Primary Effusion Lymphoma

Preferred Label : Primary Effusion Lymphoma;

NCIt synonyms : PEL;

NCIt definition : A large B-cell lymphoma usually presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV8), also called Kaposi sarcoma-associated herpesvirus. It mostly occurs in the setting of immunodeficiency. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. Rare HHV8-positive lymphomas indistinguishable from primary effusion lymphomas (PEL) present as solid tumor masses, and have been termed extracavitary PEL. The prognosis is extremely unfavorable. (WHO 2017);

Alternative definition : NCI-GLOSS: A rare, aggressive (fast-growing) type of B-cell non-Hodgkin lymphoma marked by an abnormal build-up of fluids in a body cavity. It usually occurs together with a human herpesvirus in people who have weakened immune systems, such as in AIDS.;

Neoplastic status : Malignant;

ICD-O code : 9678/3;

Codes from synonyms : 10065857; CDR0000455144;

Details

Origin ID

C6915;

UMLS CUI

C1292753;

Automatic exact mappings (from CISMeF team)
- Primary effusion lymphoma [ICD-11 More detail]
Currated CISMeF NLP mapping
- PEL (body structure) [SNOMED CT concept]
- Primary effusion lymphoma [ICD-O code]
- Primary effusion lymphoma [PASCAL descriptor]
- Primary effusion lymphoma [ICD-11 Subcategory]
- Primary effusion lymphoma [MedDRA Preferred Term]
- Primary effusion lymphoma [ORDO Disease]
- Primary effusion lymphoma (disorder) [SNOMED CT concept]
- Primary effusion lymphoma (morphologic abnormality) [SNOMED CT concept]
- lymphoma, primary effusion [MeSH Descriptor]
- lymphoma, primary effusion [MeSH concept]
- primary effusion lymphoma [Disease (Nov.)]
Disease excludes abnormal cell
- Neoplastic T-Lymphocyte and Neoplastic Natural Killer Cell [NCIt concept]
- Reed-Sternberg Cell [NCIt concept]
Disease excludes normal cell origin
- Myeloid Cell [NCIt concept]
- Plasma Cell [NCIt concept]
- Precursor B-Lymphoblast [NCIt concept]
Disease may have findings
- Aggressive Clinical Course [NCIt concept]
Has associated anatomic sites
- Hematopoietic and Lymphatic System [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Primary effusion lymphoma [ORDO Disease]
- Primary effusion lymphoma [ICD-O code]
- Primary effusion lymphoma [MedDRA Preferred Term]
- Primary effusion lymphoma (disorder) [SNOMED CT concept]
- Primary effusion lymphoma (morphologic abnormality) [SNOMED CT concept]
- lymphoma, primary effusion [MeSH concept]
- lymphoma, primary effusion [MeSH Descriptor]
- primary effusion lymphoma [Disease (Nov.)]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
- Cellosaurus Disease Terminology [NCIt concept]
- Cellosaurus Terminology [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology Terminology [NCIt concept]
- Mapped ICDO3.1 Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCI CTEP SDC Non-Hodgkin Lymphoma Sub-Category Terminology [NCIt concept]
- NCI CTEP Simplified Disease Classification Terminology [NCIt concept]
- NCIt Neoplasm Core Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
disease_has_abnormal_cell
- Abnormal Lymphocyte [NCIt concept]
- Malignant Cell [NCIt concept]
- Neoplastic B-Lymphocyte [NCIt concept]
- Neoplastic Cell [NCIt concept]
- Neoplastic Hematopoietic and Lymphoid Cell [NCIt concept]
- Neoplastic Large B-Lymphocyte [NCIt concept]
- Neoplastic Lymphocyte [NCIt concept]
disease_has_associated_disease
- HHV8 Infection [NCIt concept]
- Infectious Disorder [NCIt concept]
- Viral Infection [NCIt concept]
disease_has_finding
- Diffuse Pattern [NCIt concept]
- Effusion [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Primary Lesion [NCIt concept]
disease_has_molecular_abnormality
- Clonal Immunoglobulin Gene Rearrangement [NCIt concept]
- Clonal Immunoglobulin Kappa Light Chain Gene Rearrangement [NCIt concept]
disease_has_normal_cell_origin
- B-Lymphocyte [NCIt concept]
- Hematopoietic and Lymphoid Cell [NCIt concept]
- Lymphocyte [NCIt concept]
- Mature B-Lymphocyte [NCIt concept]
- Postgerminal Center B-Lymphocyte [NCIt concept]
disease_has_normal_tissue_origin
- Hematopoietic and Lymphoid Tissue [NCIt concept]
- Lymphoid Tissue [NCIt concept]
disease_has_primary_anatomic_site
- Hematopoietic and Lymphatic System [NCIt concept]
- Lymphatic System [NCIt concept]
disease_may_have_abnormal_cell
- Anaplastic Large B-Lymphocyte [NCIt concept]
- Neoplastic B-Immunoblast [NCIt concept]
- Plasmablastic Immunoblast [NCIt concept]
disease_may_have_associated_disease
- Kaposi Sarcoma [NCIt concept]
disease_may_have_cytogenetic_abnormality
- Gain of Chromosome 12 [NCIt concept]
- Gain of Chromosome X [NCIt concept]
- Trisomy 12 [NCIt concept]
- Trisomy 7 [NCIt concept]
- Trisomy 8 [NCIt concept]
disease_may_have_molecular_abnormality
- Clonal Immunoglobulin Heavy Chain Gene Rearrangement [NCIt concept]
- Clonal Immunoglobulin Lambda Light Chain Gene Rearrangement [NCIt concept]
- Clonal T-Cell Receptor Gene Rearrangement [NCIt concept]

Keyword's position in hierarchy(ies) :

You can consult :

recommendations
documents concerning education
documents for patients