Bohn Jan-Paul, Gastl Guenther, Steurer Michael
Department of Internal Medicine V, Medical University of Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.
Memo. 2016;9:63-65. doi: 10.1007/s12254-016-0269-1. Epub 2016 Jun 17.
Classic hairy cell leukemia (HCL) is a rare indolent B‑cell-lymphoproliferative disorder, first described as a distinct disease entity in 1958. After more than two decades without effective chemotherapeutic options and a dismal prognosis of less than 5 years, only the introduction of interferon‑α (IFN‑α) allowed for response rates between 80-90 % and survival improvement. Nowadays, however, patients are rarely treated with IFN-α as purine analogues were found to be highly effective in HCL facilitating a near normal life span in most cases. Moreover, novel therapeutic tools for patients with relapsed or refractory disease after purine analogues have emerged such as rituximab and, more recently, vemurafenib. In the absence of long-term safety data for these novel agents, however, IFN-α may still represent a viable therapeutic option when the profound immunosuppressive side effects of purine analogues are to be avoided. We herein report a HCL patient, who has received multiple lines of therapy, including pentostatin, cladribine, and a total of 164 months of treatment with IFN‑α yielding long-term disease control. Our case illustrates that long-term administration of IFN-α with adequate dose-adjustments according to toxicity and disease activity is feasible in HCL and may still be a viable therapeutic option when purine analogues are considered unsuitable.
经典型毛细胞白血病(HCL)是一种罕见的惰性B细胞淋巴增殖性疾病,于1958年首次被描述为一种独特的疾病实体。在二十多年没有有效的化疗方案且预后不到5年的情况下,只有引入干扰素-α(IFN-α)才使缓解率达到80%至90%,并改善了生存率。然而如今,由于发现嘌呤类似物在HCL中非常有效,能使大多数患者的寿命接近正常,因此很少有患者接受IFN-α治疗。此外,针对嘌呤类似物治疗后复发或难治性疾病的患者,已出现了新的治疗手段,如利妥昔单抗,以及最近的维莫非尼。然而,由于缺乏这些新型药物的长期安全性数据,当需要避免嘌呤类似物严重的免疫抑制副作用时,IFN-α可能仍然是一种可行的治疗选择。我们在此报告一例HCL患者,该患者接受了多线治疗,包括喷司他丁、克拉屈滨,以及总共164个月的IFN-α治疗,实现了长期疾病控制。我们的病例表明,在HCL中根据毒性和疾病活动情况进行适当剂量调整后长期使用IFN-α是可行的,并且当认为嘌呤类似物不合适时,它可能仍然是一种可行的治疗选择。