Murphy S B, Stass S, Kalwinsky D, Rivera G
Br J Haematol. 1983 Oct;55(2):285-93. doi: 10.1111/j.1365-2141.1983.tb01249.x.
A 6-year-old boy with T-cell acute lymphoblastic leukaemia (ALL) in relapse was treated with the adenosine deaminase inhibitor, 2'-deoxycoformycin (DCF). Remarkably, his residual leukaemia underwent an abrupt phenotypic shift, coincident with a massive anti-leukaemic effect of DCF. Both at diagnosis and prior to therapy with DCF, blast cells had typical lymphoblastic morphology and T-cell characteristics (terminal transferase +, T-antigen +, Ia -, cALLa -, myeloperoxidase -, and high in adenosine deaminase content). After four courses of DCF by constant infusion, the blast cells were myeloid in appearance and reactivity to a variety of tests (terminal transferase -, myeloperoxidase +, Sudan black B +, esterase +, My-1 +). We hypothesize that DCF therapy created a selection pressure, blocking pathways of T-cell differentiation and proliferation, permitting the emergence of a newly dominant myeloid subclone of a multipotential leukaemic cell progenitor with the innate capacity for both T-lymphocytic and myeloid differentiation.
一名复发的T细胞急性淋巴细胞白血病(ALL)6岁男孩接受了腺苷脱氨酶抑制剂2'-脱氧助间型霉素(DCF)治疗。值得注意的是,他残留的白血病发生了突然的表型转变,这与DCF的巨大抗白血病作用同时出现。在诊断时以及使用DCF治疗之前,原始细胞具有典型的淋巴细胞形态和T细胞特征(末端转移酶阳性、T抗原阳性、Ia阴性、普通急性淋巴细胞白血病抗原阴性、髓过氧化物酶阴性,腺苷脱氨酶含量高)。通过持续输注进行四个疗程的DCF治疗后,原始细胞在外观上呈髓样,并且对各种检测有反应(末端转移酶阴性、髓过氧化物酶阳性、苏丹黑B阳性、酯酶阳性、My-1阳性)。我们推测,DCF治疗产生了选择压力,阻断了T细胞分化和增殖途径,使得具有T淋巴细胞和髓样分化先天能力的多能白血病细胞祖细胞中新的优势髓样亚克隆得以出现。