Plasma cell leukemia: update on biology and therapy.
Plasma cell leukemia (PCL) is a rare, but very aggressive, plasma cell dyscrasia, representing a distinct clinicopathological entity as compared to multiple myeloma (MM), with peculiar biological and clinical features. A hundred times rarer than MM, the disease course is characterized by short remissions and poor survival. PCL is defined by an increased percentage (>20%) and absolute number (>2 × 109/l) of plasma cells in the peripheral blood. PCL is defined as 'primary' when peripheral plasmacytosis is detected at diagnosis, 'secondary' when leukemization occurs in a patient with preexisting MM. Novel agents have revolutionized the outcomes of MM patients and have been introduced also for the treatment of PCL. Here, we provide an update on biology and treatment options for PCL.
a Myeloma Unit, Division of Hematology , Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino , Torino , Italy.
Roberto
R
Mina
a Myeloma Unit, Division of Hematology , Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino , Torino , Italy.
Mattia
M
D'Agostino
a Myeloma Unit, Division of Hematology , Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino , Torino , Italy.
Chiara
C
Cerrato
a Myeloma Unit, Division of Hematology , Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino , Torino , Italy.
Francesca
F
Gay
a Myeloma Unit, Division of Hematology , Azienda Ospedaliero-Universitaria Città della Salute e della Scienza di Torino , Torino , Italy.
Antonio
A
Palumbo
D000818:Animals; D015415:Biomarkers; D003131:Combined Modality Therapy; D019468:Disease Management; D020022:Genetic Predisposition to Disease; D023281:Genomics / Q000379:methods; D006801:Humans; D007952:Leukemia, Plasma Cell / Q000175:diagnosis / Q000209:etiology* / Q000378:metabolism / Q000628:therapy*; D010641:Phenotype
D015415:Biomarkers
Plasma cell leukemia; autologous stem cell transplantation; novel agents; primary PCL; secondary PCL