Matutes E, Brito-Babapulle V, Swansbury J, Ellis J, Morilla R, Dearden C, Sempere A, Catovsky D
Academic Department of Haematology and Cytogenetics, Royal Marsden Hospital, London, UK.
Blood. 1991 Dec 15;78(12):3269-74.
We describe the clinical and laboratory findings of 78 adult patients with T-prolymphocytic leukemia (T-PLL) studied over the last 12 years. The main disease features were splenomegaly (73%), lymphadenopathy (53%), hepatomegaly (40%), skin lesions (27%), and a high leukocyte count (greater than 100 x 10(9)/L in 75%) with nucleolated prolymphocytes. A variant form with small, less typical cells was recognized in 19%. Membrane markers defined a postthymic phenotype TdT-, CD2+, CD3+, CD5+, CD7+; in 65%, the cells were CD4+ CD8-, in 21%, they coexpressed CD4 and CD8, and, in 13%, they were CD4- CD8+. Serology for human T-cell leukemia/lymphoma virus Type-I (HTLV-I) was negative in the 27 cases investigated. Cytogenetic analysis in 30 cases showed a consistent abnormality of chromosome 14, usually inv (14), with breakpoints at q11 and q32 in 76% of cases. Trisomy 8, including iso8q, was shown in 53%; t (11;14)(q13;q32) was documented in one case; and one had a normal karyotype. The clinical course was progressive with a median survival of 7.5 months. Thirty-one patients were treated with 2' deoxycoformycin and 15 responded (3 complete remissions and 12 partial remissions); the response rate (48%) increased to 58% in patients with a CD4+ CD8- phenotype. The median survival of responders was 16 months and of nonresponders 10 months; other treatments were less effective. T-PLL is a distinct clinico-pathologic entity with aggressive course and characteristic chromosome abnormalities. A subgroup of patients may benefit from deoxycoformycin.
我们描述了过去12年中研究的78例成年T-原淋巴细胞白血病（T-PLL）患者的临床和实验室检查结果。主要疾病特征为脾肿大（73%）、淋巴结病（53%）、肝肿大（40%）、皮肤病变（27%）以及白细胞计数高（75%的患者白细胞计数大于100×10⁹/L）且有核仁原淋巴细胞。19%的患者表现为一种具有小的、不太典型细胞的变异型。膜标志物确定为胸腺后表型TdT-、CD2+、CD3+、CD5+、CD7+；65%的细胞为CD4+ CD8-，21%的细胞共表达CD4和CD8，13%的细胞为CD4- CD8+。在27例接受检测的患者中，人类T细胞白血病/淋巴瘤病毒I型（HTLV-I）血清学检测为阴性。30例患者的细胞遗传学分析显示14号染色体存在一致的异常，通常为inv(14)，76%的病例在q11和q32处有断点。53%的病例显示8号染色体三体，包括等臂8q；1例记录有t(11;14)(q13;q32)；1例核型正常。临床病程呈进行性，中位生存期为7.5个月。31例患者接受了2'-脱氧助间型霉素治疗，15例有反应（3例完全缓解，12例部分缓解）；CD4+ CD8-表型的患者缓解率（48%）增至58%。有反应者的中位生存期为16个月，无反应者为10个月；其他治疗效果较差。T-PLL是一种具有侵袭性病程和特征性染色体异常的独特临床病理实体。一部分患者可能从脱氧助间型霉素治疗中获益。
