Bastion Y, Campos L, Roubi N, Bienvenu J, Felman P, Dumontet C, Coiffier B
Service d'Hématologie, Centre Hospitalier Lyon-Sud, Pierre-Bénite, France.
Br J Haematol. 1995 Feb;89(2):413-6. doi: 10.1111/j.1365-2141.1995.tb03320.x.
In most patients with Diamond-Blackfan anaemia (DBA) the addition of IL-3 to in vitro cultures increases the number of erythroid progenitors. However, a small proportion of patients show an erythroid response to in vivo administration of IL-3. We treated two adult patients with corticosteroid-refractory DBA, who had shown a clear in vitro erythroid response to IL-3, with increasing doses of IL-3 (from 2.5 to 10 micrograms/kg/d) by subcutaneous injections for 7 and 9 weeks. Monitoring of marrow and circulating progenitors showed a sustained elevation of BFU-E and CFU-E as well as an increase of other progenitors and CD34+ cells during therapy. However, this effect did not translate into a clear clinical response, although transfusion requirements decreased transiently in one patient. This report shows that a sustained elevation of erythroid progenitors induced by in vivo IL-3 administration may not translate into an increase of mature red cells production in DBA patients.
在大多数先天性纯红细胞再生障碍性贫血（DBA）患者中，体外培养时添加白细胞介素-3（IL-3）可增加红系祖细胞数量。然而，一小部分患者对体内给予IL-3有红系反应。我们对两名患有皮质类固醇难治性DBA的成年患者进行了治疗，这两名患者在体外对IL-3有明显的红系反应，通过皮下注射给予递增剂量的IL-3（从2.5至10微克/千克/天），持续7周和9周。对骨髓和循环祖细胞的监测显示，治疗期间爆式红系集落形成单位（BFU-E）和红系集落形成单位（CFU-E）持续升高，其他祖细胞和CD34+细胞也增加。然而，尽管一名患者的输血需求短暂减少，但这种效应并未转化为明显的临床反应。本报告表明，体内给予IL-3诱导的红系祖细胞持续升高可能不会转化为DBA患者成熟红细胞生成的增加。
