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Title: Rapid and durable complete remission of refractory AITL with azacitidine treatment in absence of TET2 mutation or concurrent MDS.
Epworth Authors: Yannakou, Costas
Prince, Miles
Other Authors: Gregory, Gareth
Dickinson, Michael
Wong, Jonathon
Bomberry, Piers
Corboy, Greg
Kats, Lev
Crozier, Tim
Kumar, Beena
Opat, Stephen
Short, Jake
Keywords: Refractory AITL
Angioimmunoblastic T-Cell Lymphoma (AITL)
Azacitidine Treatment
TET2 Mutation
Concurrent MDS
Epigenetic Modifiers
Hypomethylating Agents (HMA)
Myelodysplastic Syndromes
Hematopoietic Cells
Sequencing Studies
DNA Methylation
Cutaneous Lesions
Complete Metabolic Remission (CMR)
Hybridization-Based NGS Panel
T-cell Lymphoma Phenotypes
Clinical Remission
Genomic Instability
Mutational Profiling
HMA Therapy
Epworth Centre for Immunotherapies and Snowdome Laboratories
Molecular Oncology and Cancer Immunology
Cancer Services Clinical Institute, Epworth HealthCare, Victoria, Australia
Issue Date: Feb-2019
Publisher: Lippincott Williams & Wilkins
Citation: HemaSphere, 3(2), e187.
Abstract: Angioimmunoblastic T-cell lymphoma (AITL) is a rare disease entity associated with poor prognosis and no improvement in overall survival over the last 20 years. The genomic landscape of AITL has revealed frequent mutation of epigenetic modifiers TET2 (76%), DNMT3A (33%) and IDH2 (20%), genetic mutations that may be predictive of response to hypomethylating agents (HMA) in myelodysplastic syndromes. Genomic profiling has also demonstrated TET2 mutations to be present in both malignant and non-malignant hematopoietic cells of affected individuals, suggesting loss of TET2 to be the initiating mutation, following which secondary mutations direct the lineage phenotype of subsequent malignancy [eg, secondary RHOA mutations in AITL versus myeloid-lineage associated mutations in genes such as RAS leading to myelodysplasia (MDS) / chronic myelomonocytic leukemia (CMML)]. The potential efficacy of HMAs in the treatment of AITL has emerged from the observation of regressing lymphadenopathy in patients treated for their concomitant MDS, however, such AITL responses may have been confounded by frequent concurrent rituximab administration for Epstein-Barr virus (EBV)-reactivation which is characteristic of this disease.
DOI: 10.1097/HS9.0000000000000187
PubMed URL:
ISSN: 2572-9241
Journal Title: Hemasphere
Type: Journal Article
Affiliated Organisations: Monash Health, Clayton, Victoria, Australia
School of Clinical Sciences at Monash Health, Monash University, Clayton, Victoria, Australia
Clinical Haematology, Peter MacCallum Cancer Centre and Royal Melbourne Hospital, Parkville, Victoria, Australia
Sir Peter MacCallum Department of Oncology, University of Melbourne, Parkville, Victoria, Australia
Research Division, Peter MacCallum Cancer Centre, Parkville, Victoria, Australia
Type of Clinical Study or Trial: Case Reports
Appears in Collections:Cancer Services

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