Yılmaz Fergün, Atilla Dilan, Akkaş Nagihan, Bülbül Hale, Soyer Nur, Demir Derya, Kiper Demet, Avcı Aylin, Vural Filiz, Saydam Güray, Şahin Fahri, Hekimgil Mine, Özsan Nazan, Durusoy Raika, Payzın Bahriye
1Department of Hematology, Internal Medicine, Marmara University, Istanbul, Turkey.
2Department of Hematology, Internal Medicine, Ege University, Izmir, Turkey.
Indian J Hematol Blood Transfus. 2019 Oct;35(4):692-698. doi: 10.1007/s12288-019-01132-7. Epub 2019 May 13.
We aimed to analyze the characteristics and response rates of different treatment modalities in hairy cell leukemia patients over 20 diagnosed as hairy cell leukemia (HCL). Clinical data, response rates and survival outcome of the patients who were diagnosed with HCL were retrospectively analyzed. Fifty-two patients with a median age of 50 (28-87) years were enrolled in the study. 38 patients (73%) were male and male to female ratio was 2.7. First line therapy was cladrabine in 36 patients (69.2%). The overall response rate was 97%. CR and PR rates were 86.1% and 11.1%, respectively. Interferon was used in 10(19.2%) patients who were diagnosed before 2000s years. CR and PR rates were 70% and 30%, respectively. Although the CR rates were lower in IFN group, this difference could not be reached statistically significance ( = 0.24). The median follow up was 48 months (12-252). The median OS was not reached and median PFS was 150 months (95% CI, 116-214). The OS at 36 and 48 months were 95.9% and 92.3%, respectively and the PFS at 36 and 48 months were 90.2% and 83.4%, respectively. After the introduction of purine analogues, the fate of the HCL patients have been changed. Cladrabin achieved very high response rates in both young and older patients, in our study. Although relapse still constitutes a problem, another single dose of cladrabine results in good response rates.
我们旨在分析20例以上诊断为毛细胞白血病(HCL)的患者中不同治疗方式的特点和缓解率。对诊断为HCL的患者的临床资料、缓解率和生存结果进行回顾性分析。52例患者纳入研究,中位年龄50(28 - 87)岁。38例(73%)为男性,男女比例为2.7。36例(69.2%)患者一线治疗采用克拉屈滨。总缓解率为97%。完全缓解(CR)率和部分缓解(PR)率分别为86.1%和11.1%。10例(19.2%)在21世纪前诊断的患者使用了干扰素。CR率和PR率分别为70%和30%。虽然干扰素组的CR率较低,但这种差异未达到统计学意义(P = 0.24)。中位随访时间为48个月(12 - 252个月)。中位总生存期未达到,中位无进展生存期为150个月(95%置信区间,116 - 214)。36个月和48个月时的总生存率分别为95.9%和92.3%,36个月和48个月时的无进展生存率分别为90.2%和83.4%。嘌呤类似物引入后,HCL患者的预后发生了改变。在我们的研究中,克拉屈滨在年轻和老年患者中均取得了非常高的缓解率。虽然复发仍然是一个问题,但再次给予单剂量克拉屈滨仍可获得良好的缓解率。