ORPHA:2584
Classic mycosis fungoides
Synonyms: Mycosis fungoides, Alibert-Bazin type
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
A form of cutaneous T-cell lymphoma characterized by slow progression from patches to more infiltrated plaques and eventually to tumors. In classic mycosis fungoides (MF), extracutaneous involvement may occur.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Classic mycosis fungoides | Mycosis fungoides, Alibert-Bazin type |
| fr | Mycosis fongoïde classique | Mycosis fongoïde type Alibert-Bazin |
| de | Klassische Mycosis fungoides | Mycosis fungoides Typ Alibert-Bazin |
| es | Micosis fungoide clásica | Micosis fungoide tipo Alibert-Bazin |
| it | Micosi fungoide classica | Micosi fungoide tipo Alibert-Bazin |
| nl | Klassieke mycosis fungoides | Mycosis fungoides, Alibert-Bazin-type |
| pt | Micose fungoide clássica | Micose fungoide, tipo Alibert-Bazin |
| pl | Klasyczny ziarniniak grzybiasty | Ziarniniak grzybiasty typu Aliberta i Bazina |
| cs | Klasická forma mycosis fungoides | Mycosis fungoides, typ Alibert-Bazin |
| uk | Класичні грибкові мікози | Мікоз фунгоїдний, тип Аліберта-Базена |
| zh | 经典型蕈样真菌病 | 蕈样肉芽肿病，Alibert-Bazin亚型 |
| tr | Klasik mikoz fungoides | Mikoz fungoides, Alibert-Bazin tipi |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 254400 | E | validated | |
| ICD-10 | C84.0 | NTBT | attributed | validated |
| ICD-11 | 2B01 | E | specific | validated |
| ICD-11 | XH8R56 | E | specific | validated |
| MONDO | 0009691 | E | validated | |
| UMLS | C4707404 | E | validated | |
| GARD | 3863 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:178566Mycosis fungoides and variants
rare skin diseases; rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
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Genes
3 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| TNFRSF1B | Disease-causing somatic mutation(s) in | Assessed | HGNC:11917 | ENSG00000028137 | 191191 | 1p36.22 | TNF receptor superfamily member 1B | 26258847[PMID] |
| CD28 | Part of a fusion gene in | Assessed | HGNC:1653 | ENSG00000178562 | 186760 | 2q33.2 | CD28 molecule | 26258847[PMID] |
| CTLA4 | Part of a fusion gene in | Assessed | HGNC:2505 | ENSG00000163599 | 123890 | 2q33.2 | cytotoxic T-lymphocyte associated protein 4 | 26258847[PMID] |
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Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.58 / 100 000 | United States | Value and class | Validated | 32141115[PMID] |
| Annual incidence | 1-9 / 1 000 000 | 0.5 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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HPO phenotypes
29 annotations · validated 2024-09-20| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0004332 | Abnormal lymphocyte morphology | |
| HP:0000958 | Dry skin | |
| HP:0000964 | Eczematoid dermatitis | |
| HP:0010783 | Erythema | |
| HP:0002665 | Lymphoma | |
| HP:0008069 | Neoplasm of the skin | |
| HP:0000989 | Pruritus | |
| HP:0200035 | Skin plaque | |
| HP:0000988 | Skin rash | |
| Frequent (79-30%) | ||
| HP:0001596 | Alopecia | |
| HP:0012192 | Cutaneous T-cell lymphoma | |
| HP:0025475 | Erythematous macule | |
| HP:0001053 | Hypopigmented skin patches | |
| HP:0007400 | Irregular hyperpigmentation | |
| HP:0002716 | Lymphadenopathy | |
| HP:0001029 | Poikiloderma | |
| Occasional (29-5%) | ||
| HP:0000492 | Abnormal eyelid morphology | |
| HP:0001597 | Abnormal nail morphology | |
| HP:0005561 | Abnormality of bone marrow cell morphology | |
| HP:0007488 | Diffuse skin atrophy | |
| HP:0000969 | Edema | |
| HP:0001019 | Erythroderma | |
| HP:0002240 | Hepatomegaly | |
| HP:0000962 | Hyperkeratosis | |
| HP:0200042 | Skin ulcer | |
| HP:0001744 | Splenomegaly | |
| Very rare (<4-1%) | ||
| HP:0001945 | Fever | |
| HP:0030166 | Night sweats | |
| HP:0001824 | Weight loss | |
Run the differential on the 9 obligate / very frequent terms
Annotation source: 37124514[PMID]_27871620[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/2584
- This record as JSON: /data/d/2584.json