Patients with angioimmunoblastic T-cell lymphoma (AITL) may demonstrate a sustained response when treated with 5-azacitidine, according to a study published in Blood.

Prognosis for peripheral T-cell lymphomas such as AITL is poor, with a median overall survival of approximately 6 months. Of patients with AITL, 80% have mutations in TET2, 25% have mutations in DNMT3A, and 25% have mutations in IDH2; these genes are also mutated in myeloid neoplasms. Treatment of both patients with AITL and patients with myeloid neoplasms appears efficacious to a degree that directly correlates with mutations in these genes.

In this study, researchers retrospectively analyzed data from 12 patients (median age 70.5 years) with AITL who were treated with 5-azacitidine for concomitant myeloid neoplasm or as compassionate therapy in relapsing/refractory AITL; 5 patients (41%) had an associated myeloid neoplasm. Patients received 75 mg/m2 5-azacitidine daily, subcutaneously, for 7 consecutive days, every 28 days, for a median of 5.5 cycles. Half the patients received rituximab in addition to 5-azacitidine due to the presence of Epstein-Barr virus replication or B-blasts. Tumor samples were collected from the patients and sequenced to determine TET2, IDH2, DNMT3A, and RHOA status.

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The patients tolerated the treatment well. No patients developed febrile neutropenia, and there were no treatment-related deaths or deaths due to myeloid neoplasm. Six patients (50%) showed a complete response and 3 patients (25%) showed a partial response, yielding an overall response rate of 75%.

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The median progression-free survival was 15 months; the median overall survival was 21 months, with 5 patients showing sustained response and complete remission more than 23 months after treatment was initiated.

TET2, DNMT3A, RHOA, and IDH2 mutations were found in 58%, 33%, 41%, and 8% of patients, respectively. No relationship between number or presence of mutations and response to treatment was found.

All patients with an associated myeloid neoplasm responded to the treatment, and 4 of 7 patients without an associated myeloid neoplasm demonstrated a response, suggesting that success of the treatment is not limited only to patients with an associated myeloid disease.

The researchers concluded, “The mechanism of action of 5-azacitidine in AITL is not elucidated yet…. Several other questions remain unanswered [and] warrant a prospective study.”

Disclosure: Multiple authors declare affiliations with Celgene.


1. Lemonnier F, Dupuis J, Sujobert P, et al. Treatment with 5-Azacytidine Induces a Sustained Response in Patients with Angioimmunoblastic T-Cell Lymphoma [published online October 2, 2018]. Blood. doi: 10.1182/blood-2018-04-840538