Chapter 9
Angioimmunoblastic T-cell Lymphoma
Jehan Dupuis,
Franck Morschhauser,
Jehan Dupuis
Lymphoid Malignancies Unit, Henri Mondor University Hospital, Créteil, France
Search for more papers by this authorFranck Morschhauser
Department of Hematology, Lille University Hospital, Lille, France
Search for more papers by this authorJehan Dupuis,
Franck Morschhauser,
Jehan Dupuis
Lymphoid Malignancies Unit, Henri Mondor University Hospital, Créteil, France
Search for more papers by this authorFranck Morschhauser
Department of Hematology, Lille University Hospital, Lille, France
Search for more papers by this authorBook Editor(s):Owen A. O'Connor M.D., Ph.D.,
Won Seog Kim,
Pier Luigi Zinzani M.D., Ph.D.,
Owen A. O'Connor M.D., Ph.D.
American Cancer Society Research Professor Professor of Medicine
Department of Medicine, Division of Hematology and Oncology, Program for T-Cell Lymphoma Research, Department of Microbiology, Immunology, and Cancer Research, University of Virginia Cancer Center, Charlottesville, VA, USA
Search for more papers by this authorWon Seog Kim
Sungkyunkwan University School of Medicine, Seoul, Korea
Search for more papers by this authorPier Luigi Zinzani M.D., Ph.D.
Professor of Hematology
Department of Medicine, Program for Lymphomas and Chronic Lymphocytic Leukemia, University of Bologna, Bologna, Italy
Search for more papers by this authorFirst published: 19 February 2021
Summary
The disease today referred to as angioimmunoblastic T-cell lymphoma (AITL) was first described in the 1970s. The clinical presentation has some particularities that play a role in defining AITL as a distinct clinicopathological entity. As AITL is associated with recurrent genetic mutations related to clonal hematopoiesis, it is probable that clonal hematopoiesis of indeterminate potential represents the major risk factor for the development of AITL. The cellular derivation of AITL from Tfh cells provides a rational model to explain several of the peculiar pathological and biological features inherent to AITL. Several conventional chemotherapy regimens used in the treatment of relapsed or refractory B-cell malignancies have been specifically explored in the treatment of T-cell lymphomas. Several drugs have obtained approval in the United States and/or other countries for the treatment of relapsed or refractory T-cell lymphomas.
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