plasma cell disorders (88%) associated with monotypic κ light chain. In BM aspirates, crystals within histiocytes exhibited a morphologic spectrum including brightly eosinophilic, needle-like, or globule-like. In BM core biopsies, the histiocytes were often in aggregates with intracellular needle-like and/or globular, refractile inclusions.
CONCLUSIONS
BM-CSH is a rare phenomenon and exhibits a heterogeneous crystalline and histiocytic appearance warranting accurate recognition to avoid misinterpretation of a granulomatous condition or storage disorder. In addition, prompt assessment for an underlying B-cell lymphoma or clonal plasmacytic neoplasm is indicated.
目的
我们报告了 8 例伴有骨髓（BM）受累的晶体贮积性组织细胞增生症（CSH）的临床病理特征，并复习了英文文献中报道的 CSH 病例。
方法
我们在 2011 年 6 月至 2016 年 8 月的病理数据库中查询了最终诊断/评论中提到有 CSH 的 BM 病例。
结果
确定了 8 例 BM-CSH。潜在诊断主要为浆细胞疾病（88%），与单克隆 κ 轻链相关。在 BM 抽吸物中，组织细胞内的晶体表现出形态学谱，包括亮嗜酸性、针状或球状。在 BM 核心活检中，组织细胞常呈聚集状，有细胞内针状和/或球状、折射内含物。
结论
BM-CSH 是一种罕见现象，表现出异质性晶体和组织细胞外观，需要准确识别，以避免误诊为肉芽肿性疾病或贮积性疾病。此外，需要及时评估潜在的 B 细胞淋巴瘤或克隆性浆细胞肿瘤。
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