Bourgault-Rouxel A S, Loughran T P, Zambello R, Epling-Burnette P K, Semenzato G, Donadieu J, Amiot L, Fest T, Lamy T
Service d'Hématologie Clinique, Rennes, France.
Leuk Res. 2008 Jan;32(1):45-8. doi: 10.1016/j.leukres.2007.04.011. Epub 2007 Jun 1.
We report on the clinico-biological characteristics of 20 cases of gammadelta T cell large granular lymphocyte (LGL) leukemia. All the data were compared to that of 196 cases with alphabeta T cell subtype, which represents the majority of T cell LGL leukemias. Clinical findings were quite similar in the two groups regarding age, sex ratio, recurrent infections, and association with auto-immune diseases especially rheumatoid arthritis. Gammadelta LGL predominantly expressed a CD3+/CD4-/CD8+/CD16+/CD57+ phenotype, in 50% of cases. Clinical outcome was favorable for these patients with overall survival of 85% at 3 years. Fifty percent of gammadelta patients required treatment and the response to therapy was estimated at 55%. gammadelta and alphabeta T cell LGL leukemia harbor a very similar clinico-biological behavior and represent part of an antigen-driven T cell lymphoproliferation.
我们报告了20例γδT细胞大颗粒淋巴细胞(LGL)白血病的临床生物学特征。所有数据均与196例αβT细胞亚型病例的数据进行了比较,αβT细胞亚型代表了大多数T细胞LGL白血病。两组在年龄、性别比例、反复感染以及与自身免疫性疾病尤其是类风湿性关节炎的关联方面,临床发现非常相似。γδLGL在50%的病例中主要表达CD3+/CD4-/CD8+/CD16+/CD57+表型。这些患者的临床结局良好,3年总生存率为85%。50%的γδ患者需要治疗,对治疗的反应估计为55%。γδ和αβT细胞LGL白血病具有非常相似的临床生物学行为,是抗原驱动的T细胞淋巴增殖的一部分。