流式免疫表型分析在11例侵袭性NK细胞白血病中的诊断价值分析

The diagnostic role of flow cytometry immunophenotyping analysis in 11 cases of aggressive NK-cell leukemia

摘要:

目的 探讨流式细胞术（flow cytometry，FCM）免疫分型及临床和实验室特征对侵袭性NK细胞白血病（aggressive NK-cell leukemia，ANKL）的诊断价值。方法 回顾性分析2019年1月至2019年12月在四川大学华西医院确诊的11例ANKL患者临床资料，包括骨髓细胞形态学、流式免疫表型及病理结果。结果 所有患者均出现发热症状并均伴噬血细胞淋巴组织细胞增生症（hemophagocytic lymphohistocytosis，HLH）。8例骨髓涂片中可见噬血细胞。流式细胞学示11例ANKL患者免疫表型均为：CD2⁺sCD3^-CD4^-CD8^-CD56⁺CD19^-，CD45和CD56表达强度增高（P=0.048）、细胞体积偏大（P=0.000），5例CDl6^-，6例CD7表达缺失或部分缺失。8例行免疫组化检测，结果均为：CD3ε⁺、粒酶B⁺及EBER1/2-ISH⁺。结论 ANKL的免疫表型有独特的特征，在ANKL的诊断和鉴别诊断中具有重要意义。综合分析病例的临床特点、细胞形态学、免疫表型等检查结果可实现对ANKL的及时诊断和鉴别诊断。

Abstract:

Objective To explore the diagnostic role of flow cytometry（FCM） immunophenotyping and the clinical and laboratory characteristics of aggressive NK-cell leukemia（ANKL）.Methods A retrospective analysis was performed on clinical data of 11 patients diagnosed of ANKL in West China Hospital from January 2019 to December 2019. Bone marrow cytomorphology，immunophenotype and pathological findings were collected and analyzed.Results All patients suffered from fever accompanied with hemophagocytic lymphohistiocytosis（HLH）. Hemophagocytic cells were found in bone marrow smears of 8 cases. FCM revealed an immunophenotype of CD2⁺sCD3^-CD4^-CD8^-CD56⁺CD19^- in all 11 cases of ANKL. The expression of CD45 and CD56 increased（P=0.048），and the volume of cells was relatively large（P=0.000）. CD16^- was found in 5 patients，and partial or complete loss of CD7 expression was identified in 6 patients. Immunohistochemical tests were performed on 8 patients，and the results were CD3ε⁺，granzyme B⁺，and EBER1/2-ISH⁺.Conclusion The immunophenotype of ANKL has unique characteristics and is of great significance in the diagnosis and differential diagnosis of ANKL. Timely and differential diagnosis of ANKL can be achieved by comprehensive analysis of clinical characteristics， bone marrow cell morphology，immunophenotype and immunohistochemistry.