ORPHA:545
Follicular lymphoma
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adult
- Status
- active
Follicular lymphoma is a form of non-Hodgkin lymphoma characterized by a proliferation of B cells whose nodular structure of follicular architecture is preserved.
1
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Follicular lymphoma | |
| fr | Lymphome folliculaire | |
| de | Lymphom, follikuläres | |
| es | Linfoma folicular | |
| it | Linfoma follicolare | |
| nl | Folliculair lymfoom | |
| pt | Linfoma folicular | |
| pl | Chłoniak grudkowy | |
| cs | Folikulární lymfom | |
| uk | Фолікулярна лімфома | |
| zh | 滤泡型淋巴瘤 | |
| tr | Foliküler lenfoma |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 613024 | BTNT | validated | |
| ICD-10 | C82.0 | BTNT | specific | validated |
| ICD-10 | C82.1 | BTNT | specific | validated |
| ICD-10 | C82.2 | BTNT | specific | validated |
| ICD-10 | C82.3 | BTNT | specific | validated |
| ICD-10 | C82.4 | BTNT | specific | validated |
| ICD-10 | C82.5 | BTNT | specific | validated |
| ICD-10 | C82.6 | BTNT | specific | validated |
| ICD-10 | C82.7 | BTNT | specific | validated |
| ICD-10 | C82.9 | BTNT | specific | validated |
| ICD-11 | 2A80 | E | specific | validated |
| ICD-11 | 2A80.0 | BTNT | specific | validated |
| ICD-11 | 2A80.1 | BTNT | specific | validated |
| ICD-11 | 2A80.2 | BTNT | specific | validated |
| ICD-11 | 2A80.4 | BTNT | specific | validated |
| ICD-11 | 2A80.5 | BTNT | specific | validated |
| ICD-11 | 2A80.6 | BTNT | specific | validated |
| ICD-11 | 2A80.Y | BTNT | attributed | validated |
| MONDO | 0018906 | E | validated | |
| MeSH | D008224 | E | validated | |
| UMLS | C0024301 | E | validated | |
| GARD | 2356 | E | validated | |
| MedDRA | 10085128 | 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:300842Indolent B-cell non-Hodgkin lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
4
Genes
4 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| HLA-DRB1 | Major susceptibility factor in | Assessed | HGNC:4948 | ENSG00000196126 | 142857 | 6p21.32 | major histocompatibility complex, class II, DR beta 1 | 23791106[PMID] |
| BCL2 | Part of a fusion gene in | Assessed | HGNC:990 | ENSG00000171791 | 151430 | 18q21.33 | BCL2 apoptosis regulator | 23953419[PMID]_22684552[PMID] |
| BCL6 | Part of a fusion gene in | Assessed | HGNC:1001 | ENSG00000113916 | 109565 | 3q27.3 | BCL6 transcription repressor | 22968395[PMID] |
| IGH | Part of a fusion gene in | Assessed | HGNC:5477 | 146910 | 14q32.33 | immunoglobulin heavy locus | 23953419[PMID] |
5
Prevalence and incidence
26 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-5 / 10 000 | 37 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2018[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.95 / 100 000 | France | Value and class | Validated | OTHERitut National du Cancer 2013[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.192 / 100 000 | Europe | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.348 / 100 000 | Bulgaria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.72 / 100 000 | Estonia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.579 / 100 000 | Finland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.363 / 100 000 | Latvia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.277 / 100 000 | Lithuania | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 1 000 000 | 0.368 / 100 000 | Poland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.52 / 100 000 | Austria | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.04 / 100 000 | Belgium | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.665 / 100 000 | Croatia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.687 / 100 000 | Czech Republic | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.456 / 100 000 | Germany | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.769 / 100 000 | Iceland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.08 / 100 000 | Ireland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.541 / 100 000 | Italy | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.759 / 100 000 | Malta | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 3.298 / 100 000 | Norway | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.364 / 100 000 | Portugal | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.046 / 100 000 | Slovakia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 1.389 / 100 000 | Slovenia | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.375 / 100 000 | Spain | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.916 / 100 000 | Switzerland | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.977 / 100 000 | Netherlands | Value and class | Validated | RARECARE surveillance of rare cancers in Europe[OTHER] |
| Annual incidence | 1-9 / 100 000 | 2.766 / 100 000 | United Kingdom | 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
17 annotations · validated 2024-01-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001945 | Fever | |
| HP:0002716 | Lymphadenopathy | |
| HP:0002665 | Lymphoma | |
| HP:0100721 | Mediastinal lymphadenopathy | |
| HP:0001824 | Weight loss | |
| Frequent (79-30%) | ||
| HP:0012378 | Fatigue | |
| HP:0030166 | Night sweats | |
| HP:0001744 | Splenomegaly | |
| Occasional (29-5%) | ||
| HP:0002585 | Abnormality of the peritoneum | |
| HP:0001541 | Ascites | |
| HP:0003072 | Hypercalcemia | |
| HP:0025435 | Increased circulating lactate dehydrogenase concentration | |
| HP:0001004 | Lymphedema | |
| HP:0001287 | Meningitis | |
| HP:0002202 | Pleural effusion | |
| HP:0200036 | Skin nodule | |
| Very rare (<4-1%) | ||
| HP:0033823 | Mediastinal mass | |
Run the differential on the 5 obligate / very frequent terms
Annotation source: 30855794[PMID]_31814159[PMID]
7
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/545
- This record as JSON: /data/d/545.json