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Start free with EleplanNodal T-follicular helper cell lymphoma, follicular type
ORPHA:652650Disease
Also called Follicular T-cell Lymphoma · Follicular helper T-cell lymphoma, follicular type · Nodal T-cell lymphoma with TFH phenotype · Nodal TFH lymphoma, follicular type · nTFHL-F
What it is
A rare T-cell non-Hodgkin lymphoma characterized by generalized lymphadenopathy and predominantly follicular or perifollicular growth pattern, consistent with the expression of T-follicular helper markers. Affected individuals distinctly lack histologic features (such as proliferation of high endothelial venules and expanded follicular dendritic meshworks) typically associated to angioimmunoblastic T-cell lymphoma. It affects elderly individuals (mostly at the sixth decade of life) that present with advanced-stage disease. Majority of the cases have disseminated nodal involvement, whereas involvement of extranodal sites including skin, liver, spleen and bone marrow are also reported. Patients may therefore develop skin rash and immune manifestations.
Key facts
- Inheritance
- Not applicable
- Classified as
- Disease
Recorded for the broader condition
- Prevalence
- 1-9 / 1 000 000 (annual incidence, Europe)T-cell non-Hodgkin lymphoma
Orphanet records these for the broader condition rather than for this specific form. They are a starting point, not a figure for this subtype — subtypes often differ, and some are defined by being rarer than the condition they sit under.
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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