Öngören Şeniz, Eşkazan Ahmet Emre, Berk Selin, Elverdi Tuğrul, Salihoğlu Ayşe, Ar Muhlis Cem, Başlar Zafer, Aydın Yıldız, Tüzüner Nükhet, Soysal Teoman
İstanbul University Cerrahpaşa Faculty of Medicine, Department of Hematology, İstanbul, Turkey.
İstanbul University Cerrahpaşa Faculty of Medicine, Department of Pathology, İstanbul, Turkey.
Turk J Haematol. 2017 Dec 1;34(4):291-299. doi: 10.4274/tjh.2016.0443.
In this study, we retrospectively analyzed the clinical outcome, treatment responses, infectious complications, and survival rates of 71 hairy cell leukemia (HCL) cases.
Sixty-seven patients received a first-line treatment and 2-chlorodeoxyadenosine (cladribine-2-CdA) was administered in 31 cases, 19 patients received interferon-alpha (INF-α), splenectomy was performed in 16 cases, and rituximab was used in one.
Although the highest overall response rate (ORR) was observed in patients receiving 2-CdA upfront, ORRs were comparable in the 2-CdA, INF-α, and splenectomy subgroups. Relapse rates were significantly lower in patients who received first-line 2-CdA. The progression-free survival (PFS) rate with 2-CdA was significantly higher than in patients with INF-α and splenectomy, but we found similar overall survival rates with all three upfront treatment modalities. Infections including tuberculosis were a major problem.
Although purine analogues have improved the ORRs and PFS, there is still much progress to make with regard to overall survival and relapsed/refractory disease in patients with HCL.
在本研究中，我们回顾性分析了71例毛细胞白血病（HCL）患者的临床结局、治疗反应、感染并发症及生存率。
67例患者接受一线治疗，31例使用2-氯脱氧腺苷（克拉屈滨-2-CdA），19例使用α干扰素（INF-α），16例行脾切除术，1例使用利妥昔单抗。
尽管接受2-CdA一线治疗的患者总体缓解率（ORR）最高，但2-CdA、INF-α和脾切除术亚组的ORR相当。接受一线2-CdA治疗的患者复发率显著更低。2-CdA治疗的无进展生存期（PFS）率显著高于INF-α和脾切除术治疗的患者，但我们发现三种一线治疗方式的总生存率相似。包括结核病在内的感染是一个主要问题。
尽管嘌呤类似物提高了ORR和PFS，但在HCL患者的总生存率及复发/难治性疾病方面仍有很大进展空间。
