Primary effusion lymphoma
diseaseOn this page
Also known as AIDS-related lymphomabody cavity-based lymphomaPEL
Summary
Primary effusion lymphoma (MONDO:0018842) is a cancer and 19 clinical trials. Top therapeutic interventions include procarbazine, filgrastim, and epoetin beta. A subtype of human herpesvirus 8-related tumor — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Prevalence: <1 / 1 000 000 (Worldwide) [Orphanet-validated]
- Phenotypes (HPO): 8
- Clinical trials: 19
Clinical features
Epidemiology
Prevalence records
2 prevalence record(s), Orphanet:
| Type | Class | Value | Geography | Validation |
|---|---|---|---|---|
| Cases/families | 200 | Worldwide | Validated | |
| Point prevalence | <1 / 1 000 000 | Worldwide | Validated |
Signs & symptoms
Clinical features (HPO)
8 HPO clinical features (Orphanet curated; top 8 by frequency):
| HPO ID | Term | Frequency |
|---|---|---|
| HP:0001698 | Pericardial effusion | Very frequent (80-99%) |
| HP:0002027 | Abdominal pain | Very frequent (80-99%) |
| HP:0002094 | Dyspnea | Very frequent (80-99%) |
| HP:0002202 | Pleural effusion | Very frequent (80-99%) |
| HP:0002585 | Abnormality of the peritoneum | Very frequent (80-99%) |
| HP:0003270 | Abdominal distention | Very frequent (80-99%) |
| HP:0012191 | B-cell lymphoma | Very frequent (80-99%) |
| HP:0002721 | Immunodeficiency | Frequent (30-79%) |
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | primary effusion lymphoma |
| Mondo ID | MONDO:0018842 |
| EFO | EFO:1000491 |
| MeSH | D054685 |
| Orphanet | 48686 |
| ICD-11 | 697911710 |
| NCIT | C6915 |
| SNOMED CT | 713516007 |
| UMLS | C1292753 |
| MedGen | 220953 |
| GARD | 0009247 |
| MedDRA | 10065857 |
| Is cancer (heuristic) | yes |
Also known as: AIDS-related lymphoma · body cavity-based lymphoma · PEL · primary Effusion Lymphoma
Data availability: 27 cell lines.
Disease family
This is a subtype of human herpesvirus 8-related tumor. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: disease › human disease › disease by etiologic mechanism › cancer or benign tumor › neoplastic disease or syndrome › neoplasm › virus associated tumor › human herpesvirus 8-related tumor › primary effusion lymphoma
Related subtypes (2): Kaposi’s sarcoma, multicentric Castleman disease
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 19.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| Not specified | 7 |
| PHASE2 | 6 |
| PHASE1/PHASE2 | 4 |
| PHASE1 | 2 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT02911142 | PHASE1/PHASE2 | ACTIVE_NOT_RECRUITING | Lenalidomide Combined With Modified DA-EPOCH and Rituximab (EPOCH-R2) in Primary Effusion Lymphoma or KSHV-associated Large Cell Lymphoma |
| NCT03038672 | PHASE2 | ACTIVE_NOT_RECRUITING | Nivolumab With or Without Varlilumab in Treating Patients With Relapsed or Refractory Aggressive B-cell Lymphomas |
| NCT00001563 | PHASE2 | COMPLETED | EPOCH Chemotherapy +/- IL-12 for Previously Untreated and EPOCH Plus Rituximab for Previously Treated Patients With AIDS-Associated Lymphoma |
| NCT00126243 | PHASE2 | TERMINATED | Efficacy of Rituximab and Cyclophosphamide, Doxorubicin, Vincristine, Prednisone (CHOP) in Patients With HIV Associated Non-Hodgkin’s Lymphoma |
| NCT00183976 | PHASE2 | TERMINATED | Pegylated Liposomal Doxorubicin (Doxil) With Rituximab in Relapsed AIDS-Related Non-Hodgkin’s Lymphomas |
| NCT00310128 | PHASE2 | WITHDRAWN | Combination Chemotherapy Followed by Rituximab and Yttrium Y 90 Ibritumomab Tiuxetan in Treating Patients With Relapsed or Refractory AIDS-Related Non-Hodgkin’s Lymphoma |
| NCT00858793 | PHASE1/PHASE2 | TERMINATED | High-dose Chemotherapy With Transplantation of Gene-modified Haematopoietic Stem Cells for HIV-positive Patients With Malignant Diseases Indicating an HSCT |
| NCT01193842 | PHASE1/PHASE2 | COMPLETED | Vorinostat and Combination Chemotherapy With Rituximab in Treating Patients With HIV-Related Diffuse Large B-Cell Non-Hodgkin Lymphoma or Other Aggressive B-Cell Lymphomas |
| NCT01775475 | PHASE2 | COMPLETED | Intravenous Chemotherapy or Oral Chemotherapy in Treating Patients With Previously Untreated Stage III-IV HIV-Associated Non-Hodgkin Lymphoma |
| NCT02228512 | PHASE1/PHASE2 | WITHDRAWN | Study of Pomalidomide Combined With Modified DA-EPOCH and Rituximab in KSHV-Associated Lymphomas |
| NCT03220022 | PHASE1 | ACTIVE_NOT_RECRUITING | Ibrutinib, Rituximab, Etoposide, Prednisone, Vincristine Sulfate, Cyclophosphamide, and Doxorubicin Hydrochloride in Treating Patients With HIV-Positive Stage II-IV Diffuse Large B-Cell Lymphomas |
| NCT05544019 | PHASE1 | RECRUITING | Study of SGR-1505 in Mature B-Cell Neoplasms |
| NCT00006518 | Not specified | RECRUITING | Specimen Collections From Participants With HIV Infection, KSHV Infection, Viral-Related Pre-malignant Lesions and Cancer |
| NCT04742387 | Not specified | ACTIVE_NOT_RECRUITING | Assessment of Bone Mineral Density in People Living With HIV and KSHV Associated Malignancies: A Retrospective Longitudinal Analysis |
| NCT05231135 | Not specified | RECRUITING | A Registry Study on HIV-related Lymphoma |
| NCT01769911 | Not specified | WITHDRAWN | Genetically Modified Peripheral Blood Stem Cell Transplant in Treating Patients With HIV-Associated Non-Hodgkin or Hodgkin Lymphoma |
| NCT02652715 | Not specified | COMPLETED | Salvia Hispanica Seed in Reducing Risk of Disease Recurrence in Patients With Non-Hodgkin Lymphoma |
| NCT03690895 | Not specified | COMPLETED | Long-term Outcome of AIDS-related Primary Central Nervous System Lymphoma Treated With High Dose Methotrexate and Combined Antiretroviral Therapy |
| NCT05510908 | Not specified | COMPLETED | Use of a Screening Tool to Describe HIV-Related Cancer Burden and Patient Characteristics in the AMC |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| PROCARBAZINE | 4 | 3 |
| FILGRASTIM | 4 | 2 |
| EPOETIN BETA | 4 | 1 |
| LOMUSTINE | 4 | 1 |
| POMALIDOMIDE | 4 | 1 |
| VORINOSTAT | 4 | 1 |
| YTTRIUM Y 90 IBRITUMOMAB TIUXETAN | 4 | 1 |
| 6-O-BENZYLGUANINE | 3 | 1 |
| VARLILUMAB | 2 | 1 |
| SGR-1505 | 1 | 1 |
| CHEMBL2093113 | 0 | 1 |