TY - JOUR
T1 - Prognostic indicators in primary plasma cell leukaemia
T2 - a multicentre retrospective study of 117 patients
AU - Jurczyszyn, Artur
AU - Radocha, Jakub
AU - Davila, Julio
AU - Fiala, Mark A.
AU - Gozzetti, Alessandro
AU - Grząśko, Norbert
AU - Robak, Paweł
AU - Hus, Iwona
AU - Waszczuk-Gajda, Anna
AU - Guzicka-Kazimierczak, Renata
AU - Atilla, Erden
AU - Mele, Giuseppe
AU - Sawicki, Waldemar
AU - Jayabalan, David S.
AU - Charliński, Grzegorz
AU - Szabo, Agoston G.
AU - Hajek, Roman
AU - Delforge, Michel
AU - Kopacz, Agnieszka
AU - Fantl, Dorotea
AU - Waage, Anders
AU - Avivi, Irit
AU - Rodzaj, Marek
AU - Leleu, Xavier
AU - Richez, Valentine
AU - Knopińska-Posłuszny, Wanda
AU - Masternak, Anna
AU - Yee, Andrew J.
AU - Barchnicka, Agnieszka
AU - Druzd-Sitek, Agnieszka
AU - Guerrero-Garcia, Thomas
AU - Liu, Jieqi
AU - Vesole, David H.
AU - Castillo, Jorge J.
N1 - Publisher Copyright:
© 2018 John Wiley & Sons Ltd
PY - 2018/3
Y1 - 2018/3
N2 - 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.
AB - 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.
KW - myeloma
KW - plasma cell leukaemia
KW - prognosis
KW - survival
KW - therapeutic response
UR - http://www.scopus.com/inward/record.url?scp=85042936275&partnerID=8YFLogxK
U2 - 10.1111/bjh.15092
DO - 10.1111/bjh.15092
M3 - Article
C2 - 29315478
AN - SCOPUS:85042936275
SN - 0007-1048
VL - 180
SP - 831
EP - 839
JO - British Journal of Haematology
JF - British Journal of Haematology
IS - 6
ER -