title: Management of prolymphocytic leukemia.
abstract: B-cell (B-PLL) and T-cell (T-PLL) prolymphocytic leukemias are rare, poor-prognosis lymphoid neoplasms with similar presentation characterized by symptomatic splenomegaly and lymphocytosis. They can be distinguished from each other and from other T- and B-cell leukemias by careful evaluation of morphology, immunophenotyping, and molecular genetics. The clinical behavior is typically aggressive, although a subset of patients may have an indolent phase of variable length. First-line therapy for T-PLL is with intravenous alemtuzumab and for B-PLL is with combination purine analog-based chemo-immunotherapy. New B-cell receptor inhibitors, such as ibrutinib and idelalisib, may have a role in the management of B-PLL, especially for the patients harboring abnormalities of TP53. Allogenic stem cell transplantation should still be considered for eligible patients and may be the only current therapy capable of delivering a cure. In the past few years, many of the molecular mechanisms underlying disease pathogenesis and progression have been revealed and are likely to lead to the development of novel targeted approaches.
authors.affiliation: Department of Haemato-Oncology, Royal Marsden Biomedical Research Centre, London, UK.
authors.forename: Claire
authors.initials: C
authors.lastname: Dearden
mesh_terms: D000225:Adenine / Q000031:analogs & derivatives; D000074323:Alemtuzumab; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D001402:B-Lymphocytes / Q000166:cytology; D018450:Disease Progression; D006801:Humans; D016130:Immunophenotyping; D007167:Immunotherapy / Q000379:methods; D015463:Leukemia, Prolymphocytic / Q000235:genetics / Q000628:therapy*; D015461:Leukemia, Prolymphocytic, T-Cell / Q000628:therapy*; D009154:Mutation; D010880:Piperidines; D011379:Prognosis; D011687:Purines / Q000627:therapeutic use; D011720:Pyrazoles / Q000627:therapeutic use; D011743:Pyrimidines / Q000627:therapeutic use; D052999:Quinazolinones / Q000627:therapeutic use; D012008:Recurrence; D033581:Stem Cell Transplantation; D013601:T-Lymphocytes / Q000166:cytology; D016896:Treatment Outcome; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D010880:Piperidines; D011687:Purines; D011720:Pyrazoles; D011743:Pyrimidines; D052999:Quinazolinones; C495901:TP53 protein, human; D016159:Tumor Suppressor Protein p53; C551803:ibrutinib; D000074323:Alemtuzumab; D000225:Adenine; C552946:idelalisib