ORPHA:391
Classic Hodgkin lymphoma
Synonyms: Classic Hodgkin disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- All ages
- Status
- active
Classical Hodgkin lymphoma (CHL) is a B-cell lymphoma characterized histologically by the presence of large mononuclear Hodgkin cells and multinucleated Reed-Sternberg (HRS) cells.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Classic Hodgkin lymphoma | Classic Hodgkin disease |
| fr | Lymphome de Hodgkin classique | Maladie de Hodgkin classique |
| de | Hodgkin-Lymphom, klassisches | Hodgkin-Krankheit, klassische |
| es | Linfoma de Hodgkin clásico | Enfermedad de Hodgkin clásica |
| it | Linfoma di Hodgkin classico | Malattia di Hodgkin classica |
| nl | Klassiek Hodgkin-lymfoom | Klassieke ziekte van Hodgkin |
| pt | Doença de Hodgkin | Linfoma de Hodgkin |
| pl | Klasyczny chłoniak Hodgkina | Klasyczna choroba Hodgkina |
| cs | Klasický Hodgkinův lymfom | Klasická Hodgkinova choroba |
| uk | Класична лімфома Ходжкіна | Класична хвороба Ходжкіна |
| zh | 经典霍奇金淋巴瘤 | 典型霍奇金病 |
| tr | Klasik Hodgkin lenfoma | Klasik Hodgkin hastalığı |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 236000 | E | validated | |
| OMIM | 300221 | BTNT | validated | |
| OMIM | 400021 | BTNT | validated | |
| ICD-10 | C81.0 | BTNT | specific | validated |
| ICD-10 | C81.1 | BTNT | specific | validated |
| ICD-10 | C81.2 | BTNT | specific | validated |
| ICD-10 | C81.3 | BTNT | specific | validated |
| ICD-10 | C81.4 | BTNT | specific | validated |
| ICD-10 | C81.7 | BTNT | specific | validated |
| ICD-10 | C81.9 | BTNT | specific | validated |
| ICD-11 | 2B30.1 | E | specific | validated |
| MONDO | 0009348 | E | validated | |
| UMLS | C1333064 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
3
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:98293Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
4
Genes
0 direct · 1 via subtypesOrphanet records no gene directly on this code.
Recorded by Orphanet on subtypes of this disorder:
| Gene | Subtype | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|---|
| KLHDC8B | ORPHA:98843Classic Hodgkin lymphoma, nodular sclerosis type | Candidate gene tested in | Not yet assessed | HGNC:28557 | ENSG00000185909 | 613169 | 3p21.31 | kelch domain containing 8B | 19706467[PMID] |
5
Prevalence and incidence
30 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 100 000 | 2.463 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe [OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.95 / 100 000 | France | Value and class | Validated | OTHERitut National du Cancer 2013[OTHER] |
| Point prevalence | 1-5 / 10 000 | — | Europe | Class only | Validated | RARECARE surveillance of rare cancers in Europe 2010[OTHER] |
| Lifetime Prevalence | 1-5 / 10 000 | 22.9 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe 2010[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.8 / 100 000 | United States | Value and class | Validated | National Cancer OTHERitute 2008[OTHER]_emedicine[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.25 / 100 000 | China | Value and class | Not yet validated | emedicine[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.25 / 100 000 | Japan | Value and class | Not yet validated | emedicine[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.012 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.267 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.2 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.435 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.261 / 100 000 | Slovakia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.265 / 100 000 | Slovenia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.617 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.091 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.27 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.504 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.443 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.083 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.923 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.418 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.392 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.232 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.333 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.599 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.355 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.361 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.868 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.372 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.701 / 100 000 | Malta | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
6
HPO phenotypes
32 annotations · validated 2024-03-06| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0005374 | Cellular immunodeficiency | |
| HP:0012378 | Fatigue | |
| HP:0002716 | Lymphadenopathy | |
| HP:0002665 | Lymphoma | |
| Frequent (79-30%) | ||
| HP:0002039 | Anorexia | |
| HP:0003418 | Back pain | |
| HP:0100749 | Chest pain | |
| HP:0012735 | Cough | |
| HP:0011227 | Elevated circulating C-reactive protein concentration | |
| HP:0001945 | Fever | |
| HP:0000975 | Hyperhidrosis | |
| HP:0030166 | Night sweats | |
| HP:0004396 | Poor appetite | |
| HP:0000989 | Pruritus | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0001903 | Anemia | |
| HP:0001251 | Ataxia | |
| HP:0005528 | Bone marrow hypocellularity | |
| HP:0002653 | Bone pain | |
| HP:0002105 | Hemoptysis | |
| HP:0002240 | Hepatomegaly | |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0001880 | Increased total eosinophil count | |
| HP:0001974 | Increased total leukocyte count | |
| HP:0001888 | Lymphopenia | |
| HP:0002076 | Migraine | |
| HP:0002664 | Neoplasm | |
| HP:0002797 | Osteolysis | |
| HP:0009830 | Peripheral neuropathy | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0000988 | Skin rash | |
| HP:0001744 | Splenomegaly | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 33493434[PMID]_32703953[PMID]
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/391
- This record as JSON: /data/d/391.json