Pasqualetti D, Cafaro A, Gastaldi R, Lopez M, Malagnino F, Manzari V, De Rossi G
Blut. 1987 May;54(5):289-98. doi: 10.1007/BF00320877.
A patient with Chronic Lymphocytic Leukemia (CLL) characterized by an expansion of helper phenotype mature T lymphocytes is here described. The phenotype of these cells was OKT3+, OKT4+, Leu 9+, 5/9+, OKT8-, Tac- and functional studies showed a strong helper activity on B cell differentiation; an "in vivo" presence of an IgG-lambda paraproteinaemia has been demonstrated. Cytogenetic studies showed multiple clonal, numerical and structural rearrangements which included a tandem t(14;14) (q11;32) translocation. Hybridization showed HTLV I related specific bands indicating the presence of exogenous sequences related to prototype virus but derived from a different Retrovirus (HTLV 1c). The clinical course was aggressive and unsuccessful treatments with various polichemotherapeutic protocols, associated with multiple leukaphereses, were performed. The authors underline that despite the morphological, immunological, biological and virological heterogeneity, the common feature of T-helper CLL is the inexorable clinical course which needs a new therapeutic approach.
本文描述了一名慢性淋巴细胞白血病(CLL)患者,其特征为辅助表型成熟T淋巴细胞扩增。这些细胞的表型为OKT3 +、OKT4 +、Leu 9 +、5/9 +、OKT8 -、Tac -,功能研究显示其对B细胞分化具有强大的辅助活性;已证实存在IgG-λ副蛋白血症的“体内”表现。细胞遗传学研究显示存在多种克隆性、数量和结构重排,其中包括串联t(14;14) (q11;32)易位。杂交显示与HTLV I相关的特异性条带,表明存在与原型病毒相关的外源序列,但源自不同的逆转录病毒(HTLV 1c)。临床病程进展迅速,采用了各种联合化疗方案并多次进行白细胞分离术,但治疗均未成功。作者强调,尽管在形态学、免疫学、生物学和病毒学方面存在异质性,但T辅助性CLL的共同特征是无情的临床病程,需要新的治疗方法。