Intravascular large B-cell lymphoma
Synonyms: Angioendotheliomatosis proliferans systemisata · Angiotropic large cell lymphoma · Intravascular lymphomatosis · Malignant angioendotheliomatosis · Tappeiner-Pfleger disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Status
- active
Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Intravascular large B-cell lymphoma | Angioendotheliomatosis proliferans systemisata · Angiotropic large cell lymphoma · Intravascular lymphomatosis · Malignant angioendotheliomatosis · Tappeiner-Pfleger disease |
| fr | Lymphome B à grandes cellules intravasculaires | Angioendothéliomatose maligne · Lymphomatose intravasculaire · Lymphome angiotrope à grandes cellules · Maladie de Tappeiner-Pfleger |
| de | B-Zell-Lymphom, intravaskuläres großzelliges | Angioendotheliomatose, maligne · Angioendotheliomatosis proliferans systematisata · Angiotropes großzelliges B-Zell-Lymphom · Lymphomatose, intravaskuläre · Tappeiner-Pfleger-Krankheit |
| es | Linfoma B intravascular de células grandes | Angioendoteliomatosis maligna · Angioendoteliomatosis sistémica proliferante · Enfermedad de Tappeiner-Pfleger · Linfoma angiotrópico de células grandes · Linfomatosis intravascular |
| it | Linfoma intravascolare a grandi cellule B | Angioendoteliomatosi maligna · Angioendoteliomatosi proliferante sistemica · Linfoma angiotropico a grandi cellule · Linfomatosi intravascolare · Malattia di Tappeiner-Pfleger |
| nl | Intravasculair grootcellig B-cellymfoom | Angio-endotheliomatosis proliferans systemisata · Angiotroop grootcellig lymfoom · Intravasculaire lymfomatose · Kwaadaardige angio-endotheliomatose · Maligne angio-endotheliomatose · Ziekte van Tappeiner-Pfleger |
| pt | Linfoma, intravascular, de grandes células B | |
| pl | Chłoniak wewnątrznaczyniowy z dużych komórek B | Angiotropowy chłoniak wielkokomórkowy · Choroba Tappeinera i Pflegera · Limfomatoza wewnątrznaczyniowa · Złośliwa angioendoteliomatoza |
| cs | Intravaskulární velkobuněčný B-lymfom | Angioendotheliomatosis proliferans systemisata · Intravaskulární lymfomatóza · Maligní angioendoteliomatóza · Tappeinerova-Pflegerova nemoc · Angiotropní velkobuněčný lymfom · Tappeiner-Pflegerova nemoc |
| uk | Внутрішньосудинна велика В-клітинна лімфома | Ангіоендотеліоматоз проліферативний системний (Angioendotheliomatosis proliferans systemisata) · Ангіотропна великоклітинна лімфома · Внутрішньосудинний лімфоматоз · Злоякісний ангіоендотеліоматоз · Хвороба Таппейнера-Пфлегера |
| zh | 血管内大B细胞淋巴瘤 | 系统性增生性血管内皮瘤病 |
| tr | İntravasküler büyük B hücreli lenfoma | Tappeiner-Pfleger hastalığı · İntravasküler lenfomatoz · Anjiyotropik büyük hücreli lenfoma · Habis anjioendotelyomatoz · Anjiyoendotelyomatozis proliferans sistemisata |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | C83.3 | NTBT | attributed | validated |
| ICD-11 | 2A81.1 | E | specific | validated |
| MONDO | 0020324 | E | validated | |
| UMLS | C0334660 | E | validated | |
| MedDRA | 10069643 | 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
2 direct| Gene | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|
| BCL2 | Biomarker tested in | Assessed | HGNC:990 | ENSG00000171791 | 151430 | 18q21.33 | BCL2 apoptosis regulator | 19040469[PMID] |
| BCL6 | Disease-causing somatic mutation(s) in | Assessed | HGNC:1001 | ENSG00000113916 | 109565 | 3q27.3 | BCL6 transcription repressor |
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/98839
- This record as JSON: /data/d/98839.json