Overview
Primary effusion lymphoma (PEL), sometimes called body cavity lymphoma, is a rare and aggressive type of blood cancer (lymphoma) that affects a type of white blood cell called a B-cell. Unlike most lymphomas that form solid tumors in lymph nodes, PEL is unusual because the cancer cells grow mainly in the fluid-filled spaces of the body — most often around the lungs (pleural cavity), around the heart (pericardial cavity), or in the abdomen (peritoneal cavity). This buildup of fluid is called an effusion, which is how the disease gets its name. PEL is almost always linked to infection with a virus called Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV-8). Most people who develop PEL also have a weakened immune system, especially those living with HIV/AIDS. In some cases, it can occur in people who have had organ transplants or elderly individuals without HIV, particularly in regions where HHV-8 is common. The main symptoms are caused by fluid buildup in body cavities, leading to shortness of breath, chest pain, and abdominal swelling. PEL is a very aggressive cancer that tends to progress quickly. Treatment usually involves chemotherapy, and in people with HIV, antiretroviral therapy (ART) is also a key part of care. Unfortunately, outcomes remain poor even with treatment, and researchers are actively looking for better therapies.
Also known as:
Key symptoms:
Shortness of breath or difficulty breathingChest pain or tightnessSwollen or bloated abdomenUnexplained weight lossPersistent fatigue and weaknessFever without a clear causeNight sweatsReduced appetiteFeeling of fullness in the bellyRapid heartbeat (from fluid around the heart)
Clinical phenotype terms (8)— hover any for plain English
Sporadic
Usually appears on its own, not inherited from a parent
Adult
Begins in adulthood (age 18 or older)
FDA & Trial Timeline
2 eventsFederal University of Pelotas — NA
National Cancer Institute (NCI) — PHASE1, PHASE2
Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.
Treatments
No FDA-approved treatments are currently listed for Primary effusion lymphoma.
1 clinical trialare actively recruiting — trials can provide access to cutting-edge therapies.
View clinical trials →Rare Disease Specialist
Rare Disease Specialist
Treatment Centers
8 centersBaylor College of Medicine Rare Disease Center ↗
Baylor College of Medicine
📍 Houston, TX
🏥 NORDStanford Medicine Rare Disease Center ↗
Stanford Medicine
📍 Stanford, CA
🔬 UDNNIH Clinical Center Undiagnosed Diseases Program ↗
National Institutes of Health
📍 Bethesda, MD
🔬 UDNUCLA UDN Clinical Site ↗
UCLA Health
📍 Los Angeles, CA
🔬 UDNBaylor College of Medicine UDN Clinical Site ↗
Baylor College of Medicine
📍 Houston, TX
🔬 UDNHarvard/MGH UDN Clinical Site ↗
Massachusetts General Hospital
📍 Boston, MA
🏥 NORDMayo Clinic Center for Individualized Medicine ↗
Mayo Clinic
📍 Rochester, MN
👤 Mayo Clinic Center for Individualized Medicine
🏥 NORDUCLA Rare Disease Day Program ↗
UCLA Health
📍 Los Angeles, CA
Travel Grants
No travel grants are currently matched to Primary effusion lymphoma.
Community
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Start the conversation →Latest news about Primary effusion lymphoma
Disease timeline:
New recruiting trial: CARDEC-PEL: Comparison of Two Methods of Caries Risk Assessment in Children
A new clinical trial is recruiting patients for Primary effusion lymphoma
Caregiver Resources
NORD Caregiver Resources
Support, advocacy, and financial assistance for caregivers of rare disease patients.
Mental Health Support
Rare disease caregiving can be isolating. Connect with counseling and peer support.
Family & Caregiver Grants
Financial assistance programs specifically for caregivers of rare disease patients.
Social Security Disability
Learn how rare disease patients may qualify for SSDI/SSI benefits.
Questions for your doctor
Bring these to your next appointment
- Q1.What stage is my PEL and has it spread beyond the fluid cavities?,Which chemotherapy regimen do you recommend for me, and what are the side effects?,Should I be tested for HIV and HHV-8 if I haven't been already?,Are there any clinical trials I might be eligible for?,How often will I need fluid drained, and can this be managed at home?,What does my prognosis look like, and how will we know if treatment is working?,What palliative care or support services are available to help me and my family?
Common questions about Primary effusion lymphoma
What is Primary effusion lymphoma?
Primary effusion lymphoma (PEL), sometimes called body cavity lymphoma, is a rare and aggressive type of blood cancer (lymphoma) that affects a type of white blood cell called a B-cell. Unlike most lymphomas that form solid tumors in lymph nodes, PEL is unusual because the cancer cells grow mainly in the fluid-filled spaces of the body — most often around the lungs (pleural cavity), around the heart (pericardial cavity), or in the abdomen (peritoneal cavity). This buildup of fluid is called an effusion, which is how the disease gets its name. PEL is almost always linked to infection with a vi
How is Primary effusion lymphoma inherited?
Primary effusion lymphoma follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Primary effusion lymphoma typically begin?
Typical onset of Primary effusion lymphoma is adult. Age of onset can vary across affected individuals.
Are there clinical trials for Primary effusion lymphoma?
Yes — 1 recruiting clinical trial is currently listed for Primary effusion lymphoma on UniteRare. See the clinical trials section on this page for phase, sponsor, and site details sourced from ClinicalTrials.gov.
Which specialists treat Primary effusion lymphoma?
19 specialists and care centers treating Primary effusion lymphoma are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.