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Update on primary plasma cell leukemia.

ABSTRACT: 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.

SUBMITTER: Jung SH

PROVIDER: S-EPMC9057670 | biostudies-literature | 2022 Apr

REPOSITORIES: biostudies-literature

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Update on primary plasma cell leukemia.

Jung Sung-Hoon SH Lee Je-Jung JJ

Blood research 20220401 S1

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 ef ...[more]

PMID: 35483928

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Project description:IntroductionThe definition of primary plasma cell leukemia (pPCL) has been revised from ≥20% to ≥5% circulating plasma cells (CPC). However, the precise prognosis associated with CPC remains controversial. This study aimed to investigate prognostic biomarkers for myeloma patients based on CPC presence.MethodsA comprehensive analysis was conducted on 309 consecutive patients diagnosed with either multiple myeloma or pPCL, utilizing peripheral blood smears stained with Wright-Giemsa.ResultsPatients were grouped by CPC percentage: 0% (221, 71.5%), 1-4% (49, 15.9%), 5-19% (16, 5.2%), ≥20% (23, 7.4%). CPC >5% correlated with unfavorable characteristics, including anemia, renal dysfunction, and advanced International Staging System. Common cytogenetic abnormalities such as 1q21 amplification, 17p deletion, and Myc rearrangement were prevalent among CPC-positive patients. Median progression-free survival (PFS) and overall survival (OS) were shorter in patients with CPC ≥5% (29.47 vs. 10.03 months; 64.10 vs. 12.30 months). Additionally, PFS and OS were shorter in CPC-positive patients without autologous hematopoietic stem cell transplantation (ASCT) and those with response < partial remission to the first-line regimen. Furthermore, an association emerged between soft tissue-related extramedullary disease and inferior PFS, while Myc rearrangement correlated with abbreviated OS.ConclusionBiological characteristics displayed greater aggressiveness in patients with positive CPC, leading to significantly shorter PFS and OS. The presence of CPC, ASCT, and overall response rate were independent prognostic factors. While no new threshold for pPCL with CPCs is proposed, Myc rearrangements and CPC positivity could serve as ultra-high-risk factors for multiple myeloma.IntroductionThe definition of primary plasma cell leukemia (pPCL) has been revised from ≥20% to ≥5% circulating plasma cells (CPC). However, the precise prognosis associated with CPC remains controversial. This study aimed to investigate prognostic biomarkers for myeloma patients based on CPC presence.MethodsA comprehensive analysis was conducted on 309 consecutive patients diagnosed with either multiple myeloma or pPCL, utilizing peripheral blood smears stained with Wright-Giemsa.ResultsPatients were grouped by CPC percentage: 0% (221, 71.5%), 1-4% (49, 15.9%), 5-19% (16, 5.2%), ≥20% (23, 7.4%). CPC >5% correlated with unfavorable characteristics, including anemia, renal dysfunction, and advanced International Staging System. Common cytogenetic abnormalities such as 1q21 amplification, 17p deletion, and Myc rearrangement were prevalent among CPC-positive patients. Median progression-free survival (PFS) and overall survival (OS) were shorter in patients with CPC ≥5% (29.47 vs. 10.03 months; 64.10 vs. 12.30 months). Additionally, PFS and OS were shorter in CPC-positive patients without autologous hematopoietic stem cell transplantation (ASCT) and those with response < partial remission to the first-line regimen. Furthermore, an association emerged between soft tissue-related extramedullary disease and inferior PFS, while Myc rearrangement correlated with abbreviated OS.ConclusionBiological characteristics displayed greater aggressiveness in patients with positive CPC, leading to significantly shorter PFS and OS. The presence of CPC, ASCT, and overall response rate were independent prognostic factors. While no new threshold for pPCL with CPCs is proposed, Myc rearrangements and CPC positivity could serve as ultra-high-risk factors for multiple myeloma.

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Update on primary plasma cell leukemia.

Publications

Update on primary plasma cell leukemia.

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