Primary cutaneous gamma/delta-positive T-cell lymphoma
- Type
- Disease
- Group
- Disorder
- Status
- active
Primary cutaneous gamma/delta-positive T-cell lymphoma is a rare, usually aggressive, subtype of cutaneous T-cell lymphoma characterized by infiltration of the epidermis, dermis or subcutaneous tissue by a clonal population of mature, gamma/delta positive cytotoxic T-cells. Typically it presents with ulcerating plaques, tumors, or subcutaneous nodules on the skin of the extremities, however, frequent involvement of mucosal and extranodal sites (such as the nasal cavity, gastrointestinal tract or lungs) is also observed. Cases associated with panniculitis may present with hemophagocytic syndrome (abrupt onset of fever, rash, cytopenia, hepatosplenomegaly and neurological compromise). Infiltration of lymph nodes, spleen and bone marrow is uncommon and resistance to multilineage chemotherapy is reported.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary cutaneous gamma/delta-positive T-cell lymphoma | |
| fr | Lymphome T cutané primitif de phénotype TCRgamma/delta | |
| de | Primär kutanes T-Zell-Lymphom, gamma/delta-positives | |
| es | Linfoma cutáneo primario de células T gamma/delta | |
| it | Linfoma cutaneo primitivo a cellule T gamma/delta-positivo | |
| nl | Primair cutaan gamma/delta-positief T-cellymfoom | |
| pt | Linfoma cutâneo de células gama/delta-positivas | |
| pl | Pierwotny chłoniak skóry z gamma/delta pozytywnych komórek T | |
| cs | Primární kožní gama/delta pozitivní T-buněčný lymfom | |
| uk | Первинна шкірна гамма/дельта-позитивна Т-клітинна лімфома | |
| zh | 原发性皮肤γ/δ阳性T细胞淋巴瘤 | |
| tr | Primer kutanöz gama / delta pozitif T hücresi lenfoma |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C84.5 | NTBT | attributed | validated |
| ICD-11 | 2B0Y | NTBT | index | validated |
| MONDO | 0015812 | E | validated | |
| UMLS | C1707547 | 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:178551Aggressive primary cutaneous T-cell lymphoma
rare skin diseases; 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/178533
- This record as JSON: /data/d/178533.json