Peripheral T-cell lymphomas, unspecified (or not otherwise specified): a review.
Peripheral T-cell lymphomas (PTCL) comprises a heterogeneous group of haematological tumours, which originate from mature T-cells, and constitute less than 15% of all non-Hodgkin's lymphomas (NHLs) in adults. The current WHO classification recognizes nine distinct clinicopathologic peripheral T-cell NHLs, being the 'unspecified variant' (PTCL-U) the most common subtype. These neoplasms often present in advanced stage at diagnosis, and most commonly have an aggressive clinical course requiring prompt treatment. The rarity of these tumours requires additional studies to better understand their biology and search for new therapies which may hopefully improve the dismal outcome of most patients. This review aims to describe the pathobiological aspects as well the clinical characteristics and current therapeutic strategies of the PTCLs, with special attention to the group of PTCL-U.
Rodriguez-Abreu
Delvys
D
IOSI, Oncology Institute of Southern Switzerland, Ospedale San Giovanni, Bellinzona, Switzerland.
Filho
Volmar Belisario
VB
Zucca
Emanuele
E
D020533:Angiogenesis Inhibitors / Q000627:therapeutic use; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D020869:Gene Expression Profiling; D018380:Hematopoietic Stem Cell Transplantation; D006801:Humans; D016130:Immunophenotyping; D000922:Immunotoxins / Q000627:therapeutic use; D016411:Lymphoma, T-Cell, Peripheral / Q000235:genetics / Q000276:immunology / Q000628:therapy*; D011379:Prognosis; D061988:Proteasome Inhibitors
D020533:Angiogenesis Inhibitors; D000911:Antibodies, Monoclonal; D000922:Immunotoxins; D061988:Proteasome Inhibitors