Primary plasma cell leukemia in the era of new drugs: Has something changed?

• Published in: Critical reviews in oncology/hematology 2012-05, Vol.82 (2), p.141-149
• Main Authors: Musto, Pellegrino, Pagano, Livio, Petrucci, Maria Teresa, Morabito, Fortunato, Caravita, Tommaso, Di Raimondo, Francesco, Baldini, Luca, Tosi, Patrizia, Bringhen, Sara, Offidani, Massimo, Omede’, Paola, Neri, Antonino, D’Auria, Fiorella, Bochicchio, Giovanni Battista, Cavo, Michele, Boccadoro, Mario, Palumbo, Antonio
• Format: Article
• Language: English
• Subjects: Antineoplastic Agents - therapeutic use; Biological and medical sciences; Bortezomib; Hematologic and hematopoietic diseases; Hematology, Oncology and Palliative Medicine; Hematopoietic Stem Cell Transplantation; Humans; Immunodeficiencies. Immunoglobulinopathies; Immunoglobulinopathies; Immunopathology; Lenalidomide; Leukemia, Plasma Cell - drug therapy; Leukemia, Plasma Cell - surgery; Leukemias. Malignant lymphomas. Malignant reticulosis. Myelofibrosis; Medical sciences; Myeloma; Plasma cell leukemia; Stem cell transplantation; Thalidomide
• ISSN: 1040-8428; 1879-0461
• DOI: 10.1016/j.critrevonc.2011.04.005

Abstract Primary plasma cell leukemia (PPCL) is a rare and aggressive variant of multiple myeloma. This disease is associated with a very poor prognosis, and unfortunately it has not significantly improved during the last three decades. Autologous stem cell transplantation is generally recommended in eligible patients, but survival in transplanted PPCL patients is significantly lower than that of multiple myeloma. Recent preliminary data indicate that new drugs, in particular lenalidomide and bortezomib, could significantly improve the clinical outcome of PPCL, increasing response rate and duration, as well as survival. In this review we report an updated literature analysis about the current therapeutic scenario of PPCL, with a particular focus on the use of novel agents.