L Pagano1, C G Valentini2, V De Stefano2, A Venditti3, G Visani4, M T Petrucci5, A Candoni6, G Specchia7, C Visco8, E M Pogliani9, F Ferrara10, P Galieni11, A Gozzetti12, L Fianchi2, M De Muro13, G Leone2, P Musto14, A Pulsoni5. 1. Institute of Hematology, Catholic University, Rome. Electronic address: lpagano@rm.unicatt.it. 2. Institute of Hematology, Catholic University, Rome. 3. Division of Hematology, "Tor Vergata" University, Rome. 4. Hematology and Hematopoietic Stem Cell Transplant Center, San Salvatore Hospital, Pesaro. 5. Division of Hematology, Department of Cellular Biotechnologies and Hematology, University "La Sapienza," Rome. 6. Division of Hematology and Bone Marrow Transplantation, University of Udine, Udine. 7. Hematology Section, University of Bari, Bari. 8. Department of Hematology, Ospedale San Bortolo, Vicenza. 9. Department of Hematology, "San Gerardo" Hospital, Monza. 10. Division of Hematology and Stem Cell Transplantation Unit, Cardarelli Hospital, Naples. 11. Division of Hematology, Ascoli Piceno Hospital, Ascoli Piceno. 12. Department of Medicine and Immunological Sciences, Division of Hematology and Transplants, University of Siena, Siena. 13. Division of Hematology, Campus Biomedico University, Rome. 14. Unit of Hematology and Stem Cell Transplantation, IRCCS-CROB, Rionero in Vulture, Italy.
Abstract
BACKGROUND: Epidemiological and clinical information on primary plasma cell leukemia (pPCL) are rarely reported. The aims are to evaluate the clinical features, prognostic factors, and efficacy of treatments in pPCL. PATIENTS AND METHODS: A multicenter retrospective cohort study was carried out from January 2000 to December 2008 in 26 Italian hematology divisions. A total of 128 cases of plasma cell leukemia were collected, and 73 of them (57%) were classified as primary (male/female 43/30). RESULTS: Sixty-four patients had at least 1 sign of end-organ damage and 10 had extramedullary localization. One patient died early; of the remaining patients, 36 (50%) received anthracycline-based regimens as first-line therapy, 17 (24%) single alkylating agents, and 30 (42%) bortezomib or thalidomide as additional (n = 11) or unique treatments (n = 19). Twenty-three patients (31%) underwent autologous and/or allogeneic hematopoietic stem cell transplantation (HSCT). The median overall survival (OS) was 12.6 months; complete or partial response was achieved in 22 (30%) and 18 patients (25%), respectively; the median duration of response (DOR) was 16.4 months. HSCT patients had a longer OS and DOR (median 38.1 and 25.8 months, respectively) compared with nontransplanted patients (9.1 and 7.3 months, respectively, P < 0.001). OS was influenced by nonresponse to treatment, hypoalbuminemia, and HSCT. DOR was favorably influenced only by HSCT. CONCLUSIONS: pPCL is an aggressive disease with a poor prognosis and a low response rate to conventional therapy. HSCT is effective, increasing OS and DOR by 69% and 88%, respectively. The use of bortezomib and thalidomide may improve outcomes.
BACKGROUND: Epidemiological and clinical information on primary plasma cell leukemia (pPCL) are rarely reported. The aims are to evaluate the clinical features, prognostic factors, and efficacy of treatments in pPCL. PATIENTS AND METHODS: A multicenter retrospective cohort study was carried out from January 2000 to December 2008 in 26 Italian hematology divisions. A total of 128 cases of plasma cell leukemia were collected, and 73 of them (57%) were classified as primary (male/female 43/30). RESULTS: Sixty-four patients had at least 1 sign of end-organ damage and 10 had extramedullary localization. One patient died early; of the remaining patients, 36 (50%) received anthracycline-based regimens as first-line therapy, 17 (24%) single alkylating agents, and 30 (42%) bortezomib or thalidomide as additional (n = 11) or unique treatments (n = 19). Twenty-three patients (31%) underwent autologous and/or allogeneic hematopoietic stem cell transplantation (HSCT). The median overall survival (OS) was 12.6 months; complete or partial response was achieved in 22 (30%) and 18 patients (25%), respectively; the median duration of response (DOR) was 16.4 months. HSCT patients had a longer OS and DOR (median 38.1 and 25.8 months, respectively) compared with nontransplanted patients (9.1 and 7.3 months, respectively, P < 0.001). OS was influenced by nonresponse to treatment, hypoalbuminemia, and HSCT. DOR was favorably influenced only by HSCT. CONCLUSIONS: pPCL is an aggressive disease with a poor prognosis and a low response rate to conventional therapy. HSCT is effective, increasing OS and DOR by 69% and 88%, respectively. The use of bortezomib and thalidomide may improve outcomes.
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