Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers?

Anna Suska; David H. Vesole; Jorge J. Castillo; Shaji K. Kumar; Hari Parameswaran; Maria V. Mateos; Thierry Facon; Alessandro Gozzetti; Gabor Mikala; Marta Szostek; Joseph Mikhael; Roman Hajek; Evangelos Terpos; Artur Jurczyszyn

doi:10.2991/chi.k.200706.002

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Volume 2, Issue 4, December 2020, Pages 133 - 142

Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers?

Authors

Anna Suska¹, David H. Vesole², Jorge J. Castillo³, Shaji K. Kumar⁴, Hari Parameswaran⁵, Maria V. Mateos⁶, Thierry Facon⁷, Alessandro Gozzetti⁸, Gabor Mikala⁹, Marta Szostek¹, Joseph Mikhael¹⁰, Roman Hajek¹¹, Evangelos Terpos¹², Artur Jurczyszyn¹^{, *}

¹Department of Hematology, Jagiellonian University Medical College, Kopernika 17, Krakow 31-501, Poland

²The John Theurer Cancer Center at Hackensack UMC, Hackensack, NJ, USA

³Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA, USA

⁴Division of Hematology, Mayo Clinic, Rochester, MN, USA

⁵Medical College of Wisconsin, Milwaukee, WI, USA

⁶Complejo Asistencial Universitario de Salamanca, Instituto de Investigación Biomédica de Salamanca (CAUSA/IBSAL), Salamanca, Spain

⁷Service des Maladies du Sang, Hôpital Claude Huriez, Lille, France

⁸Division of Hematology and Transplants, University of Siena, Siena, Italy

⁹Department of Hematology and Stem Cell Transplantation, South-Pest Central Hospital, Natl. Inst. Hematol. Infectol, Budapest, Hungary

¹⁰Translational Genomics Research Institute, City of Hope Cancer Center, Phoenix, Arizona, USA

¹¹University Hospital Ostrava and Faculty of Medicine, University of Ostrava, Ostrava, Czech Republic

¹²Department of Clinical Therapeutics, School of Medicine, National and Kapodistrian University of Athens, Athens, Greece

^*Corresponding author. Email: mmjurczy@cyf-kr.edu.pl

Corresponding Author

Artur Jurczyszyn

Received 7 April 2020, Accepted 1 June 2020, Available Online 19 July 2020.

DOI: 10.2991/chi.k.200706.002 How to use a DOI?
Keywords: Plasma cell leukemia; plasma cell dyscrasia; chemotherapy; novel agents; stem cell transplantation; clinical trials; prognostic index
Abstract: Plasma cell leukemia (PCL) is an aggressive hematological malignancy characterized by an uncontrolled clonal proliferation of plasma cells (PCs) in the bone marrow and peripheral blood. PCL has been defined by an absolute number of circulating PCs exceeding 2.0 × 10⁹/L and/or >20% PCs in the total leucocyte count. It is classified as primary PCL, which develops de novo, and secondary PCL, occurring at the late and advanced stages of multiple myeloma (MM). Primary and secondary PCL are clinically and biologically two distinct entities. After the diagnosis, treatment should be immediate and should include a proteasome inhibitor and immunomodulator-based combination regimens as induction, followed by stem cell transplantation (SCT) in transplant-eligible individuals who have cleared the peripheral blood of circulating PCs. Due to the rarity of the condition, there have been very few clinical trials. Furthermore, virtually all of the myeloma trials exclude patients with active PCL. The evaluation of response has been defined by the International Myeloma Working Group and consists of both acute leukemia and MM criteria. With conventional chemotherapy, the prognosis of primary PCL has been ominous, with reported overall survival (OS) ranging from 6.8 to 12.6 months. The use of novel agents and autologous SCT appears to be associated with deeper response and an improved survival, although it still remains low. The PCL prognostic index provides a simple score to risk-stratify PCL. The prognosis of secondary PCL is extremely poor, with OS of only 1 month.
Open Access: This is an open access article distributed under the CC BY-NC 4.0 license (http://creativecommons.org/licenses/by-nc/4.0/).

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Next Article In Issue>

Journal: Clinical Hematology International
Volume-Issue: 2 - 4
Pages: 133 - 142
Publication Date: 2020/07/19
ISSN (Online): 2590-0048
DOI: 10.2991/chi.k.200706.002 How to use a DOI?
Open Access: This is an open access article distributed under the CC BY-NC 4.0 license (http://creativecommons.org/licenses/by-nc/4.0/).

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ris enw bib

TY  - JOUR
AU  - Anna Suska
AU  - David H. Vesole
AU  - Jorge J. Castillo
AU  - Shaji K. Kumar
AU  - Hari Parameswaran
AU  - Maria V. Mateos
AU  - Thierry Facon
AU  - Alessandro Gozzetti
AU  - Gabor Mikala
AU  - Marta Szostek
AU  - Joseph Mikhael
AU  - Roman Hajek
AU  - Evangelos Terpos
AU  - Artur Jurczyszyn
PY  - 2020
DA  - 2020/07/19
TI  - Plasma Cell Leukemia – Facts and Controversies: More Questions than Answers?
JO  - Clinical Hematology International
SP  - 133
EP  - 142
VL  - 2
IS  - 4
SN  - 2590-0048
UR  - https://doi.org/10.2991/chi.k.200706.002
DO  - 10.2991/chi.k.200706.002
ID  - Suska2020
ER  -

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