Blood Res 2022; 57(S1):
Published online April 30, 2022
https://doi.org/10.5045/br.2022.2022033
© The Korean Society of Hematology
Correspondence to : Je-Jung Lee, M.D., Ph.D.
Department of Hematology-Oncology, Chonnam National University Hwasun Hospital, 322 Seoyangro, Hwasun 58128, Korea
E-mail: drjejung@chonnam.ac.kr
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Plasma cell leukemia (PCL) is a rare and highly aggressive plasma cell neoplasm developing in 0.5?4% of patients with multiple myeloma (MM). The diagnostic criteria were recently revised from 20% to ≥5% of circulating plasma cells in peripheral blood smears. PCL is classified as primary or secondary; primary PCL is when it presents in patients with no MM. Primary PCL shows clinical and laboratory features at presentation that differ from MM and exhibits a dismal prognosis even with the use of effective agents against MM. Therefore, intensive chemotherapy should be initiated immediately after diagnosis, and autologous stem cell transplantation is recommended for transplant-eligible patients. Maintenance therapy after transplantation may reduce the rate of early relapses. We reviewed the definitions of PCL, revised diagnostic criteria, clinical features, and appropriate initial treatments for primary PCL.
Keywords Plasma cell leukemia, Diagnosis, Treatment
Blood Res 2022; 57(S1): S62-S66
Published online April 30, 2022 https://doi.org/10.5045/br.2022.2022033
Copyright © The Korean Society of Hematology.
Sung-Hoon Jung, Je-Jung Lee
Department of Hematology-Oncology, Chonnam National University Hwasun Hospital, Chonnam National University Medical School, Hwasun, Korea
Correspondence to:Je-Jung Lee, M.D., Ph.D.
Department of Hematology-Oncology, Chonnam National University Hwasun Hospital, 322 Seoyangro, Hwasun 58128, Korea
E-mail: drjejung@chonnam.ac.kr
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
Plasma cell leukemia (PCL) is a rare and highly aggressive plasma cell neoplasm developing in 0.5?4% of patients with multiple myeloma (MM). The diagnostic criteria were recently revised from 20% to ≥5% of circulating plasma cells in peripheral blood smears. PCL is classified as primary or secondary; primary PCL is when it presents in patients with no MM. Primary PCL shows clinical and laboratory features at presentation that differ from MM and exhibits a dismal prognosis even with the use of effective agents against MM. Therefore, intensive chemotherapy should be initiated immediately after diagnosis, and autologous stem cell transplantation is recommended for transplant-eligible patients. Maintenance therapy after transplantation may reduce the rate of early relapses. We reviewed the definitions of PCL, revised diagnostic criteria, clinical features, and appropriate initial treatments for primary PCL.
Keywords: Plasma cell leukemia, Diagnosis, Treatment
Table 1 . Summary of prospective and retrospective studies of treatment with novel agents for primary plasma cells leukemia..
References | Study design | Patients, N | Median age, yr (range) | ORR/CR (%) | ASCT, N | Median PFS (mo) | Median OS (mo) |
---|---|---|---|---|---|---|---|
Musto | Prospective | 23 | 60 (44–80) | 73.9/13 | 9 | 14 | 28 |
Royer | Prospective | 39 | 57 (27–71) | 69/10 | 25 | 15.1 | 36.3 |
Nandakumar | Retrospective | 68 | 62 (34–91) | 90/47 | 20 | 13 | 23 |
Dhakal | Retrospective | 277 | 60 (25–77) | 87/19 | 277 | 17% at 4 yr | 28% at 4 yr |
Mina | Retrospective | 38 | 58 (34–80) | 82/18 | 28 | 20 | 33 |
Gowda | Retrospective | 23 | 56 (42–71) | 65/13 | 23 | 5.5 | 18.1 |
D’Arena | Retrospective | 29 | 62 (42–82) | 79/28 | 12 | 40% at 2 yr | 55% at 2 yr |
Hyun Jung Lee
Blood Res 2023; 58(S1): S96-S108Hyewon Lee
Blood Res 2023; 58(S1): S66-S82Sang Hyuk Park, Yoo Jin Lee, Youjin Kim, Hyun-Ki Kim, Ji-Hun Lim, Jae-Cheol Jo
Blood Res 2023; 58(S1): S52-S57