Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia
Abstract Background Plasma cell leukemia (PCL) is a rare monoclonal gammopathy, associated with short survival. Because of its very low incidence, only a few cohorts have been reported and thus, information on this disease is scarce. The goal of this study was to better understand the clinical featu...
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Wiley
2024-09-01
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| Series: | Cancer Medicine |
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| Online Access: | https://doi.org/10.1002/cam4.70192 |
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| author | Camille Tessier Richard LeBlanc Jean Roy Sabrina Trudel Julie Côté Marc Lalancette Jean‐Samuel Boudreault Émilie Lemieux‐Blanchard Rayan Kaedbey Michel Pavic |
| author_facet | Camille Tessier Richard LeBlanc Jean Roy Sabrina Trudel Julie Côté Marc Lalancette Jean‐Samuel Boudreault Émilie Lemieux‐Blanchard Rayan Kaedbey Michel Pavic |
| author_sort | Camille Tessier |
| collection | DOAJ |
| description | Abstract Background Plasma cell leukemia (PCL) is a rare monoclonal gammopathy, associated with short survival. Because of its very low incidence, only a few cohorts have been reported and thus, information on this disease is scarce. The goal of this study was to better understand the clinical features, prognostic factors, and efficacy of modern treatments in both primary PCL (pPCL) and secondary PCL (sPCL). Methods We performed a retrospective, multicenter study of patients diagnosed with PCL, defined as circulating plasma cells ≥20% of total leukocytes and/or ≥2 × 109/L. Results We identified 99 eligible PCL patients, of whom 33 were pPCL and 66 were sPCL. The median progression‐free survival (PFS) to frontline treatment and overall survival (OS) were, respectively, 4.8 (95% CI, 0.4–9.2) and 18.3 months (95% CI, 0.0–39.0) for pPCL and 0.8 (95% CI, 0.5–1.1) and 1.2 months (95% CI, 0.9–1.5) for sPCL (both p < 0.001). We observed no improvement in OS over time (2005–2012 vs. 2013–2020, p = 0.629 for pPCL and p = 0.329 for sPCL). Finally, our data suggested that sPCL originates from a high‐risk multiple myeloma (MM) population with a short OS (median 30.2 months), early relapse after stem cell transplant (median 11.9 months) and a high proportion of patients with multiple cytogenetic abnormalities (36% with ≥2 abnormalities). Conclusions This study is one of the largest PCL cohorts reported. We are also the first to investigate characteristics of MM before its transformation into sPCL and demonstrate that high‐risk biologic features already present at the time of MM diagnosis. Moreover, our data highlights the lack of improvement in PCL survival in recent years and the urgent need for better treatment options. |
| format | Article |
| id | doaj-art-41fd1e661a10445ab6a281d4f98c7702 |
| institution | Kabale University |
| issn | 2045-7634 |
| language | English |
| publishDate | 2024-09-01 |
| publisher | Wiley |
| record_format | Article |
| series | Cancer Medicine |
| spelling | doaj-art-41fd1e661a10445ab6a281d4f98c77022025-02-07T09:08:08ZengWileyCancer Medicine2045-76342024-09-011317n/an/a10.1002/cam4.70192Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemiaCamille Tessier0Richard LeBlanc1Jean Roy2Sabrina Trudel3Julie Côté4Marc Lalancette5Jean‐Samuel Boudreault6Émilie Lemieux‐Blanchard7Rayan Kaedbey8Michel Pavic9Université de Sherbrooke Sherbrooke Quebec CanadaHôpital Maisonneuve‐Rosemont Montreal Quebec CanadaHôpital Maisonneuve‐Rosemont Montreal Quebec CanadaHôpital Charles‐Le Moyne Greenfield Park Quebec CanadaCentre Hospitalier Universitaire de Québec (CHUQ), Hôpital de l'Enfant‐Jésus Quebec Quebec CanadaCentre Hospitalier Universitaire de Québec (CHUQ), Hôtel‐Dieu de Québec Quebec Quebec CanadaHôpital du Sacré‐Cœur de Montréal Montreal Quebec CanadaCentre Hospitalier de l'Université de Montréal (CHUM) Montreal Quebec CanadaJewish General Hospital Montreal Quebec CanadaCentre Hospitalier Universitaire de Sherbrooke (CHUS) Sherbrooke Quebec CanadaAbstract Background Plasma cell leukemia (PCL) is a rare monoclonal gammopathy, associated with short survival. Because of its very low incidence, only a few cohorts have been reported and thus, information on this disease is scarce. The goal of this study was to better understand the clinical features, prognostic factors, and efficacy of modern treatments in both primary PCL (pPCL) and secondary PCL (sPCL). Methods We performed a retrospective, multicenter study of patients diagnosed with PCL, defined as circulating plasma cells ≥20% of total leukocytes and/or ≥2 × 109/L. Results We identified 99 eligible PCL patients, of whom 33 were pPCL and 66 were sPCL. The median progression‐free survival (PFS) to frontline treatment and overall survival (OS) were, respectively, 4.8 (95% CI, 0.4–9.2) and 18.3 months (95% CI, 0.0–39.0) for pPCL and 0.8 (95% CI, 0.5–1.1) and 1.2 months (95% CI, 0.9–1.5) for sPCL (both p < 0.001). We observed no improvement in OS over time (2005–2012 vs. 2013–2020, p = 0.629 for pPCL and p = 0.329 for sPCL). Finally, our data suggested that sPCL originates from a high‐risk multiple myeloma (MM) population with a short OS (median 30.2 months), early relapse after stem cell transplant (median 11.9 months) and a high proportion of patients with multiple cytogenetic abnormalities (36% with ≥2 abnormalities). Conclusions This study is one of the largest PCL cohorts reported. We are also the first to investigate characteristics of MM before its transformation into sPCL and demonstrate that high‐risk biologic features already present at the time of MM diagnosis. Moreover, our data highlights the lack of improvement in PCL survival in recent years and the urgent need for better treatment options.https://doi.org/10.1002/cam4.70192hematologic malignanciesmultiple myelomaplasma cell disorderplasma cell leukemiaretrospective study |
| spellingShingle | Camille Tessier Richard LeBlanc Jean Roy Sabrina Trudel Julie Côté Marc Lalancette Jean‐Samuel Boudreault Émilie Lemieux‐Blanchard Rayan Kaedbey Michel Pavic Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia Cancer Medicine hematologic malignancies multiple myeloma plasma cell disorder plasma cell leukemia retrospective study |
| title | Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia |
| title_full | Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia |
| title_fullStr | Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia |
| title_full_unstemmed | Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia |
| title_short | Poor outcome despite modern treatments: A retrospective study of 99 patients with primary and secondary plasma cell leukemia |
| title_sort | poor outcome despite modern treatments a retrospective study of 99 patients with primary and secondary plasma cell leukemia |
| topic | hematologic malignancies multiple myeloma plasma cell disorder plasma cell leukemia retrospective study |
| url | https://doi.org/10.1002/cam4.70192 |
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