title: Primary plasma cell leukemia 2.0: advances in biology and clinical management.
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.
authors.affiliation: a Department of Oncology and Hemato-Oncology , University of Milan , Milan , Italy.|b Hematology Unit , Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico , Milan , Italy.
authors.forename: Antonino
authors.initials: A
authors.lastname: Neri
authors.affiliation: c Laboratory of Pre-clinical and Translational Research , IRCCS-CROB, Referral Cancer Center of Basilicata , Rionero in Vulture , Italy.
authors.forename: Katia
authors.initials: K
authors.lastname: Todoerti
authors.affiliation: a Department of Oncology and Hemato-Oncology , University of Milan , Milan , Italy.|b Hematology Unit , Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico , Milan , Italy.
authors.forename: Marta
authors.initials: M
authors.lastname: Lionetti
authors.affiliation: c Laboratory of Pre-clinical and Translational Research , IRCCS-CROB, Referral Cancer Center of Basilicata , Rionero in Vulture , Italy.
authors.forename: Vittorio
authors.initials: V
authors.lastname: Simeon
authors.affiliation: a Department of Oncology and Hemato-Oncology , University of Milan , Milan , Italy.|b Hematology Unit , Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico , Milan , Italy.
authors.forename: Marzia
authors.initials: M
authors.lastname: Barbieri
authors.affiliation: d Laboratory of Clinical Research and Advanced Diagnostics , IRCCS-CROB, Referral Cancer Center of Basilicata , Rionero in Vulture , Italy.
authors.forename: Filomena
authors.initials: F
authors.lastname: Nozza
authors.affiliation: d Laboratory of Clinical Research and Advanced Diagnostics , IRCCS-CROB, Referral Cancer Center of Basilicata , Rionero in Vulture , Italy.
authors.forename: Gabriella
authors.initials: G
authors.lastname: Vona
authors.affiliation: a Department of Oncology and Hemato-Oncology , University of Milan , Milan , Italy.|b Hematology Unit , Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico , Milan , Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Pompa
authors.affiliation: a Department of Oncology and Hemato-Oncology , University of Milan , Milan , Italy.|b Hematology Unit , Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico , Milan , Italy.
authors.forename: Luca
authors.initials: L
authors.lastname: Baldini
authors.affiliation: e Scientific Direction , IRCCS-CROB, Referral Cancer Center of Basilicata , Rionero in Vulture , Italy.
authors.forename: Pellegrino
authors.initials: P
authors.lastname: Musto
mesh_terms: D000818:Animals; D000970:Antineoplastic Agents / Q000008:administration & dosage / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D019468:Disease Management; D020022:Genetic Predisposition to Disease; D005820:Genetic Testing / Q000379:methods; D023281:Genomics / Q000379:methods; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D007952:Leukemia, Plasma Cell / Q000175:diagnosis* / Q000209:etiology / Q000401:mortality / Q000628:therapy*; D058990:Molecular Targeted Therapy; D011379:Prognosis
chemical_list: D000970:Antineoplastic Agents
keywords: IMIDs; Plasma cell leukemia; bortezomib; lenalidomide; molecular profiling; monoclonal antibodies; multiple myeloma; proteasome inhibitors; risk stratification; stem cell transplantation