Outcomes and treatment patterns in primary and secondary plasma cell leukemia: insights from a large US cohort study
Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder with a short survival duration despite the use of novel therapeutics. PCL remains an understudied disease for which there is no current standard of care treatment. Knowledge on optimal novel drug sequencing, including the role...
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| Main Authors: | , , , , , , , , , , , , , , , |
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| Format: | Article |
| Language: | English |
| Published: |
Ferrata Storti Foundation
2025-04-01
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| Series: | Haematologica |
| Online Access: | https://haematologica.org/article/view/12031 |
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| Summary: | Plasma cell leukemia (PCL) is a rare and aggressive plasma cell disorder with a short survival duration despite the use of novel therapeutics. PCL remains an understudied disease for which there is no current standard of care treatment. Knowledge on optimal novel drug sequencing, including the role and timing of hematopoietic stem cell transplant as primary and salvage therapy is needed. We conducted a large retrospective analysis of 153 PCL patients, using the new definition of ≥ 5% circulating plasma cells, including clinical features and treatment outcomes across seven academic centers in United States. This is, to the best of our knowledge, the largest multicenter study conducted in the US of this rare disease. Disease presentation, clinical and genetic characteristics, and treatment patterns of 93 patients with primary PCL and 57 with secondary PCL were described. Additionally, associations between patient characteristics and mortality were investigated using Cox proportional-hazards regression models. Secondary PCL was associated with a worse prognosis, with a median overall survival of 3.2 months compared to 36.6 months for primary PCL (p |
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| ISSN: | 0390-6078 1592-8721 |