We report a multicentre retrospective study that analysed clinical characteristics and outcomes in 117 patients with primary plasma cell leukaemia (pPCL) treated at the participating institutions between January 2006 and December 2016. The median age at the time of pPCL diagnosis was 61 years. Ninety-eight patients were treated with novel agents, with an overall response rate of 78%. Fifty-five patients (64%) patients underwent upfront autologous stem cell transplantation (ASCT). The median follow-up time was 50 months (95% confidence interval [CI] 33; 76), with a median overall survival (OS) for the entire group of 23 months (95% CI 15; 34). The median OS time in patients who underwent upfront ASCT was 35 months (95% CI 24·3; 46) as compared to 13 months (95% CI 6·3; 35·8) in patients who did not receive ASCT (P = 0·001). Multivariate analyses identified age ≥60 years, platelet count ≤100 × 109 /l and peripheral blood plasma cell count ≥20 × 109 /l as independent predictors of worse survival. The median OS in patients with 0, 1 or 2-3 of these risk factors was 46, 27 and 12 months, respectively (P < 0·001). Our findings support the use of novel agents and ASCT as frontline treatment in patients with pPCL. The constructed prognostic score should be independently validated.

4th Department of Medicine Haematology Charles University Hospital Hradec Kralove Hradec Kralove Czech Republic

Dana Farber Cancer Institute Harvard Medical School Boston MA USA

Department of Doctoral Studies School of Public Health in Bytom Medical University of Silesia Katowice Poland

Department of Experimental Haematology Medical University of Lublin Lublin Poland

Department of Haematology and Bone Marrow Transplantation Medical University of Lublin Lublin Poland

Department of Haematology Medical University of Lodz Copernicus Memorial Hospital Łódź Poland

Department of Haematology Nicolaus Copernicus Hospital Torun Poland

Department of Haematology Oncology and Internal Medicine Warsaw Medical University Warsaw Poland

Department of Haematology Pomeranian Medical University Szczecin Poland

Department of Haematology St John's Cancer Centre Lublin Poland

Department of Haematology State Hospital Cracow Poland

Department of Haematology State Hospital Opole Poland

Department of Haematology University of Rzeszów Rzeszów Poland

Department of Haematology UZ Leuven Leuven Belgium

Department of Medicine Section of Haematology Vejle Denmark

Dept of Internal Medicine and Haematology Military Institute of Medicine Warsaw Poland

Division of Hematology and Oncology Dana Farber Cancer Institute at St Elizabeth's Medical Center Brighton MA USA

Haematology Department and Bone Marrow Transplantation Unit Ankara University Medical School Ankara Turkey

Hospital Universitario de Salamanca Salamanca Spain

Jagiellonian University Medical College Cracow Poland

John Theurer Cancer Center at Hackensack University Medical Center Hackensack NJ USA

Le Scotte Hospital Siena Italy

Maria Sklodowska Curie Institute Oncology Centre Warsaw Poland

Massachusetts General Hospital Cancer Center Harvard Medical School Boston MA USA

Ministry of Interior Hospital in Olsztyn with Warmia and Masuria Oncology Centre Olsztyn Poland

Norwegian University of Science and Technology Trondheim Norway

Ospedale A Perrino Haemetaology Brindisi Italy

Rutgers New Jersey Medical School Newark NJ USA

Seccion Hematologia Adultos Hospital Italiano de Buenos Aires Buenos Aires Argentina

Service d'Hematologie CHU Hopital de la Miletrie Poitiers France

Tel Aviv Medical Centre Tel Aviv Israel

University Hospital Ostrava and Faculty of Medicine University of Ostrava Ostrava Czech Republic

Washington University School of Medicine Saint Louis MO USA

Weill Cornell Medical College New York NY USA

References provided by Crossref.org

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