6533b82efe1ef96bd12933fa
RESEARCH PRODUCT
Angioimmunoblastic T-cell lymphoma
Emilio IannittoViviana MinardiHans KreipeClaudio TripodoAndrés J.m. Ferrerisubject
CD4-Positive T-LymphocytesMaleEpstein-Barr Virus InfectionsPathologyAutologous transplantHerpesvirus 4 HumanHepatosplenomegalyImmunosuppressive AgentEpstein-Barr Virus InfectionHypergammaglobulinemiaLymph nodeNon-Hodgkin lymphomaAngioimmunoblastic lymphomaB-Lymphocyteseducation.field_of_studyB-LymphocyteLymph NodeHematologyThalidomideSurvival RateTransplantation Autologoumedicine.anatomical_structureOncologyCD4-Positive T-LymphocyteFemaleNeprilysinmedicine.symptomImmunosuppressive AgentsHumanmedicine.medical_specialtyAngioimmunoblastic T-cell lymphomaPopulationHigh endothelial venulesDendritic CellLymphoma T-CellTransplantation AutologousmedicineHumanseducationCell Proliferationbusiness.industryPeripheral T-cell lymphomaDendritic Cellsmedicine.diseasePeripheral T-cell lymphomaLymphomaTransplantationImmunologyLymph NodesGeriatrics and GerontologybusinessStem Cell Transplantationdescription
Angioimmunoblastic T-cell lymphoma (AITL) is a rare and aggressive neoplasm clinically characterized by sudden onset of constitutional symptoms, lymphadenopathy, hepatosplenomegaly, frequent autoimmune phenomena, particularly hemolytic anemia and thrombocytopenia, and polyclonal hypergammaglobulinemia. The lymph node histological picture is also distinctive, constituted by a polymorphic infiltrate, a marked proliferation of high endothelial venules, and a dense meshwork of dentritic cells. The neoplastic CD4+ T-cells represent a minority of the lymph node cell population; its detection is facilitated by the aberrant expression of CD10. Almost all cases arbor an EBV infected B-cell population. Patients with AITL have a poor prognosis with conventional treatment, with a median overall survival of less than 3 years. Patients achieving a good clinical response seem beneficiate from a consolidation with high-dose therapy and autologous stem cell transplantation. Constitutional symptoms and autoimmune phenomena, and some times also the neoplastic masses may respond to immunosuppressive or immunomodulatory agents such as thalidomide. © 2008 Elsevier Ireland Ltd. All rights reserved.
| year | journal | country | edition | language |
|---|---|---|---|---|
| 2008-12-01 |