Hairy cell leukemia (HCL) is a rare lymphoproliferative disease with an excellent prognosis after treatment with cladribine (2CDA), although relapse may occur during follow-up. The aim of the study is to review the efficacy, safety, long-term remission rate, and overall survival (OS) in those patients who received 2CDA as first-line treatment. We retrospectively reviewed data of HCL patients treated with 2CDA between March 1991 and May 2019 at 18 Italian Hematological centers: 513 patients were evaluable for study purpose. The median age was 54 years (range 24–88) and ECOG was 0 in 84.9% of cases. A total of 330 (64.3%) patients received 2CDA intravenously and 183 (35.7%) subcutaneously. ORR was 91.8%: CR was obtained in 335 patients (65.3%), PR in 96 (18.7%), and hematological response in 40 (7.8%) patients; in 42 (8.2%) no response was observed. Hemoglobin value (p = 0.044), frequency of circulating hairy cells (p = 0.039), recovery of absolute neutrophil count (p = 0.006), and normalization of spleen (p ≤ 0.001) were associated with CR compared to PR in univariable analysis. At a median follow-up of 6.83 years (range 0.04–28.52), the median time to relapse was 12.2 years. A significant difference in duration of response was identified between patients that obtained a CR and PR (19.4 years versus 4.8 years, p < 0.0001). Non-hematological grade 3 or higher early toxicity was reported in 103 (20.1%) patients. Median OS was not reached: 95.3%, 92.4%, and 81.8% of patients were estimated to be alive at 5, 10, and 15 years, respectively. Forty-nine patients died (9.5%), following an infection in 14 cases (2.7%), natural causes in 14 (2.7%), cardiovascular events in 13 (2.5%), a second neoplasm in 6 (1.2%), and progression of HCL in 2 cases (0.4%). Following treatment of HCL with 2CDA, 80% of patients are estimated to be alive 15 years after diagnosis.

Pagano, L., Criscuolo, M., Broccoli, A., Piciocchi, A., Varettoni, M., Galli, E., Anastasia, A., Cantonetti, M., Trentin, L., Kovalchuk, S., Orsucci, L., Frustaci, A., Spolzino, A., Volpetti, S., Annibali, O., Storti, S., Stelitano, C., Marchesi, F., Offidani, M., Casadei, B., Nizzoli, M. E., De Luca, M. L., Fianchi, L., Motta, M., Guarnera, L., Simonetti, E., Visentin, A., Vassallo, F., Deodato, M., Sarlo, C., Olivieri, A., Falini, B., Pulsoni, A., Tiacci, E., Zinzani, P. L., Long-term follow-up of cladribine treatment in hairy cell leukemia: 30-year experience in a multicentric Italian study, <<BLOOD CANCER JOURNAL>>, 2022; 12 (7): 109-120. [doi:10.1038/s41408-022-00702-9] [https://hdl.handle.net/10807/223791]

Long-term follow-up of cladribine treatment in hairy cell leukemia: 30-year experience in a multicentric Italian study

Pagano, Livio
Writing – Review & Editing
;
Criscuolo, Marianna
Writing – Original Draft Preparation
;
Galli, Eugenio
Membro del Collaboration Group
;
Storti, Sergio
Membro del Collaboration Group
;
Fianchi, Luana
Membro del Collaboration Group
;
2022

Abstract

Hairy cell leukemia (HCL) is a rare lymphoproliferative disease with an excellent prognosis after treatment with cladribine (2CDA), although relapse may occur during follow-up. The aim of the study is to review the efficacy, safety, long-term remission rate, and overall survival (OS) in those patients who received 2CDA as first-line treatment. We retrospectively reviewed data of HCL patients treated with 2CDA between March 1991 and May 2019 at 18 Italian Hematological centers: 513 patients were evaluable for study purpose. The median age was 54 years (range 24–88) and ECOG was 0 in 84.9% of cases. A total of 330 (64.3%) patients received 2CDA intravenously and 183 (35.7%) subcutaneously. ORR was 91.8%: CR was obtained in 335 patients (65.3%), PR in 96 (18.7%), and hematological response in 40 (7.8%) patients; in 42 (8.2%) no response was observed. Hemoglobin value (p = 0.044), frequency of circulating hairy cells (p = 0.039), recovery of absolute neutrophil count (p = 0.006), and normalization of spleen (p ≤ 0.001) were associated with CR compared to PR in univariable analysis. At a median follow-up of 6.83 years (range 0.04–28.52), the median time to relapse was 12.2 years. A significant difference in duration of response was identified between patients that obtained a CR and PR (19.4 years versus 4.8 years, p < 0.0001). Non-hematological grade 3 or higher early toxicity was reported in 103 (20.1%) patients. Median OS was not reached: 95.3%, 92.4%, and 81.8% of patients were estimated to be alive at 5, 10, and 15 years, respectively. Forty-nine patients died (9.5%), following an infection in 14 cases (2.7%), natural causes in 14 (2.7%), cardiovascular events in 13 (2.5%), a second neoplasm in 6 (1.2%), and progression of HCL in 2 cases (0.4%). Following treatment of HCL with 2CDA, 80% of patients are estimated to be alive 15 years after diagnosis.
Inglese
Pagano, L., Criscuolo, M., Broccoli, A., Piciocchi, A., Varettoni, M., Galli, E., Anastasia, A., Cantonetti, M., Trentin, L., Kovalchuk, S., Orsucci, L., Frustaci, A., Spolzino, A., Volpetti, S., Annibali, O., Storti, S., Stelitano, C., Marchesi, F., Offidani, M., Casadei, B., Nizzoli, M. E., De Luca, M. L., Fianchi, L., Motta, M., Guarnera, L., Simonetti, E., Visentin, A., Vassallo, F., Deodato, M., Sarlo, C., Olivieri, A., Falini, B., Pulsoni, A., Tiacci, E., Zinzani, P. L., Long-term follow-up of cladribine treatment in hairy cell leukemia: 30-year experience in a multicentric Italian study, <<BLOOD CANCER JOURNAL>>, 2022; 12 (7): 109-120. [doi:10.1038/s41408-022-00702-9] [https://hdl.handle.net/10807/223791]
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