Tordjman R, Macintyre E, Emile J F, Valensi F, Ribrag V, Burtin M L, Varet B, Brousse N, Hermine O
Department of Clinical and Biological Hematology, Hopital Necker, Paris, France.
Leukemia. 1996 Sep;10(9):1514-9.
In the majority of clonal expansions of CD3+ large granular lymphocytes (LGL), referred to as T-LGL leukemia, patients have a chronic disease, often manifested by severe neutropenia, rheumatoid arthritis, and mild to moderate splenomegaly. The characteristic leukemic phenotype is CD3+, CD8+, CD16+, CD57+ and CD56-. Here we report an unusual case of T-LGL (CD3cyt+, CD3surface-, CD16+, CD56-) with clinicopathological features (acute presentation, large tumor mass, and systemic illness with highLGL counts at diagnosis) similar to those described for patients with CD3-natural killer (NK)-LGL leukemia. Two distinct stages of maturation arrest were observed: in the lymph node abnormal cells were CD4+, CD8+ whereas the majority of circulating leukemic cells expressed only CD8. TCR gamma (TCR gamma) gene configuration demonstrated that these originated from the same T cell clone, suggesting a maturation process between the two populations, or preferential passage of CD8 single positive cells into the blood.
在大多数被称为T-LGL白血病的CD3⁺大颗粒淋巴细胞（LGL）克隆性增殖中，患者患有慢性疾病，常表现为严重中性粒细胞减少、类风湿性关节炎以及轻度至中度脾肿大。特征性白血病表型为CD3⁺、CD8⁺、CD16⁺、CD57⁺和CD56⁻。在此，我们报告一例不寻常的T-LGL（CD3胞浆⁺、CD3表面⁻、CD16⁺、CD56⁺）病例，其临床病理特征（急性起病、巨大肿瘤肿块以及诊断时LGL计数高的全身疾病）与CD3自然杀伤（NK）-LGL白血病患者所描述的特征相似。观察到两个不同的成熟停滞阶段：在淋巴结中异常细胞为CD4⁺、CD8⁺，而大多数循环白血病细胞仅表达CD8。TCRγ（TCRγ）基因构型表明这些细胞起源于同一个T细胞克隆，提示这两个群体之间存在成熟过程，或者CD8单阳性细胞优先进入血液。
