Orphanet Rare Disease Ontology

Last uploaded: July 3, 2024
Preferred Name

Plasmablastic lymphoma

Synonyms

PBL

Definitions

A rare aggressive B-cell non-Hodgkin lymphoma characterized by neoplastic cells resembling B immunoblasts or plasmablasts with a CD20-negative plasmacytic phenotype. The tumor may occur in the oral cavity, the gastrointestinal tract, or other, predominantly extranodal, sites and is typically associated with immunodeficiency or -suppression. The tumor cells are EBV-positive in most cases. Patients often present with disseminated bone involvement. Paraproteinemia may also be detected. Prognosis is generally poor.

ID

http://www.orpha.net/ORDO/Orphanet_289666

alternative_term

PBL

definition

A rare aggressive B-cell non-Hodgkin lymphoma characterized by neoplastic cells resembling B immunoblasts or plasmablasts with a CD20-negative plasmacytic phenotype. The tumor may occur in the oral cavity, the gastrointestinal tract, or other, predominantly extranodal, sites and is typically associated with immunodeficiency or -suppression. The tumor cells are EBV-positive in most cases. Patients often present with disseminated bone involvement. Paraproteinemia may also be detected. Prognosis is generally poor.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=en&Expert=289666

hasDbXref

ICD-10:C83.3

MedDRA:10065039

ICD-11:2A81.2

UMLS:C3472614

MeSH:D000069293

label

Plasmablastic lymphoma

notation

ORPHA:289666

part_of

http://www.orpha.net/ORDO/Orphanet_300846

http://www.orpha.net/ORDO/Orphanet_289644

prefixIRI

ORDO:Orphanet_289666

prefLabel

Plasmablastic lymphoma

treeView

http://www.orpha.net/ORDO/Orphanet_300846

http://www.orpha.net/ORDO/Orphanet_289644

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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