T-cell/histiocyte rich large B cell lymphoma
Synonyms: THRLBCL
- Type
- Disease
- Group
- Disorder
- Inheritance
- Multigenic/multifactorial, Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
T-cell/histiocyte rich large B cell lymphoma (THRLBCL) is a rare variant of diffuse large B-cell lymphoma (DLBCL), mainly affecting middle-aged men and often not being discovered until an advanced disease stage, with involvement of the spleen, liver and bone marrow occurring at a greater frequency than in DLBCL. It is often difficult to diagnose due to its similarity with other lymphoid diseases such as classic Hodgkin lymphoma and nodular lymphocyte-predominant Hodgkin lymphoma and has an aggressive clinical course.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | T-cell/histiocyte rich large B cell lymphoma | THRLBCL |
| fr | Lymphome à grandes cellules B riche en cellules T/histiocytes | = en |
| de | T-Zell/histiozytenreiches großzelliges B-Zell-Lymphom | = en |
| es | Linfoma B de células grandes rico en células -T/histiocitos | = en |
| it | Linfoma a grandi cellule B ricco in istiociti/cellule T | = en |
| nl | T-cel/histiocytenrijk grootcellig B-cellymfoom | = en |
| pt | Linfoma de células B grandes com abundantes células T / histiócitos | |
| pl | Chłoniak z dużych komórek B bogaty w komórki T/histiocyty | = en |
| cs | T-buněčný/na histiocyty bohatý velkobuněčný B-lymfom | = en |
| uk | Велика В-клітинна лімфома з високим вмістом Т-клітин/гістіоцитів | = en |
| zh | T细胞组织细胞丰富型大B细胞淋巴瘤 | T细胞组织细胞丰富型大B细胞淋巴瘤 |
| tr | T hücresi / histiositten zengin büyük B hücreli lenfoma | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | inclusion | validated |
| ICD-11 | 2A81.4 | E | specific | validated |
| ICD-11 | XH0WP6 | E | specific | validated |
| MONDO | 0017597 | E | validated | |
| UMLS | C1321547 | E | validated | |
| GARD | 12257 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:544Diffuse large B-cell lymphoma
rare hematological diseases; rare neoplastic diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | <1 / 1 000 000 | 0.023 / 100 000 | United States | Value and class | Validated | 29845599[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/300857
- This record as JSON: /data/d/300857.json