γδT细胞大颗粒淋巴细胞白血病17例临床及实验室表现

Clinical and laboratory analysis of 17 patients withγδT-cell large granular lymphocyte leukemia

目的比较γδT细胞大颗粒淋巴细胞白血病(γδT-LGLL)与αβT细胞大颗粒淋巴细胞白血病(αβT-LGLL)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贫血诊疗中心2009年1月至2019年1月17例γδT-LGLL患者的临床及实验室结果,与同时期91例αβT-LGLL患者进行对比。结果17例γδT-LGLL患者中位年龄54(25~73)岁,10例就诊原因为贫血。γδT-LGLL与αβT-LGLL一致,脾大(41%和44%)较为常见,肝大(12%和5%)及淋巴结肿大(6%和8%)较为少见;两者均有较高的抗核抗体阳性率(59%和45%),较低的风湿因子阳性率(6%和10%);两者中性粒细胞绝对值、淋巴细胞绝对值、HGB及PLT差异均无统计学意义(P值均>0.05)。γδT-LGLL患者的典型免疫分型为CD3+/CD4?/CD8?/CD57+/TCRγδ+,CD4?/CD8?双阴性表型显著多于αβT-LGLL患者(P<0.001)。17例γδT-LGLL患者有1例口服泼尼松治疗,3例口服环孢素A治疗,13例口服环孢素A联合泼尼松治疗,治疗4个月后2例获得完全缓解,4例获得部分缓解,总体有效率为35%。结论γδT-LGLL是一种少见的成熟T淋巴细胞增殖性疾病,其临床及实验室表现与αβT-LGLL相比除CD4?/CD8?双阴性表型外均无显著差异。环孢素A可作为γδT-LGLL的首选治疗药物。

Objective To compare the difference of the clinical and laboratory characteristics betweenγδT-cell large granular lymphocyte leukemia(γδT-LGLL)andαβT-cell large granular lymphocyte leukemia(αβT-LGLL).Methods The clinical and laboratory characteristics of 17 patients withγδT-LGLL and 91 patients withαβT-LGLL in the department of therapeutic center of anemia of enrolled in our hospital from January 2009 to January 2019 were retrospectively analyzed.Results The median age of the 17 patients withγδT-LGLL was 54 years(range,25-73 years),the most common presenting symptom was anemia.In comparison withαβT-LGLL patients,splenomegaly was common(41%and 44%,respectively),whereas hepatomegaly(12%and 5%,respectively)and lymphadenopathy(6%and 8%,respectively)were rare.The positive rates of antinuclear antibody(59%and 45%,respectively)were high,whereas the positive rates of rheumatoid factor(6%and 10%,respectively)were rare for both groups.There were no differences on peripheral blood counts between the two groups.However,γδT-LGLL patients were found to be predominantly expressed a CD4?/CD8?phenotype.Steroid therapy with prednisone was used alone as first-line therapy for 1 patient.Cyclosporin A(CsA)was used alone as first-line therapy for 3 patients.CsA in combination with steroids were administered in 13 patients.After 4 months treatment,2 patients acquired complete response,4 patients acquired partial response,the overall response was 35%.ConclusionγδT-LGLL is a rare mature T-lymphocyte proliferative disease.Clinical and laboratory characteristics were quite similar forγδT-LGLL in compare withαβT-LGLL.γδTLGLL predominantly expressed a CD4?/CD8?phenotype.The data presented here indicate the CsA is an effective option for the first-line treatment ofγδT-LGLL.