title: T-cell prolymphocytic leukemia: update and focus on alemtuzumab (Campath-1H).
abstract: T-cell prolymphocytic leukemia (T-PLL) is a rare mature T-cell lymphoproliferative disorder. While the etiology of T-PLL is unknown, recent progress in unraveling the molecular basis of leukemogenesis has been substantial and may yield novel therapeutic targets. T-PLL is a distinct disease entity and the diagnosis can be readily made based on characteristic clinical features and laboratory findings. Prior to the appearance of pentostatin and alemtuzumab in clinical protocols, outcome for T-PLL patients was exceedingly poor with median survival measured in months. While the use of alemtuzumab in particular has improved remissions, the disease remains incurable. Future collaborative efforts investigating novel treatment approaches will be crucial to improving survival for patients with this disease.
authors.affiliation: Department of Medicine, Division of Hematology, Stanford University School of Medicine, CA 94305-5112, USA.
authors.forename: Thai M
authors.initials: TM
authors.lastname: Cao
authors.forename: Steven E
authors.initials: SE
authors.lastname: Coutre
mesh_terms: D000074323:Alemtuzumab; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm / Q000627:therapeutic use*; D000964:Antimetabolites, Antineoplastic / Q000627:therapeutic use; D002869:Chromosome Aberrations; D002880:Chromosomes, Human, Pair 11 / Q000648:ultrastructure; D002883:Chromosomes, Human, Pair 14 / Q000235:genetics / Q000648:ultrastructure; D002898:Chromosomes, Human, Pair 8 / Q000648:ultrastructure; D041321:Chromosomes, Human, X / Q000648:ultrastructure; D002986:Clinical Trials as Topic; D003937:Diagnosis, Differential; D018450:Disease Progression; D005260:Female; D006801:Humans; D007167:Immunotherapy; D017404:In Situ Hybridization, Fluorescence; D015463:Leukemia, Prolymphocytic / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D015461:Leukemia, Prolymphocytic, T-Cell / Q000145:classification / Q000175:diagnosis / Q000235:genetics / Q000628:therapy*; D008297:Male; D015649:Pentostatin / Q000627:therapeutic use; D017346:Protein Serine-Threonine Kinases / Q000502:physiology; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051057:Proto-Oncogene Proteins c-akt; D016693:Receptors, Antigen, T-Cell, alpha-beta / Q000235:genetics; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; D000912:Antibodies, Neoplasm; D000964:Antimetabolites, Antineoplastic; D011518:Proto-Oncogene Proteins; D016693:Receptors, Antigen, T-Cell, alpha-beta; C090851:TCL1A protein, human; D015649:Pentostatin; D000074323:Alemtuzumab; D017346:Protein Serine-Threonine Kinases; D051057:Proto-Oncogene Proteins c-akt