Prolymphocytic Leukemia: New Insights in Diagnosis and in Treatment.
OBJECTIVE
We aimed to produce a comprehensive update on clinical and biological data regarding two rare lymphoid neoplasms, B and T prolymphocytic leukemias, and assess therapeutic management in the light of new molecular insights and the advent of targeted therapies.


RESULTS
B cell prolymphocytic leukemia (B-PLL) diagnosis remains challenging in the absence of clear immunophenotypic or cytogenetic signature and overlap with mantle cell lymphoma. New molecular defects have been identified in T cell prolymphocytic leukemia (T-PLL), especially in the JAK STAT pathway. Like in chronic lymphocytic leukemia (CLL), B-PLL treatment depends on the presence of TP53 dysfunction. In T-PLL, alemtuzumab still remains the standard of care. Allogeneic transplantation is the only curable option. Thanks to reduced intensity conditioning regimens, it has become accessible to a larger number of patients. PLL prognosis remains poor with conventional therapies. However, great advances in the understanding of both T- and B-PLL pathogenesis lead to promising new therapeutic agents.
Department of Hematology, Institut Paoli-Calmettes, 232 Bd Ste Margerite, 13009, Marseille, France.
Aude
A
Collignon
Department of Hematology, Institut Paoli-Calmettes, 232 Bd Ste Margerite, 13009, Marseille, France.
Anne
A
Wanquet
Centre Hospitalo-universitaire de Caen, Caen, France.
Elsa
E
Maitre
Centre Hospitalo-universitaire de Caen, Caen, France.
Edouard
E
Cornet
Centre Hospitalo-universitaire de Caen, Caen, France.
Xavier
X
Troussard
Department of Hematology, Institut Paoli-Calmettes, 232 Bd Ste Margerite, 13009, Marseille, France. aurrant@ipc.unicancer.fr.
Thérèse
T
Aurran-Schleinitz
D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D006801:Humans; D016130:Immunophenotyping / Q000379:methods; D054403:Leukemia, Prolymphocytic, B-Cell / Q000175:diagnosis* / Q000235:genetics* / Q000628:therapy*; D015461:Leukemia, Prolymphocytic, T-Cell / Q000175:diagnosis* / Q000235:genetics / Q000628:therapy; D014184:Transplantation, Homologous / Q000379:methods
D061067:Antibodies, Monoclonal, Humanized; D000074323:Alemtuzumab
B cell; Biology; Diagnosis; Genetics, treatment; Prolymphocytic leukemia; T cell