Overview
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is a very rare and serious condition in which the Epstein-Barr virus infects certain immune cells called T cells (or sometimes natural killer cells). Normally, EBV causes mild illness like mononucleosis ("mono"), but in this disease, the virus triggers an uncontrolled and dangerous multiplication of infected T cells throughout the body. This is not a typical infection — it behaves more like an aggressive cancer of the immune system. The disease usually affects children and young adults who were previously healthy. Symptoms often begin with a high fever that does not go away, a swollen liver and spleen, swollen lymph nodes, and abnormal blood counts. The condition can rapidly progress to life-threatening complications including liver failure, multi-organ failure, and a dangerous inflammatory condition called hemophagocytic lymphohistiocytosis (HLH), where the immune system attacks the body's own blood cells. Unfortunately, this disease is very aggressive and often does not respond well to standard chemotherapy. The most effective treatment currently available is an allogeneic hematopoietic stem cell transplant (bone marrow transplant from a donor), which is the only potentially curative option. Without transplant, the prognosis is generally poor. Supportive care and chemotherapy may help control symptoms temporarily, but early referral to a specialized center is critical.
Also known as:
Key symptoms:
High fever that does not go awayEnlarged liver (hepatomegaly)Enlarged spleen (splenomegaly)Swollen lymph nodesSevere fatigue and weaknessYellowing of the skin or eyes (jaundice)Skin rashLow blood cell counts (anemia, low platelets, low white blood cells)Liver failureAbnormal bleeding or bruisingWeight lossNight sweatsSwelling of the body due to fluid retentionMulti-organ failure
Sporadic
Usually appears on its own, not inherited from a parent
Childhood
Begins in childhood, roughly ages 1 to 12
Treatments
No FDA-approved treatments are currently listed for Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood.
View clinical trials →Clinical Trials
View all trials with filters →No actively recruiting trials found for Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood at this time.
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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 Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood.
Community
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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.How advanced is my child's disease, and has it spread to multiple organs?,Is my child a candidate for a stem cell transplant, and how quickly can we begin the process?,Does my child show signs of hemophagocytic lymphohistiocytosis (HLH), and how will it be treated?,Should we test for an underlying immune deficiency that may have caused this?,Are there any clinical trials or experimental treatments available for this condition?,What supportive care will my child need, and how long might hospitalization last?,What is the realistic prognosis for my child given their current condition?
Common questions about Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood
What is Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood?
Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is a very rare and serious condition in which the Epstein-Barr virus infects certain immune cells called T cells (or sometimes natural killer cells). Normally, EBV causes mild illness like mononucleosis ("mono"), but in this disease, the virus triggers an uncontrolled and dangerous multiplication of infected T cells throughout the body. This is not a typical infection — it behaves more like an aggressive cancer of the immune system. The disease usually affects children and young adults who were previous
How is Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood inherited?
Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood follows a sporadic inheritance pattern. Genetic counseling can help families understand recurrence risk and testing options.
At what age does Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood typically begin?
Typical onset of Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood is childhood. Age of onset can vary across affected individuals.
Which specialists treat Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood?
11 specialists and care centers treating Systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood are listed on UniteRare, sourced from ClinicalTrials.gov principal investigators, published research, and the NPPES NPI registry.