Plasma cell leukemia (PCL) is a rare, but very aggressive, plasma cell dyscrasia, representing a distinct clinicopathological entity as compared to multiple myeloma (MM), with peculiar biological and clinical features. A hundred times rarer than MM, the disease course is characterized by short remissions and poor survival. PCL is defined by an increased percentage (>20%) and absolute number (>2 × 109/l) of plasma cells in the peripheral blood. PCL is defined as ‘primary’ when peripheral plasmacytosis is detected at diagnosis, ‘secondary’ when leukemization occurs in a patient with preexisting MM. Novel agents have revolutionized the outcomes of MM patients and have been introduced also for the treatment of PCL. Here, we provide an update on biology and treatment options for PCL.
Plasma cell leukemia: update on biology and therapy
Mina R.
Co-first
;D'Agostino M.Co-first
;Cerrato C.;Gay F.;Palumbo A.Last
2017-01-01
Abstract
Plasma cell leukemia (PCL) is a rare, but very aggressive, plasma cell dyscrasia, representing a distinct clinicopathological entity as compared to multiple myeloma (MM), with peculiar biological and clinical features. A hundred times rarer than MM, the disease course is characterized by short remissions and poor survival. PCL is defined by an increased percentage (>20%) and absolute number (>2 × 109/l) of plasma cells in the peripheral blood. PCL is defined as ‘primary’ when peripheral plasmacytosis is detected at diagnosis, ‘secondary’ when leukemization occurs in a patient with preexisting MM. Novel agents have revolutionized the outcomes of MM patients and have been introduced also for the treatment of PCL. Here, we provide an update on biology and treatment options for PCL.File | Dimensione | Formato | |
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[PUBLISHED Vsn.] Mina et al - 2017 - Leukemia and Lymphoma.pdf
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Descrizione: [Restricted access - Published vsn.] Mina R, D'Agostino M, et al. Leuk Lymphoma . 2017 Jul;58(7):1538-1547. doi: 10.1080/10428194.2016.1250263. Epub 2016 Nov 6. © 2016 Informa UK Limited, trading as Taylor & Francis Group. Available at: https://www.tandfonline.com/doi/abs/10.1080/10428194.2016.1250263?journalCode=ilal20 | https://doi.org/10.1080/10428194.2016.1250263
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[Author Vsn] Mina R D'Agostino M et al - 2017 - Leuk Lymphoma - pPCL update d.pdf
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Descrizione: [Author vsn.] Mina R, D'Agostino M, et al. Leuk Lymphoma . 2017 Jul;58(7):1538-1547. doi: 10.1080/10428194.2016.1250263. Epub 2016 Nov 6. © 2016 Informa UK Limited, trading as Taylor & Francis Group. The published version is available at: https://www.tandfonline.com/doi/abs/10.1080/10428194.2016.1250263?journalCode=ilal20 | https://doi.org/10.1080/10428194.2016.1250263. When citing, please refer to the published version.
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