Objective: To analyze the clinical manifestations and laboratory features in patients with myeloid neoplasms complicated with clonal T large granular lymphocyte (T-LGL) proliferation. Methods: The clinical data of 5 patients with myeloid neoplasms complicated with clonal T-LGL proliferation from November 2017 to November 2018 in Institute of Hematology & Blood Diseases Hospital, Chinese Academy of Medical Sciences & Peking Union Medical College were analyzed retrospectively. Results: The median age was 60 years old. All patients had a history of abnormal peripheral blood cell counts for over 6 months. The absolute lymphocyte count in peripheral blood was less than 1.0×10(9)/L. In addition to the typical T-LGL phenotype, the immunophenotype was heterogenous including CD4(+)CD8(-) in 2 patients, the other 3 CD4(-)CD8(+). Four patients were αβ type T cells, the other one was γδ type. STAT3 mutation was detected in 1 patient by next-generation sequencing, the other 4 cases were negative. Conclusions: Clonal T-LGL proliferation with myeloid neoplasm develops in an indolent manner, mainly in elderly patients. Hemocytopenia is the most common manifestation. The diagnosis of T-LGL proliferation does not have specific criteria, that it should be differentiated from other T cell proliferative disorders, such as T-cell clones of undetermined significance. STAT3 or STAT5b mutation may help distinguish.
目的: 探讨髓系肿瘤合并克隆性T大颗粒淋巴细胞(T-LGL)增殖的临床及实验室特征。 方法: 回顾性分析中国医学科学院血液病医院2017年11月至2018年11月收治的5例确诊髓系肿瘤合并克隆性T-LGL增殖患者的临床资料。 结果: 5例患者中位年龄60岁,均存在>6个月的血细胞异常病史。外周血T-LGL绝对计数均<1.0×10(9)/L,LGL免疫表型2例为CD4(+)CD8(-),3例为CD4(-)CD8(+);4例为αβ型T细胞,1例为γδ型T细胞;5例均有克隆性证据;二代测序检测显示1例患者存在STAT3突变,其余4例均为阴性。 结论: 5例髓系肿瘤合并克隆性T-LGL增殖患者起病隐匿,以老年为主,临床以慢性血细胞减少多见。存在克隆性T-LGL,尤其是T-LGL绝对计数<0.5×10(9)/L的患者诊断T大颗粒淋巴细胞白血病需慎重,可能与意义未明的T细胞克隆性疾病部分重叠。STAT3或STAT5b突变检测可能是鉴别两者的主要手段。.
Keywords: Clonal proliferation; Myeloid neoplasms; T large granular lymphocyte.