Department of Internal Medicine, The Arthur G. James and Richard Solove Cancer Hospital and Research Institute, The Ohio State University, Columbus, Ohio, USA.
Leuk Lymphoma. 2011 Jun;52 Suppl 2:50-2. doi: 10.3109/10428194.2011.570819. Epub 2011 Apr 19.
The natural history of hairy cell leukemia (HCL) includes frequent and potentially life-threatening infections. Prior to the development of effective therapy, the incidence in patients followed for several years was as high as 60%, with infection as a prime cause of death in patients. Studies of the immune system of patients with HCL identified several potential reasons, including profound neutropenia and monocytopenia. In addition, treatment including chemotherapy and splenectomy further compromised the immune system. The success of new therapies has changed the frequency and severity of infections in patients with HCL. During the initial phase of treatment, however, infection risk remains high, with incidence ranging from 30 to 50%. Attempts to ameliorate the risk with growth factors in conjunction with treatment have not been successful, but lower doses of drugs and/or combination therapy have been tried with reported success. In the majority of patients, successful therapy results in normalization of the neutrophil count and marked reduction in the severity and frequency of infections. Interestingly, after purine nucleoside treatment, there is profound depression of CD4+ cells without development of the opportunistic infections seen with patients with human immunodeficiency virus (HIV). Studies to reduce morbidity and mortality should focus on initial induction regimens, as well as confirming the long-term benefit of treatment on risk of infection.
毛细胞白血病（HCL）的自然病程包括频繁且可能危及生命的感染。在开发出有效的治疗方法之前，经过数年随访的患者发病率高达 60%，感染是患者死亡的主要原因。对 HCL 患者免疫系统的研究发现了几个潜在的原因，包括严重的中性粒细胞减少症和单核细胞减少症。此外，包括化疗和脾切除术在内的治疗方法进一步损害了免疫系统。新疗法的成功改变了 HCL 患者感染的频率和严重程度。然而，在治疗的初始阶段，感染风险仍然很高，发生率为 30%至 50%。尽管尝试使用生长因子联合治疗来减轻这种风险，但并未成功，但已尝试使用较低剂量的药物和/或联合疗法，并取得了成功的报道。在大多数患者中，成功的治疗可导致中性粒细胞计数正常化，并显著减少感染的严重程度和频率。有趣的是，在用嘌呤核苷治疗后，CD4+细胞受到严重抑制，而不会出现人类免疫缺陷病毒（HIV）患者中出现的机会性感染。降低发病率和死亡率的研究应集中在初始诱导方案上，并确认治疗对感染风险的长期益处。
