OBJECTIVE: To evaluate the clinical outcomes of patients with primary plasma cell leukemia (pPCL) defined by 5% or greater clonal circulating plasma cells on peripheral blood smear and treated with novel agent induction therapies. PATIENTS AND METHODS: A cohort of 68 patients with pPCL diagnosed at the Mayo Clinic in Rochester, Minnesota, from January 1, 2000, to December 31, 2019, and treated with novel agent induction therapies was evaluated. RESULTS: The median follow-up was 46 (95% CI, 41 to 90) months. The median bone marrow plasma cell content was 85% (range, 10% to 100%) and median clonal circulaitng plasma cell percentage on the peripheral blood smear was 26% (range, 5% to 93%). There was a preponderance of t(11;14) primary cytogenetic abnormality in this cohort. The median time to next therapy (TTNT) and overall survival (OS) for all patients with pPCL patients in this cohort was 13 (95% CI, 9 to 17) and 23 (95% CI, 19 to 38) months, respectively. However, when stratified by cytogenetic risk, the median TTNT and OS were 16 and 51 months for standard risk vs 9 and 19 months for high risk (P=.01 for OS). CONCLUSION: Primary plasma cell leukemia remains an aggressive disease with poor prognosis despite novel agent-based therapies. Some patients have better than expected survival and this phenomenon may be influenced by the absence of high-risk cytogenetics. Newer treatment regimens are needed to improve the prognosis of this devastating disease.
OBJECTIVE: To evaluate the clinical outcomes of patients with primary plasma cell leukemia (pPCL) defined by 5% or greater clonal circulating plasma cells on peripheral blood smear and treated with novel agent induction therapies. PATIENTS AND METHODS: A cohort of 68 patients with pPCL diagnosed at the Mayo Clinic in Rochester, Minnesota, from January 1, 2000, to December 31, 2019, and treated with novel agent induction therapies was evaluated. RESULTS: The median follow-up was 46 (95% CI, 41 to 90) months. The median bone marrow plasma cell content was 85% (range, 10% to 100%) and median clonal circulaitng plasma cell percentage on the peripheral blood smear was 26% (range, 5% to 93%). There was a preponderance of t(11;14) primary cytogenetic abnormality in this cohort. The median time to next therapy (TTNT) and overall survival (OS) for all patients with pPCL patients in this cohort was 13 (95% CI, 9 to 17) and 23 (95% CI, 19 to 38) months, respectively. However, when stratified by cytogenetic risk, the median TTNT and OS were 16 and 51 months for standard risk vs 9 and 19 months for high risk (P=.01 for OS). CONCLUSION: Primary plasma cell leukemia remains an aggressive disease with poor prognosis despite novel agent-based therapies. Some patients have better than expected survival and this phenomenon may be influenced by the absence of high-risk cytogenetics. Newer treatment regimens are needed to improve the prognosis of this devastating disease.
Authors: Tomas Jelinek; Jana Mihalyova; Michal Kascak; Juraj Duras; Tereza Popkova; Katerina Benkova; Petra Richterova; Hana Plonkova; Jana Zuchnicka; Lucie Broskevicova; Lucie Huvarova; Lucie Cerna; Katerina Growkova; Michal Simicek; Martin Havel; Jaromir Gumulec; Milan Navratil; Zdenek Koristek; Bruno Paiva; Roman Hajek Journal: Am J Hematol Date: 2018-11-25 Impact factor: 10.047
Authors: R García-Sanz; A Orfão; M González; M D Tabernero; J Bladé; M J Moro; J Fernández-Calvo; M A Sanz; J A Pérez-Simón; A Rasillo; J F Miguel Journal: Blood Date: 1999-02-01 Impact factor: 22.113
Authors: Shaji Kumar; Jonathan L Kaufman; Cristina Gasparetto; Joseph Mikhael; Ravi Vij; Brigitte Pegourie; Lofti Benboubker; Thierry Facon; Martine Amiot; Philippe Moreau; Elizabeth A Punnoose; Stefanie Alzate; Martin Dunbar; Tu Xu; Suresh K Agarwal; Sari Heitner Enschede; Joel D Leverson; Jeremy A Ross; Paulo C Maciag; Maria Verdugo; Cyrille Touzeau Journal: Blood Date: 2017-10-10 Impact factor: 22.113
Authors: Shaji Kumar; Bruno Paiva; Kenneth C Anderson; Brian Durie; Ola Landgren; Philippe Moreau; Nikhil Munshi; Sagar Lonial; Joan Bladé; Maria-Victoria Mateos; Meletios Dimopoulos; Efstathios Kastritis; Mario Boccadoro; Robert Orlowski; Hartmut Goldschmidt; Andrew Spencer; Jian Hou; Wee Joo Chng; Saad Z Usmani; Elena Zamagni; Kazuyuki Shimizu; Sundar Jagannath; Hans E Johnsen; Evangelos Terpos; Anthony Reiman; Robert A Kyle; Pieter Sonneveld; Paul G Richardson; Philip McCarthy; Heinz Ludwig; Wenming Chen; Michele Cavo; Jean-Luc Harousseau; Suzanne Lentzsch; Jens Hillengass; Antonio Palumbo; Alberto Orfao; S Vincent Rajkumar; Jesus San Miguel; Herve Avet-Loiseau Journal: Lancet Oncol Date: 2016-08 Impact factor: 41.316
Authors: Davine Hofste Op Bruinink; Rowan Kuiper; Mark van Duin; Tom Cupedo; Vincent H J van der Velden; Remco Hoogenboezem; Bronno van der Holt; H Berna Beverloo; Erik T Valent; Michael Vermeulen; Francesca Gay; Annemiek Broijl; Hervé Avet-Loiseau; Nikhil C Munshi; Pellegrino Musto; Philippe Moreau; Sonja Zweegman; Niels W C J van de Donk; Pieter Sonneveld Journal: J Clin Oncol Date: 2022-03-31 Impact factor: 50.717