ORPHA:543
Burkitt lymphoma
Synonyms: Small non-cleaved cell lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Burkitt lymphoma is a rare form of malignant mature B-cell non-Hodgkin lymphoma.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Burkitt lymphoma | Small non-cleaved cell lymphoma |
| fr | Lymphome de Burkitt | Lymphome à petites cellules non clivées |
| de | Burkitt-Lymphom | |
| es | Linfoma de Burkitt | Linfoma de células pequeñas no hendidas |
| it | Linfoma di Burkitt | Linfoma a piccole cellule non clivate |
| nl | Burkitt-lymfoom | Klein niet-gesplitst cellymfoom |
| pt | Linfoma de Burkitt | Linfoma de células pequenas e não-clivadas |
| pl | Chłoniak Burkitta | Chłoniak z małych komórek bez wpuklonego jądra komórkowego |
| cs | Burkittův lymfom | Lymfom malých buněk s neštěpeným jádrem |
| uk | Лімфома Беркітта | Невелика неклітинна лімфома |
| zh | 伯基特淋巴瘤 | 小无裂细胞性淋巴瘤 |
| tr | Burkitt lenfoma | Küçük bölünmemiş hücreli lenfoma |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 113970 | E | validated | |
| ICD-10 | C83.7 | BTNT | specific | validated |
| ICD-10 | C91.8 | BTNT | specific | validated |
| ICD-11 | 2A85.6 | NTBT | specific | validated |
| ICD-11 | XH4KA9 | E | specific | validated |
| MONDO | 0007243 | E | validated | |
| MeSH | D002051 | E | validated | |
| UMLS | C0006413 | E | validated | |
| GARD | 5973 | E | validated | |
| MedDRA | 10006595 | 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 (2):
- ORPHA:300846Aggressive B-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders - ORPHA:289644Epstein-Barr virus-associated malignant lymphoproliferative disorder
rare neoplastic diseases
No children.
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Genes
1 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| MYC | Part of a fusion gene in | Assessed | HGNC:7553 | ENSG00000136997 | 190080 | 8q24.21 | MYC proto-oncogene, bHLH transcription factor |
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Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.17 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe 2003[OTHER] |
| Point prevalence | 1-5 / 10 000 | 12.5 / 100 000 | Africa | Value and class | Validated | National OTHERitutes of Health[OTHER] |
| Point prevalence | 1-9 / 1 000 000 | 0.4 / 100 000 | United States | Value and class | Validated | National Cancer OTHERitute 2001-2009[OTHER] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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
14 annotations · validated 2017-01-03| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0005561 | Abnormality of bone marrow cell morphology | |
| HP:0002149 | Hyperuricemia | |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0100649 | Neoplasm of the oral cavity | |
| Occasional (29-5%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0001392 | Abnormality of the liver | |
| HP:0002733 | Abnormality of the lymph nodes | |
| HP:0000137 | Abnormality of the ovary | |
| HP:0001732 | Abnormality of the pancreas | |
| HP:0001743 | Abnormality of the spleen | |
| HP:0032218 | Decreased proportion of CD4-positive T cells | |
| HP:0002239 | Gastrointestinal hemorrhage | |
| HP:0005214 | Intestinal obstruction | |
| HP:0002017 | Nausea and vomiting | |
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/543
- This record as JSON: /data/d/543.json