PMID- 34245477 OWN - NLM STAT- MEDLINE DCOM- 20210927 LR - 20210927 IS - 1096-8652 (Electronic) IS - 0361-8609 (Linking) VI - 96 IP - 10 DP - 2021 Oct 1 TI - The treatment of hairy cell leukemia with a focus on long lasting responses to cladribine: A 30-year experience. PG - 1204-1210 LID - 10.1002/ajh.26287 [doi] AB - The treatment of hairy cell leukemia (HCL) has considerably changed over years. Purine analogues, namely cladribine, now represent the treatment of choice. One hundred and eighty-four patients were followed between 1986 and 2018 and treated according to era-specific guidelines. Responses were classified by combining Consensus Resolution criteria and marrow immunohistochemistry. Patients were grouped according to the number of treatment lines they received. Patients treated first line responded in 86% of cases, with complete response (CR) in 44% of cases. Response rates remained high throughout the first four lines (84%, 81%, 79% for the second line onward, with CR in 38%, 37%, 15% of cases respectively). One hundred and twenty-two patients received cladribine as first line treatment, with a response rate of 86% and a CR rate of 54%. Among the 66 CR patients, 45 (68%) have never received further therapy: 11 patients are in continuous CR between 5 and 10 years after treatment, 14 between 10 and 20 years and three patients at more than 20 years. Median time-to-next treatment (TTNT) for frontline cladribine-treated patients was 8.2 years: partial responders had a significantly shorter median TTNT than CR patients (5.3 years vs median not reached at 25.8 years, p < 0.001). Patients with HCL require subsequent lines of therapy in more than 50% of cases. Purine analogues allow significant response rates when applied first line and upon retreatment. Some patients may enjoy long lasting treatment-free intervals after one course of cladribine. CI - (c) 2021 Wiley Periodicals LLC. FAU - Broccoli, Alessandro AU - Broccoli A AUID- ORCID: 0000-0001-5633-7313 AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Argnani, Lisa AU - Argnani L AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Nanni, Laura AU - Nanni L AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Terragna, Carolina AU - Terragna C AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. FAU - Sabattini, Elena AU - Sabattini E AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. FAU - Gabrielli, Giulia AU - Gabrielli G AD - A.O.U. Citta della Salute e della Scienza di Torino, University of Torino, Torino, Italy. FAU - Stefoni, Vittorio AU - Stefoni V AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Pellegrini, Cinzia AU - Pellegrini C AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. FAU - Casadei, Beatrice AU - Casadei B AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Morigi, Alice AU - Morigi A AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Lolli, Ginevra AU - Lolli G AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Carella, Matteo AU - Carella M AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Coppola, Paolo Elia AU - Coppola PE AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. FAU - Zinzani, Pier Luigi AU - Zinzani PL AUID- ORCID: 0000-0002-2112-2651 AD - IRCCS Azienda Ospedaliero-Universitaria di Bologna, Istituto di Ematologia "Seragnoli", Bologna, Italy. AD - Dipartimento di Medicina Specialistica, Diagnostica e Sperimentale, Universita degli Studi, Bologna, Italy. LA - eng PT - Journal Article DEP - 20210723 PL - United States TA - Am J Hematol JT - American journal of hematology JID - 7610369 RN - 0 (Antineoplastic Agents) RN - 47M74X9YT5 (Cladribine) SB - IM MH - Adult MH - Aged MH - Aged, 80 and over MH - Antineoplastic Agents/*therapeutic use MH - Cladribine/*therapeutic use MH - Female MH - Follow-Up Studies MH - Humans MH - Leukemia, Hairy Cell/*drug therapy/pathology MH - Male MH - Middle Aged MH - Treatment Outcome EDAT- 2021/07/11 06:00 MHDA- 2021/09/28 06:00 CRDT- 2021/07/10 12:16 PHST- 2021/06/28 00:00 [revised] PHST- 2021/05/05 00:00 [received] PHST- 2021/06/29 00:00 [accepted] PHST- 2021/07/11 06:00 [pubmed] PHST- 2021/09/28 06:00 [medline] PHST- 2021/07/10 12:16 [entrez] AID - 10.1002/ajh.26287 [doi] PST - ppublish SO - Am J Hematol. 2021 Oct 1;96(10):1204-1210. doi: 10.1002/ajh.26287. Epub 2021 Jul 23.