Angioimmunoblastic T-cell lymphoma
Synonyms: Immunoblastic lymphadenopathy · Lymphogranulomatosis X · T-cell lymphoma, AILD type · AILT
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare T-cell non-Hodgkin lymphoma characterized by infiltration of lymph nodes by neoplastic cells of T follicular helper cell origin with a polymorphous inflammatory background including markedly increased follicular dendritic cells and EBV-positive B-cells, as well as prominent proliferation of high endothelial venules. The spleen, liver, skin, and bone marrow are also frequently involved. Patients typically present with generalized lymphadenopathy, hepatosplenomegaly, systemic symptoms, and polyclonal hypergammaglobulinemia. Pruritic skin rash, arthritis, pleural effusion, and ascites may also be observed. The condition is aggressive with generally poor prognosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Angioimmunoblastic T-cell lymphoma | Immunoblastic lymphadenopathy · Lymphogranulomatosis X · T-cell lymphoma, AILD type · AILT |
| fr | Lymphome T angioimmunoblastique | AILT · Lymphadénopathie immunoblastique · Lymphogranulomatose X · Lymphome T type AILD |
| de | T-Zell-Lymphom, angioimmunoblastisches | AILT · Lymphadenopathie, immunoblastische · Lymphogranulomatose X · T-Zell-Lymphom vom AILD-Typ |
| es | Linfoma angioinmunoblástico de células T | Linfoma de células T tipo AILD |
| it | Linfoma angioimmunoblastico a cellule T | AILT · Linfadenopatia immunoblastica · Linfogranulomatosi X · Linfoma a cellule T, tipo AILD |
| nl | Angio-immunoblastisch T-cellymfoom | AILT · Immunoblastische lymfadenopathie · Lymfogranulomatose X · T-cellymfoom, AILD-type |
| pt | Linfoma de células T angioimunoblástico | |
| pl | Chłoniak angioimmunoblastyczny z komórek T | AILT · Chłoniak z komórek T, typ AILD · Limfadenopatia limfoblastyczna · Limfogranulomatoza X |
| cs | T-angioimunoblastický lymfom | AILT · Imunoblastická lymfadenopatie · Lymfogranulomatóza X · T-buněčný lymfom, AILD typ |
| uk | Ангіоімунобластна Т-клітинна лімфома | AILT · Імунобластна лімфаденопатія · Лімфогранулематоз X · Т-клітинна лімфома, тип AILD |
| zh | 血管免疫母细胞性T细胞淋巴瘤 | 免疫母细胞性淋巴结病 |
| tr | Anjiyoimmünoblastik T-hücreli lenfoma | AILT · Lenfogranülomatoz X · T hücreli lenfoma, AILD tipi · İmmünoblastik lenfadenopati |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C86.5 | E | specific | validated |
| ICD-11 | 2A90.9 | E | specific | validated |
| MONDO | 0004977 | E | validated | |
| UMLS | C0020981 | E | validated | |
| GARD | 11973 | E | validated | |
| MedDRA | 10002449 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.05 / 100 000 | United States | Value and class | Validated | 16150940[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/86886
- This record as JSON: /data/d/86886.json