ORPHA:544
Diffuse large B-cell lymphoma
Synonyms: DLBCL
- Type
- Clinical group
- Group
- Group of disorders
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
Diffuse large B-cell lymphoma is the most common subtype of non-Hodgkin lymphoma (NHL) in adults characterized by a median age of presentation in the sixth decade of life (but also rarely occurring in adolescents and children) with the initial presentation being single or multiple rapidly growing masses (that may or may not be painful) in nodal or extranodal sites (such as thyroid, skin, breast, gastrointestinal tract, testes, bone, or brain) and that can be accompanied by symptoms of fever, night sweats and weight loss. DLBCL has an aggressive disease course, with the elderly having a poorer prognosis than younger patients, and with relapses being common.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Diffuse large B-cell lymphoma | DLBCL |
| fr | Lymphome B diffus à grandes cellules | DLBCL · LDGC |
| de | B-Zell-Lymphom, diffuses großzelliges | = en |
| es | Linfoma difuso de células B grandes | LDCBG · DLBCL |
| it | Linfoma diffuso a grandi cellule B | = en |
| nl | Diffuus grootcellig B-cellymfoom | = en |
| pt | Linfoma difuso de células B grandes | |
| pl | Chłoniak rozlany z dużych komórek B | = en |
| cs | Difuzní velkobuněčný B-lymfom | = en |
| uk | Дифузна велика В-клітинна лімфома | = en |
| zh | 弥漫性大B细胞性淋巴瘤 | = en |
| tr | Yaygın büyük B hücreli lenfoma | = en |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | E | specific | validated |
| ICD-11 | 2A81 | E | specific | validated |
| MONDO | 0018905 | E | validated | |
| MONDO | 18905 | E | validated | |
| MeSH | D016403 | E | validated | |
| UMLS | C0079744 | E | validated | |
| GARD | 3178 | E | validated | |
| MedDRA | 10012818 | 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:300846Aggressive B-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
Children (9):
- ORPHA:364043ALK-positive large B-cell lymphoma
- ORPHA:300849Diffuse large B-cell lymphoma of the central nervous system
- ORPHA:300888Diffuse large B-cell lymphoma with chronic inflammation
- ORPHA:289661Epstein-Barr virus-positive diffuse large B-cell lymphoma
- ORPHA:98839Intravascular large B-cell lymphoma
- ORPHA:86869Lymphomatoid granulomatosis
- ORPHA:98838Primary mediastinal large B-cell lymphoma
- ORPHA:695631Primary vitreoretinal large B-cell lymphoma
- ORPHA:300857T-cell/histiocyte rich large B cell lymphoma
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
26 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 43 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2018[OTHER] |
| Annual incidence | 1-9 / 100 000 | 6.5 / 100 000 | France | Value and class | Validated | OTHERitut National du Cancer 2013[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.79 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 4.931 / 100 000 | United Kingdom | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 4.773 / 100 000 | Slovenia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 4.78 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 6.328 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 6.478 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.765 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.298 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.227 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 5.51 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.229 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.304 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.815 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.244 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 4.586 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 4.559 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.458 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 6.47 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.984 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.827 / 100 000 | Malta | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 5.115 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.673 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.635 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.259 / 100 000 | Slovakia | 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.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/544
- This record as JSON: /data/d/544.json