Retrospective evaluation of hairy cell leukemia patients treated with 3 different first-line treatment modalities in the last two decades: a single center experience

Abstract

Öz AbstractAmaç: Bu çalışmada, geriye dönük olarak 71 tüylü hücreli lösemi (SHL) olgusunu klinik sonuçlar, tedavi yanıtları, enfeksiyon komplikasyonları ve sağkalım oranları açısından analiz ettik. Gereç ve Yöntemler:Altmış yedi hasta birinci basamak tedavi almıştı ve 2-klorodeoksiadenozin (kladribin-2-CdA) 31 olguya uygulanmış, 19 hasta interferon-alfa (INF-α) almış, splenektomi 16 olguda uygulanmış ve rituksimab ise bir hastada kullanılmıştı. Bulgular:En yüksek toplam yanıt oranı (TYO) birinci basamak 2-CdA alan hastalarda görülmüş olsa da, TYO'lar 2-CdA, INF-α ve splenektomi alt gruplarında benzerdi. Nüks oranları birinci basamak 2-CdA alan hastalarda anlamlı olarak daha azdı. Progresyonsuz sağkalım (PS) oranı 2-CdA alanlarda INF-α ve splenektomi hastalarına göre anlamlı olarak daha yüksek olmakla birlikte, her üç birinci basamak tedavi yaklaşımı ile toplam sağkalım (TS) oranları benzer olarak bulundu. Tüberkülozun da dahil olduğu enfeksiyonlar önemli bir problemdi. Sonuç:Her ne kadar pürin analogları TYO ve PS'yi iyileştirmiş olsa da, SHL hastalarında TS ve nüks/dirençli hastalık açısından daha yapılması gereken çok şey vardır. Anahtar Sözcükler: Kladribin, Tüylü hücreli lösemi, İnterferon, SplenektomiObjective: In this study, we retrospectively analyzed the clinical outcome, treatment responses, infectious complications, and survival rates of 71 hairy cell leukemia (HCL) cases. Materials and Methods: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.Results: 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. Conclusion: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.

Time to Cure Hairy Cell Leukemia

“…Another disadvantage that should be carefully considered is the high rate of PA toxicity, which includes bone marrow suppression associated with neutropenia, lymphopenia, T-cell dysfunction, or stem cell toxicity with the development of hypoplasia and aplasia [ 5 , 6 , 9 , 10 ]. Similar complications were also described in the study by Öngören et al [ 3 ].…”

“…In conclusion, as reported by Öngören et al [ 3 ], PAs remain the most effective treatment for classic HCL in terms of PFS and ORR when compared to earlier therapies such as IFN-α or splenectomy, and this approach has not been changed over the past 30 years. Perhaps the time has arrived to challenge this approach and improve the outcome of HCL by using PAs in combination with some of the available biological agents, either as frontline or consolidation therapy for patients with classical HCL, in an attempt to cure this chronic neoplastic disorder.…”

“…In the current issue of this journal, Öngören et al [ 3 ] report a retrospective analysis of 67 patients treated for classic HCL and compare 3 different first-line treatment modalities. Among them, 31 patients received cladribine therapy, 19 received interferon-alpha (IFN-α), 16 underwent splenectomy, and 1 was treated with rituximab monotherapy.…”

Treatment Outcomes of Patients With Hairy Cell Leukaemia; A 16-Year Experience at a Tertiary Care Center in Pakistan

“…Rosenberg et al stated a CR of 88%, where 88 patients were treated with cladribine. 17 A number of other studies have reported very promising response rate with cladribine, reporting an overall response (OR) and CR rates ranging from 75 to 100% and 72 to 98%, respectively. 12,18 Our study showed a CR rate of 92%, which is in concordance with reported international studies.…”