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.
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.
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.
Elsewhere in DurgiPedia
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Microorganisms1
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 (National Cancer Institute (NCI), 2026).
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Representative model, for educational purposes only.