VERY-RARE CANCERLymphomaWHO 5th Edition Classification

Angioimmunoblastic T-cell Lymphoma (AITL)

Very Rare Malignancy (1–5 cases per 1,000,000 / year) • Clinical Staging, Genomic Targets & Vetted Specialists

A peripheral T-cell lymphoma arising from follicular helper T cells. It behaves as much like an immune disorder as a cancer, with widespread lymph node enlargement, rashes, autoimmune phenomena and profound systemic symptoms. Expanded EBV-positive B cells within the tumour are characteristic and can complicate diagnosis.

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Who It Affects

Older adults, median around 65; slight male predominance

Annual Incidence

Approximately 0.05 per 100,000 per year

Clinical Evidence Review

Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.

Clinical Presentation & Hallmark Symptoms

Presenting signs most frequently observed across clinical case series

Generalised lymph node enlargement developing over weeks
High fevers, drenching night sweats and weight loss
Skin rash, often itchy and widespread
Enlarged liver and spleen
Autoimmune haemolytic anaemia, arthralgia and polyclonal raised immunoglobulins

Genomic Profiling & Defining Molecular Lesions

Critical diagnostic fusions, somatic mutations, and therapeutic targets

Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.

TET2DNMT3AIDH2 R172RHOA G17VTFH phenotype - CD10, BCL6, CXCL13, PD-1, ICOS

Standard-of-Care Treatment Protocol

Frontline and multimodal strategies established under international consensus guidelines

1

CHOP or CHOEP chemotherapy as first-line therapy

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

2

Autologous stem cell transplant consolidation in first remission for fit patients

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

3

Epigenetic agents - romidepsin, azacitidine, belinostat - particularly given TET2/DNMT3A lesions

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

4

Pralatrexate or single-agent options in relapse

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

5

Allogeneic transplant in selected relapsed patients

Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.

Clinical Trial Advisory: For rare and ultra-rare malignancies, enrollment in an active clinical trial or expanded-access program is widely considered the preferred standard of care by ASCO and NCCN panels.

Prognosis & Disease Trajectory

Objective clinical outlook without false reassurance

Guarded. Five-year overall survival is roughly 30-35% with conventional chemotherapy. Younger patients who reach autologous transplant in first remission do better; the International Prognostic Index and the AITL-specific scores guide expectations.

Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.

Active Research, Biomarkers & Clinical Trials

Novel investigational agents, phase I/II trials, and international rare disease consortia

Hypomethylating agents combined with CHOP show promise given the recurrent epigenetic mutations, and IDH2-mutant disease is a candidate for IDH2 inhibition. Duvelisib and JAK inhibitors are in trials.

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Expert Clinicians

Verified Specialists for Angioimmunoblastic T-cell Lymphoma

Browse All Oncologists

Dr. Steven M. Horwitz

Medical Oncologist

20+ yrs exp

MSKCC

New York, USA

T-cell LymphomasAITLNovel Therapies
Vetted DirectoryConsult Specialist

Dr. Francesca Lim

Hematologist

20+ yrs exp

SGH

Singapore, Singapore

HematologyAITLCombined Chemo-immunotherapy
Vetted DirectoryConsult Specialist

Prof. Ulrich Jäger

Hematologist

30+ yrs exp

Medical University of Vienna

Vienna, Austria

HematologyAITL TrialsTreatment Guidelines
Vetted DirectoryConsult Specialist

Dr. Reena Nair

Hematologist

25+ yrs exp

TMC

Kolkata, India

HematologyAITLNovel Agents
Vetted DirectoryConsult Specialist
Hospital Network

Designated Cancer Centers with Dedicated Programs

Search All 72 NCI Centers

Memorial Sloan Kettering Cancer Center

NY

NCI-Designated Comprehensive Cancer Center

World-leading rare tumor board & pediatric solid tumor protocols

Proton TherapyCAR-T Cell TherapyRobotic Surgery
1,000+ Active Clinical TrialsView Center Profile

University of Texas MD Anderson Cancer Center

TX

NCI-Designated Comprehensive Cancer Center

Largest specialized rare cancer and sarcoma multidisciplinary program

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
1,200+ Active Clinical TrialsView Center Profile

Dana-Farber / Harvard Cancer Center

MA

NCI-Designated Comprehensive Cancer Center

Pioneering genomic molecular tumor boards & rare histologies

Proton TherapyCAR-T Cell TherapyPhase I Unit
1,100+ Active Clinical TrialsView Center Profile

Mayo Clinic Comprehensive Cancer Center

MN

NCI-Designated Comprehensive Cancer Center

High-volume surgical oncology & rare endocrine/neuroendocrine expertise

Proton TherapyCAR-T Cell TherapyCyberKnife SBRT
600+ Active Clinical TrialsView Center Profile

Frequently Asked Clinical Questions

Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options

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Independent Tumor Board Review

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