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

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 i...

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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
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Summary: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.
ISSN:1040-8428
1879-0461
DOI:10.1016/j.critrevonc.2011.04.005