Objective: To evaluate the reliability of flow cytometry (FCM) for diagnosing lymphoma associated hemophagocytic syndrome (LAHS). Method: The clinical data in 57 patients with hemophagocytic lymphohistiocytosis (HLH)were retrospective analyzed at Peking University Shenzhen Hospital from July 2010 to July 2019. All patients were performed bone marrow FCM and bone marrow pathological examination before final diagnoses were made. The golden diagnosis criterion was based on clinical, biochemical and histopathological evidence, which was regarded as the standard to evaluate the sensitivity and specificity of FCM analysis in diagnosing LAHS. Results: Among 57 cases, 36 cases were eventually diagnosed with LAHS, including 15 B-cell lymphoma(14 diffuse large B-cell lymphoma, 1 B-cell lymphoma with reactive T-cell hyperplasia), 13 aggressive NK/T cell lymphoma/leukemia, 2 cases of gamma-delta T-cell lymphoma, 4 angioimmunoblastic T-cell lymphoma, 1 enteropathy-associated peripheral T-cell lymphoma and 1 anaplastic T-cell lymphoma. Lymphoma cells in bone marrow were detected in all patients by FCM except one ENTCL patient. The sensitivity and the specificity of FCM in LASH compared to bone marrow biopsy were 97.2%(P=0.014)and 90.5%(P=0.488) respectively. In the other 21 non-LAHS patients, T cell receptor Vβ (TCRVβ) rearrangement was detected in 2 patients with Epstein-Barr virus (EBV) associated primary HLH. Conclusions: FCM effectively detects lymphoma cells in bone marrow of lymphoma patients with LHL, suggesting that FCM could be an important indicator for the diagnosis of LAHS. FCM also has the advantage in differentiating LAHS from other HLH.
目的： 应用流式细胞术（FCM）对骨髓淋巴瘤细胞特征性免疫表型进行分析，评估FCM对初诊以噬血细胞综合征（HLH）为首发临床表现的非霍奇金淋巴瘤的快速鉴别诊断价值。 方法： 回顾性分析2010年7月至2019年7月在北京大学深圳医院血液科收治的57例已经明确病因诊断的HLH患者的临床资料，并且所有患者在起病时至少完成1次骨髓流式细胞检测和骨髓病理检查，以同期患者的临床、生化及组织病理为金标准，评估FCM分析骨髓淋巴瘤细胞的免疫表型特征对淋巴瘤相关的噬血细胞综合征（LAHS）的诊断效能。 结果： 57例符合HLH诊断标准的患者，36例患者经临床、生化和组织病理最终明确诊断为LAHS，其中B细胞淋巴瘤15例（弥漫大B细胞淋巴瘤14例，B细胞淋巴瘤伴反应性T细胞增生1例）；侵袭性NK细胞淋巴瘤/白血病13例；肝脾γδT细胞淋巴瘤2例；血管免疫母T细胞淋巴瘤（AILT）4例；肠病性外周T细胞淋巴瘤（ENTCL）1例；间变性T细胞淋巴瘤（小细胞型）（ATCL）1例。除1例ENTCL外，FCM均在上述病例骨髓中发现有特征性免疫表型的淋巴瘤细胞，与骨髓疾病比较，FCM对本组LAHS病例诊断的敏感度为97.2%（P=0.014），特异度为90.5%（P=0.488）。在21例非LAHS患者中，FCM在2例原发EB病毒相关噬血细胞综合征检测出有TCRVβ重排，其他均未显示有异常特征性免疫表型。 结论： FCM对骨髓淋巴瘤细胞特征性免疫表型分析可作为诊断LAHS的重要指标，具有快速鉴别LAHS和非LAHS的优势，缩短了确诊时间，有利于临床作出早期诊断和快速处理。.
Keywords: Flow cytometry; Hemophagocytic lymphohistiocytosis; Immunophenotyping; Lymphoma.