Johnston J B, Glazer R I, Pugh L, Israels L G
Br J Haematol. 1986 Jul;63(3):525-34. doi: 10.1111/j.1365-2141.1986.tb07530.x.
Eight patients with hairy-cell leukaemia (HCL) complicated by pancytopenia were treated with low dose regimens of the adenosine deaminase (ADA) inhibitor 2'-deoxycoformycin (DCF). All patients had significant haematological and clinical improvement. One patient who had been splenectomized and five patients with mild to moderate splenomegaly achieved normal blood counts within 2 months, which have been maintained for up to 18 months. Complete remissions occurred in two patients and four patients had 50-95% marrow clearance of hairy cells. The initial DCF treatments produced a 1-3 g/dl fall in the haemoglobin levels and one patient had a temporary reduction in granulocyte and platelet counts. Five patients had nausea/vomiting, and/or lethargy following DCF, but there was no correlation between the plasma levels of deoxyadenosine and adenosine and the incidence or severity of these side effects. An increased incidence of infection and drug hypersensitivity may reflect the effects of DCF on the immune system. Low dose DCF is a highly effective agent in HCL.
8例毛细胞白血病(HCL)合并全血细胞减少的患者接受了低剂量腺苷脱氨酶(ADA)抑制剂2'-脱氧助间型霉素(DCF)治疗。所有患者血液学指标和临床症状均有显著改善。1例脾切除患者和5例轻至中度脾肿大患者在2个月内血细胞计数恢复正常,并维持长达18个月。2例患者完全缓解,4例患者骨髓中毛细胞清除率达50%-95%。初始DCF治疗使血红蛋白水平下降1-3g/dl,1例患者粒细胞和血小板计数暂时降低。5例患者在DCF治疗后出现恶心/呕吐和/或嗜睡,但脱氧腺苷和腺苷的血浆水平与这些副作用的发生率或严重程度之间无相关性。感染和药物超敏反应发生率增加可能反映了DCF对免疫系统的影响。低剂量DCF是治疗HCL的高效药物。