Abstract
Primary plasma cell leukemia (pPCL) is a de novo presentation of an aggressive extramedullary myeloma characterized by circulating plasma cells in peripheral blood. The clonal plasma cells may accumulate genetic aberrations rapidly in a very short period, resulting in de novo pPCL (from monoclonal gammopathy of undetermined significance to pPCL, thereby bypassing the myeloma phase). In contrast, secondary plasma cell leukemia (sPCL) indicates a terminal event in the natural progression of myeloma representing its leukemic transformation. A gradual acquisition of secondary chromosomal aberrations contributes to the evolution of this aggressive phenotype. While hyperdiploidy is very uncommon in pPCL, sPCL patients like myeloma may be hyperdiploid and have a higher incidence of IgH translocations. In this chapter, we summarize the clinical presentation of both phenotypes and the advances in therapeutic options for PCL patients.
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Fernandez de Larrea C, et al. Plasma cell leukemia: consensus statement on diagnostic requirements, response criteria and treatment recommendations by the International Myeloma Working Group. Leukemia. 2013;27(4):780–91.
An G, et al. Multiple myeloma patients with low proportion of circulating plasma cells had similar survival with primary plasma cell leukemia patients. Ann Hematol. 2015;94(2):257–64.
Gonsalves WI, et al. Trends in survival of patients with primary plasma cell leukemia: a population-based analysis. Blood. 2014;124(6):907–12.
Avet-Loiseau H, et al. Cytogenetic, interphase, and multicolor fluorescence in situ hybridization analyses in primary plasma cell leukemia: a study of 40 patients at diagnosis, on behalf of the Intergroupe Francophone du Myelome and the Groupe Francais de Cytogenetique Hematologique. Blood. 2001;97(3):822–5.
Tiedemann RE, et al. Genetic aberrations and survival in plasma cell leukemia. Leukemia. 2008;22(5):1044–52.
Bezieau S, et al. High incidence of N and K-Ras activating mutations in multiple myeloma and primary plasma cell leukemia at diagnosis. Hum Mutat. 2001;18(3):212–24.
Musto P, et al. Lenalidomide and low-dose dexamethasone for newly diagnosed primary plasma cell leukemia. Leukemia. 2014;28(1):222–5.
Royer B, et al. Bortezomib, doxorubicin, cyclophosphamide, dexamethasone induction followed by stem cell transplantation for primary plasma cell leukemia: a prospective phase II study of the Intergroupe Francophone du Myelome. J Clin Oncol. 2016;34(18):2125–32.
Drake MB, et al. Primary plasma cell leukemia and autologous stem cell transplantation. Haematologica. 2010;95(5):804–9.
Mahindra A, et al. Hematopoietic cell transplantation for primary plasma cell leukemia: results from the Center for International Blood and Marrow Transplant Research. Leukemia. 2012;26(5):1091–7.
D’Arena G, et al. Frontline chemotherapy with bortezomib-containing combinations improves response rate and survival in primary plasma cell leukemia: a retrospective study from GIMEMA Multiple Myeloma Working Party. Ann Oncol. 2012;23(6):1499–502.
Musto P, et al. Efficacy and safety of bortezomib in patients with plasma cell leukemia. Cancer. 2007;109(11):2285–90.
Lebovic D, et al. Clinical outcomes of patients with plasma cell leukemia in the era of novel therapies and hematopoietic stem cell transplantation strategies: a single-institution experience. Clin Lymphoma Myeloma Leuk. 2011;11(6):507–11.
Usmani SZ, et al. Primary plasma cell leukemia: clinical and laboratory presentation, gene-expression profiling and clinical outcome with Total Therapy protocols. Leukemia. 2012;26(11):2398–405.
Avet-Loiseau H, et al. Cytogenetic and therapeutic characterization of primary plasma cell leukemia: the IFM experience. Leukemia. 2012;26(1):158–9.
Nooka AK, et al. Plasma cell leukemia: sustained responses are possible with innovative treatment strategies haematologica 2012. Leukemia. 97(s1):1516.
Katodritou E, et al. Treatment with bortezomib-based regimens improves overall response and predicts for survival in patients with primary or secondary plasma cell leukemia: analysis of the Greek myeloma study group. Am J Hematol. 2014;89(2):145–50.
Gozzetti A, et al. Efficacy of bortezomib, lenalidomide and dexamethasone (VRD) in secondary plasma cell leukaemia. Br J Haematol. 2012;157(4):497–8.
Durie BG, et al. Bortezomib with lenalidomide and dexamethasone versus lenalidomide and dexamethasone alone in patients with newly diagnosed myeloma without intent for immediate autologous stem-cell transplant (SWOG S0777): a randomised, open-label, phase 3 trial. Lancet. 2017;389(10068):519–27.
Iriuchishima H, et al. Primary plasma cell leukemia in the era of novel agents: a multicenter study of the japanese society of myeloma. Acta Haematol. 2016;135(2):113–21.
Pagano L, et al. Primary plasma cell leukemia: a retrospective multicenter study of 73 patients. Ann Oncol. 2011;22(7):1628–35.
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Joseph, N.S., Neppalli, A.K., Nooka, A.K. (2018). Plasma Cell Leukemia. In: Usmani, S., Nooka, A. (eds) Personalized Therapy for Multiple Myeloma. Springer, Cham. https://doi.org/10.1007/978-3-319-61872-2_7
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DOI: https://doi.org/10.1007/978-3-319-61872-2_7
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