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# Nodal T-cell lymphomas

- Lesion

Uncommon, mostly aggressive lymphomas of mature T cells that arise in lymph nodes. One type, ALK positive, has a good outlook.

Updated Nov 12, 2026

## Key points

- Most are aggressive and respond poorly to chemotherapy, except ALK-positive ALCL.
- ALCL: CD30 positive large 'hallmark' cells with horseshoe-shaped nuclei; ALK rearrangement, classically t(2;5) NPM1::ALK.
- Nodal TFH lymphoma (formerly angioimmunoblastic): PD-1, CXCL13, CD10, BCL6 positive CD4 T cells, proliferating high endothelial venules, hypergammaglobulinaemia and autoimmune cytopenias.
- TFH lymphomas often contain EBV-positive B cells that can grow into B-cell lymphoma.
- T-cell lymphomas can trigger haemophagocytic lymphohistiocytosis.

On this page

- The basics Key points
- What it is
- Structure
- Step by step
- Nodal T-cell lymphomas compared
- Markers
- Memory aid
- Elsewhere in DurgiPedia

- Clinical Clinical notes

- Study Test yourself

- Connections Connections

## What it is

Peripheral (mature) T-cell lymphomas make up a minority of lymphomas in the Americas and Europe and are more common in East Asia. In nodes the main types are peripheral T-cell lymphoma not otherwise specified (a heterogeneous group), nodal T-follicular helper (TFH) cell lymphoma, which disturbs B-cell regulation and causes immune phenomena, and anaplastic large-cell lymphoma (ALCL), whose ALK-positive form occurs in children and young adults and has a good prognosis.

## Structure

- PTCL NOS: diffuse mixture of small to large atypical T cells with irregular nuclei, often with eosinophils and macrophages.
- Nodal TFH lymphoma: effaced node with clear-cell T cells around arborizing high endothelial venules and expanded follicular dendritic cell meshworks.
- ALCL: cohesive sheets of large anaplastic cells, often filling sinuses, with kidney- or horseshoe-shaped nuclei.

## Step by step

- A mature T cell acquires mutations, often in epigenetic regulators (TET2, DNMT3A) or signalling genes (RHOA).
- In TFH lymphoma the tumour cells keep the helper programme and drive B cells abnormally.
- In ALCL, a translocation creates an ALK fusion protein, a constitutive tyrosine kinase activating RAS and JAK/STAT.
- The tumour effaces nodes and spreads to skin, marrow, liver and spleen.

## Nodal T-cell lymphomas compared

| Type | Who | Key markers or genetics | Behaviour |
| --- | --- | --- | --- |
| PTCL, not otherwise specified | Older adults | Mature T-cell markers, often lost CD5 or CD7 | Aggressive |
| Nodal T-follicular helper cell lymphoma | Older adults | PD-1, CXCL13, CD10, BCL6; TET2, RHOA mutations | Aggressive, with rash, autoimmunity, high immunoglobulins |
| ALK-positive anaplastic large-cell lymphoma | Children and young adults | CD30 and ALK; t(2;5) NPM1::ALK | Good prognosis; ALK inhibitors work |

## Markers

- CD3
- CD4
- PD-1
- CXCL13
- CD30
- ALK

## Clinical notes

- Generalized lymphadenopathy with fever, weight loss, rash, eosinophilia or pruritus.
- Anti-CD30 antibody–drug conjugates are active against CD30-positive T-cell lymphomas.

## Memory aid

T-cell lymphomas of the node: mostly bad news, except when ALK lights up in a young patient.

## Test yourself

Think of the answer, then tap the question to see it.

## What are hallmark cells?

Large cells with horseshoe- or kidney-shaped nuclei typical of anaplastic large-cell lymphoma.

## Which translocation is classic for ALK-positive ALCL?

t(2;5), producing the NPM1::ALK fusion kinase.

## Why do patients with nodal TFH lymphoma develop autoimmune cytopenias?

The neoplastic follicular helper T cells disturb germinal centre B-cell maturation, causing hypergammaglobulinaemia and autoantibodies.

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## Elsewhere in DurgiPedia

- [CD4⁺ helper T cell](https://durgical.com/en/immunology/cd4-helper-t-cell/)
- [B-cell activation, class switching and affinity maturation](https://durgical.com/en/immunology/b-cell-activation-class-switching-and-affinity-maturation/)

## Connections

## Related 4

- [Histiocytic disorders: HLH and Langerhans cell histiocytosis](https://durgical.com/en/general-pathology/histiocytic-disorders-hlh-and-langerhans-cell-histiocytosis/)
- [Hodgkin lymphoma](https://durgical.com/en/general-pathology/hodgkin-lymphoma/)
- [T- and NK-cell leukaemias and extranodal lymphomas](https://durgical.com/en/general-pathology/t-and-nk-cell-leukaemias-and-extranodal-lymphomas/)
- [Oncogenes: RAS, MYC, HER2, BCR-ABL and the table to know](https://durgical.com/en/general-pathology/oncogenes-ras-myc-her2-bcr-abl-and-the-table-to-know/)

## Related conditions 2

- [Non-Hodgkin lymphoma](https://durgical.com/en/conditions/non-hodgkin-lymphoma/)
- [Autoimmune haemolytic anaemia](https://durgical.com/en/conditions/autoimmune-haemolytic-anaemia/)

## Microorganisms 1

- [Epstein–Barr virus](https://durgical.com/en/microbiology/epstein-barr-virus/)

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Educational content on the mechanisms of disease; not a diagnostic or treatment guide and no substitute for a health professional's advice. No doses are given.

Sources: [Non-Hodgkin Lymphoma Treatment (PDQ®)–Patient Version](https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq) (National Cancer Institute (NCI), 2026).

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Representative model, for educational purposes only.

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License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/general-pathology/nodal-t-cell-lymphomas/