Angioimmunoblastic T-Cell Lymphoma.
Angioimmunoblastic T-cell lymphoma is a follicular T-helper-derived neoplasm displaying a peculiar morphologic appearance and biological complexity. New mutations have been described that contribute to elucidating the underlying pathogenetic events. The disease behaves aggressively and typically affects elderly patients. The outcomes reported with anthracycline-containing regimens are poor; therefore autologous transplantation in first remission should be offered whenever possible. Newer approaches are urgently needed for relapsed and refractory patients. Newly approved agents show activity in pretreated patients but response durations are short. Innovative induction strategies (CHOP + biologic agent) should be designed to enhance response quality, facilitate transplantation, and prolong survival.
Institute of Hematology "L. e A. Seràgnoli", University of Bologna, Via Massarenti, 9, Bologna 40138, Italy.
Alessandro
A
Broccoli
Institute of Hematology "L. e A. Seràgnoli", University of Bologna, Via Massarenti, 9, Bologna 40138, Italy. Electronic address: pierluigi.zinzani@unibo.it.
Pier Luigi
PL
Zinzani
D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D064592:Autografts; D003520:Cyclophosphamide / Q000627:therapeutic use; D018572:Disease-Free Survival; D004317:Doxorubicin / Q000627:therapeutic use; D008224:Lymphoma, Follicular / Q000401:mortality / Q000628:therapy*; D016399:Lymphoma, T-Cell / Q000401:mortality / Q000628:therapy*; D011241:Prednisone / Q000627:therapeutic use; D033581:Stem Cell Transplantation; D015996:Survival Rate; D014750:Vincristine / Q000627:therapeutic use
D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide; D011241:Prednisone
Angioimmunoblastic T-cell lymphoma; Autologous stem cell transplantation; Brentuximab vedotin; CHOP; DNMT3A; Follicular T-helper; IDH2; Lenalidomide