Plasma cell leukemia: update on biology and therapy

被引:35
作者
Mina, Roberto [1 ]
D'Agostino, Mattia [1 ]
Cerrato, Chiara [1 ]
Gay, Francesca [1 ]
Palumbo, Antonio [1 ]
机构
[1] Azienda Osped Univ Citta Salute & Sci Torino, Myeloma Unit, Div Hematol, Turin, Italy
关键词
Plasma cell leukemia; primary PCL; secondary PCL; autologous stem cell transplantation; novel agents; MULTIPLE-MYELOMA; INTERGROUPE-FRANCOPHONE; GENETIC ABERRATIONS; BORTEZOMIB; SURVIVAL; DEXAMETHASONE; TRANSPLANTATION; LENALIDOMIDE; THALIDOMIDE; CYCLOPHOSPHAMIDE;
D O I
10.1080/10428194.2016.1250263
中图分类号
R73 [肿瘤学];
学科分类号
100214 ;
摘要
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 x 10(9)/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.
引用
收藏
页码:1538 / 1547
页数:10
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