BACKGROUND: Primary plasma cell leukemia (pPCL) is an aggressive plasma cell disorder characterized by circulating plasma cells and a poor prognosis. Although patients who have pPCL benefit from the use of stem cell transplantation (SCT) and novel agents, their prognosis remains inferior to that of patients who have myeloma. METHODS: This was a retrospective analysis of 38 consecutive patients with pPCL who were diagnosed between October 2005 and July 2016 and were registered in the Winship Cancer Institute of Emory University database. Baseline characteristics as well as data about treatment and survival outcomes were collected. RESULTS: The median patient age at diagnosis was 58 years. All patients received a bortezomib-based induction regimen, and 92% received both bortezomib and an immunomodulatory drug (thalidomide or lenalidomide); in addition, 74% of patients underwent autologous SCT (ASCT), and 61% received maintenance therapy. The best response to first-line therapy was a partial response or better in 87% of patients, and 45% had a complete response (CR). The achievement of ≥CR was a predictor for prolonged progression-free survival (PFS) and overall survival (OS). The median PFS was 20 months, and the median OS was 33 months. PFS was prolonged in patients who underwent ASCT compared with those who did not undergo ASCT (25 vs 6 months; P = .004), and patients who received maintenance therapy after ASCT had prolonged median PFS (27 vs 11 months; P = .03) and a trend toward prolonged OS (median, 38 vs 22 months; P = .06) compared with those who did not receive maintenance therapy. CONCLUSIONS: The current data support the use of regimens combining novel agents in the upfront treatment of patients with pPCL as well as the role of ASCT and maintenance therapy for long-term disease control.
BACKGROUND: Primary plasma cell leukemia (pPCL) is an aggressive plasma cell disorder characterized by circulating plasma cells and a poor prognosis. Although patients who have pPCL benefit from the use of stem cell transplantation (SCT) and novel agents, their prognosis remains inferior to that of patients who have myeloma. METHODS: This was a retrospective analysis of 38 consecutive patients with pPCL who were diagnosed between October 2005 and July 2016 and were registered in the Winship Cancer Institute of Emory University database. Baseline characteristics as well as data about treatment and survival outcomes were collected. RESULTS: The median patient age at diagnosis was 58 years. All patients received a bortezomib-based induction regimen, and 92% received both bortezomib and an immunomodulatory drug (thalidomide or lenalidomide); in addition, 74% of patients underwent autologous SCT (ASCT), and 61% received maintenance therapy. The best response to first-line therapy was a partial response or better in 87% of patients, and 45% had a complete response (CR). The achievement of ≥CR was a predictor for prolonged progression-free survival (PFS) and overall survival (OS). The median PFS was 20 months, and the median OS was 33 months. PFS was prolonged in patients who underwent ASCT compared with those who did not undergo ASCT (25 vs 6 months; P = .004), and patients who received maintenance therapy after ASCT had prolonged median PFS (27 vs 11 months; P = .03) and a trend toward prolonged OS (median, 38 vs 22 months; P = .06) compared with those who did not receive maintenance therapy. CONCLUSIONS: The current data support the use of regimens combining novel agents in the upfront treatment of patients with pPCL as well as the role of ASCT and maintenance therapy for long-term disease control.
Authors: Siegfried Janz; Fenghuang Zhan; Fumou Sun; Yan Cheng; Michael Pisano; Ye Yang; Hartmut Goldschmidt; Parameswaran Hari Journal: Front Genet Date: 2019-05-08 Impact factor: 4.599
Authors: Chakra P Chaulagain; Maria-Julia Diacovo; Amy Van; Felipe Martinez; Chieh-Lin Fu; Antonio Martin Jimenez Jimenez; Wesam Ahmed; Faiz Anwer Journal: Clin Med Insights Blood Disord Date: 2021-02-26
Authors: Mirian Brink; Otto Visser; Sonja Zweegman; Pieter Sonneveld; Annemiek Broyl; Niels W C J van de Donk; Avinash G Dinmohamed Journal: Blood Cancer J Date: 2021-02-04 Impact factor: 11.037
Authors: Carlos Fernández de Larrea; Robert Kyle; Laura Rosiñol; Bruno Paiva; Monika Engelhardt; Saad Usmani; Jo Caers; Wilson Gonsalves; Fredrik Schjesvold; Giampaolo Merlini; Suzanne Lentzch; Enrique Ocio; Laurent Garderet; Philippe Moreau; Pieter Sonneveld; Ashraf Badros; Gösta Gahrton; Hartmut Goldschmidt; Sascha Tuchman; Hermann Einsele; Brian Durie; Baldeep Wirk; Pellegrino Musto; Patrick Hayden; Martin Kaiser; Jesús San Miguel; Joan Bladé; S Vincent Rajkumar; Maria Victoria Mateos Journal: Blood Cancer J Date: 2021-12-02 Impact factor: 11.037