Nodal T-follicular helper cell lymphoma, follicular type
Synonyms: 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
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
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.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Nodal T-follicular helper cell lymphoma, follicular type | 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 |
| fr | Lymphome folliculaire à cellules T | Lymphome folliculaire à cellules T auxiliaires, type folliculaire · Lymphome folliculaire à cellules T helper, type folliculaire · Lymphome nodal à cellules T avec phenotype TFH |
| de | Noduläres T-follikuläres Helferzell-Lymphom, follikulärer Typ | Follikuläres T-Helferzell-Lymphom, follikulärer Typ · Follikuläres T-Zell-Lymphom · Nodales T-Zell-Lymphom mit TFH-Zell-Phänotyp · Nodales peripheres T-Zell-Lymphom mit follikulärem T-Helfer-Phänotyp · Noduläres TFH-Lymphom, follikulärer Typ · nTFHL-F |
| es | Linfoma nodal de células T cooperadoras foliculares, tipo folicular | Linfoma de células T cooperadoras foliculares, tipo folicular · Linfoma folicular de células T · Linfoma nodal TFH, tipo folicular · Linfoma nodal de células T con fenotipo TFH |
| it | Linfoma nodale a cellule T helper follicolari tipo follicolare | Linfoma a cellule T follicolari · Linfoma a cellule T helper follicolari tipo follicolare · Linfoma delle cellule follicolari T helper, tipo follicolare · Linfoma follicolare a cellule T · Linfoma nodale TFH tipo follicolare · Linfoma nodale TFH, tipo follicolare · Linfoma nodale a cellule T con fenotipo TFH · nTFHL-F |
| nl | Nodaal lymfoom van folliculaire T-helpercellen, folliculair type | Folliculair T-cellymfoom · Lymfoom van folliculaire T-helpercellen, folliculair type · Nodaal T-cellymfoom met TFH-fenotype · Nodaal T-cellymfoom met fenotype van folliculaire T-helpercellen · Nodaal TFH-lymfoom, folliculair type · nTFHL-F |
| pt | Linfoma nodal de células T-folicular helper, tipo folicular | Linfoma de células T-folicular · Linfoma de células T-helper foliculares, tipo folicular · Linfoma nodal TFH, tipo folicular · Linfoma nodal de células T com fenótipo TFH · nTFHL-F |
| pl | Węzłowy chłoniak grudkowy z komórek pomocniczych T, typ pęcherzykowy | = en |
| cs | Nodální T-folikulární lymfom z pomocných buněk, folikulární typ | = en |
| uk | Вузлова Т-фолікулярна лімфома з хелперних клітин, фолікулярний тип | Фолікулярна хелперна Т-клітинна лімфома, фолікулярний тип · Фолікулярна Т-клітинна лімфома · Вузлова Т-клітинна лімфома з фенотипом TFH · Вузлова лімфома ЩЗ, фолікулярний тип · nTFHL-F |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C82.7 | NTBT | attributed | validated |
| ICD-11 | 2A90.C | NTBT | attributed | validated |
| ICD-11 | XH6SR1 | E | specific | validated |
| MONDO | 0958095 | E | validated | |
| MONDO | 958095 | E | validated | |
| UMLS | C2700204 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:171918T-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/652650
- This record as JSON: /data/d/652650.json