Angioimmunoblastic T-Cell Lymphoma / Nodal TFH-cell lymphoma
Deterministic view of the source YAML entity. Clinical authority remains with the cited source IDs and reviewer sign-off state.
| ID | DIS-AITL |
|---|---|
| Type | Disease |
| Aliases | AILTAITLAngioimmunoblastic T-Cell LymphomaNodal TFH-cell lymphomaАнгіоімунобластна T-клітинна лімфома / нодальна TFH-лімфома |
| Status | reviewed 2026-04-25 | pending_clinical_signoff |
| Diseases | None declared |
| Sources | SRC-NCCN-BCELL-2025 |
Informational support. All recommendations must be verified by a qualified oncologist. Engineering checks are not clinical validation or treatment approval.
| icd_o_3_morphology | 9705/3 |
|---|---|
| icd_10 | C84.4 |
| who_classification | Mature T-cell neoplasms — Nodal T-follicular helper (TFH) cell lymphoma, angioimmunoblastic-type per WHO 5th ed. |
Notes
CD30 positive in ~40-50% — eligible for brentuximab-CHP. EBV-driven B-cell secondary lymphomas can develop during AITL — separate consideration. Hypogammaglobulinemia + autoimmune cytopenias common.
Used By
Actionability
BMA-IDH2-R140Q-AITL- IDH2 R140Q in AITL — much less common than R172 in T-cell lymphoma (myeloid pattern). Ena...BMA-IDH2-R172K-AITL- IDH2 R172 mutations occur in ~30% of AITL (TFH-derived PTCL). Enasidenib monotherapy acti...
Algorithms
ALGO-AITL-1L- ALGO-AITL-1LALGO-AITL-2L- ALGO-AITL-2L
Disease
DIS-PTCL-NOS- Peripheral T-Cell Lymphoma, Not Otherwise Specified
Indication
IND-AITL-1L-CHOEP- IND-AITL-1L-CHOEPIND-AITL-1L-CHP-BV- IND-AITL-1L-CHP-BVIND-AITL-2L-AZACITIDINE- IND-AITL-2L-AZACITIDINEIND-AITL-2L-BELINOSTAT- IND-AITL-2L-BELINOSTATIND-AITL-2L-ROMIDEPSIN- IND-AITL-2L-ROMIDEPSIN
Questionnaires
QUEST-AITL-1L-STUB- Angioimmunoblastic T-Cell Lymphoma — first line
Red flag
RF-AITL-AUTOIMMUNE-CYTOPENIA- AITL with autoimmune hemolytic anemia (AIHA, DAT+) and/or immune thrombocytopenia (ITP) —...RF-AITL-EBV-DRIVEN-B-CELL- AITL with EBV+ B-cell expansion (large B-cells, EBER+ in microenvironment) — risk of seco...RF-AITL-FRAILTY-AGE- AITL patient frail or elderly unfit for full-dose CHOEP / CHP-Bv induction: ECOG ≥3, OR a...RF-AITL-HYPOGAMMA- AITL with baseline hypogammaglobulinemia (IgG <600 mg/dL) and/or recurrent bacterial infe...RF-AITL-ORGAN-DYSFUNCTION- AITL with baseline organ dysfunction precluding full-dose CHOEP / CHP-Bv: LVEF <50% (anth...RF-AITL-ROMIDEPSIN-INELIGIBLE- Romidepsin inaccessible, contraindicated, or intolerant in AITL 2L+. Scenarios driving in...RF-AITL-TRANSFORMATION-PROGRESSION- AITL primary-refractory or early-relapse: failure to achieve CR after induction (interim...RF-TCELL-CD30-POSITIVE- Reported CD30 expression in T-cell lymphoma; a targeting signal requiring confirmed patho...
Sources
SRC-ESMO-LYMPHOMA-2025- Lymphomas: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-upSRC-ESMO-PTCL-2024- ESMO Clinical Practice Guideline on Peripheral T-cell Lymphomas