An update on biology, diagnosis and treatment of primary plasma cell leukemia.

INTRODUCTION: Primary plasma cell leukemia (PPCL) is one of the most aggressive hematological malignancies. The prognosis of PPCL patients remains poor, although some improvements have been made in recent years. Areas covered: In this review recent clinical and biological advances in PPCL are reported. Some recommendations for the practical management of these patients are provided, with a particular focus on the role of novel agents and transplant procedures. A brief description of the currently ongoing clinical trials with new drugs is also enclosed. Expert opinion: PPCL still represents a difficult challenge for all hematologists. Here the authors provide a personal view on how the current, generally unsatisfactory results in this neoplastic disorder could be improved. In particular, dedicated studies exploring alternative therapies are necessary and eagerly awaited. Such studies should possibly be based on new biological information that could be of help in identifying novel genetic biomarkers for risk stratification and new actionable molecular targets.

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

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.

Positive effects on hematopoiesis in patients with myelodysplastic syndrome receiving deferasirox as oral iron chelation therapy: a brief review.

Iron overload is a frequent consequence in transfusion-dependent myelodysplastic syndromes (MDSs), which often requires iron chelation therapy (ICT). Interestingly, ICT may sometimes induce a hematologic improvement that leads to significant reduction or complete interruption of blood transfusions. This phenomenon has been recently described in MDS treated with the new oral chelator deferasirox. Here we briefly review the literature about this phenomenon and discuss the possible biological mechanisms underlying hematologic effects of deferasirox in MDS, starting from a new paradigmatic case in whom both hemoglobin level and platelet count improved, inducing transfusion-independence, soon after starting the treatment with deferasirox.