Primary plasma cell leukemia (PPCL) is a rare and aggressive variant of multiple myeloma. The introduction of novel agents and modern technologies has recently partially changed the clinical and biological scenario of this malignancy, allowing limited, but not negligible, progresses. Areas covered: We will discuss: the complex landscape of genetic alterations in PPCL, derived from conventional and high-throughput technologies; the best available treatments for PPCL; the possible future therapeutic perspectives. Expert commentary: PPCL requires an immediate and intensive multi-phase treatment with short therapy-free intervals, which should include novel agents and autologous stem cell transplantation in eligible patients. Allogeneic transplantation should be considered in selected cases. In older and/or frailer individuals, personalized approaches should be applied. Integrated treatments with next generation proteasome inhibitors/IMIDs and monoclonal antibodies are currently planned or under investigation. The identification of novel genomic biomarkers may be potentially helpful for risk stratification and future personalized therapies.

Primary plasma cell leukemia 2.0: advances in biology and clinical management / A. Neri, K. Todoerti, M. Lionetti, V. Simeon, M. Barbieri, F. Nozza, G. Vona, A. Pompa, L. Baldini, P. Musto. - In: EXPERT REVIEW OF HEMATOLOGY. - ISSN 1747-4086. - 9:11(2016 Nov), pp. 1063-1073.

Primary plasma cell leukemia 2.0: advances in biology and clinical management

A. Neri
Primo
;
K. Todoerti
Secondo
;
M. Lionetti;M. Barbieri;L. Baldini
Penultimo
;
2016

Abstract

Primary plasma cell leukemia (PPCL) is a rare and aggressive variant of multiple myeloma. The introduction of novel agents and modern technologies has recently partially changed the clinical and biological scenario of this malignancy, allowing limited, but not negligible, progresses. Areas covered: We will discuss: the complex landscape of genetic alterations in PPCL, derived from conventional and high-throughput technologies; the best available treatments for PPCL; the possible future therapeutic perspectives. Expert commentary: PPCL requires an immediate and intensive multi-phase treatment with short therapy-free intervals, which should include novel agents and autologous stem cell transplantation in eligible patients. Allogeneic transplantation should be considered in selected cases. In older and/or frailer individuals, personalized approaches should be applied. Integrated treatments with next generation proteasome inhibitors/IMIDs and monoclonal antibodies are currently planned or under investigation. The identification of novel genomic biomarkers may be potentially helpful for risk stratification and future personalized therapies.
IMIDs; plasma cell leukemia; bortezomib; lenalidomide; molecular profiling; monoclonal antibodies; multiple myeloma; proteasome inhibitors; risk stratification; stem cell transplantation
Settore MED/15 - Malattie del Sangue
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/448854
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