The prognostic factors and immune microenvironment of primary plasma cell leukemia: the KMMWP-2204 study - PubMed
4 hours ago
- #Plasma cell leukemia
- #Prognostic factors
- #Immune microenvironment
- Primary plasma cell leukemia (pPCL) is a rare and aggressive malignancy with poor prognosis, studied in a Korean cohort of 127 patients.
- The revised 2021 IMWG diagnostic threshold of 5% circulating plasma cells (CPC) was supported, with similar survival for patients having 5-19% and ≥20% CPCs.
- Independent predictors of 6-month early mortality included poor performance status, elevated lactate dehydrogenase, and del(17p).
- Achieving complete remission was the strongest predictor of improved survival, while autologous stem cell transplantation (ASCT) showed a consolidative rather than independent effect.
- Survival outcomes remained similar over time despite increased use of intensive induction regimens and ASCT post-2016, attributed to baseline risk differences and retrospective study design.
- Single-cell RNA sequencing analysis suggested altered immune differentiation and myeloid-associated immunoregulatory signaling in pPCL, providing biological insights into its aggressiveness.
- The findings are hypothesis-generating and require validation in larger cohorts with functional studies.