Systemic Epstein Barr Virus Positive T Cell Lymphoproliferative Disease Of Childhood C2699747
A rare disorder in which Epstein-Barr virus infects T blood cells, causing abnormal cell growth. It primarily affects children and progresses rapidly. Treatment with chemotherapy may be attempted, though outcomes are often poor.
Key facts at a glance
- ICD-10
- C96.9
- ICD-11
- 2A00.0
- Body system
- hematologic
- Severity
- fatal
- Chronic
- false
Signs and symptoms
Common signs and symptoms that may indicate this condition.
Warning signs - seek immediate care
Generalized symptoms
Progressive symptoms that can become life-threatening without treatment.
Seek immediate medical attention if symptoms worsen.
Persistent high fever
Generalized lymphadenopathy
(hematologic)Hepatosplenomegaly
(hematologic)Skin nodules or rash
(hematologic)Diagnosis and tests
How this condition is diagnosed and what test results mean.
Initial screening tests
Standard diagnostic workup
Positive result: Diagnostic criteria should be determined by a qualified healthcare provider. [Content pending medical review.]
Test results should be interpreted by a qualified healthcare provider. [Content pending medical review.]
Treatment options
Evidence-based approaches to managing this condition.
First-line treatments
Outpatient procedure with monitoring
Treatment may require specialized care, hospitalization, or long-term management under medical supervision.
Chemotherapy regimen
Intensive multi-agent chemotherapy protocols are typically employed as first-line treatment to achieve disease remission in this aggressive lymphoproliferative condition. Treatment response varies significantly among pediatric patients.
Allogeneic hematopoietic stem cell transplantation
Curative-intent procedure involving high-dose chemotherapy followed by stem cell infusion from a compatible donor. This approach offers the best chance for long-term disease control.
Additional treatment options
Intensive disease monitoring
Regular surveillance including imaging studies, laboratory markers, and clinical assessments to detect disease progression or relapse early and guide treatment modifications.
monitoringClinical trial enrollment
Consideration of experimental targeted therapies available through clinical trials may provide access to novel treatment approaches not yet widely available.
complementaryNutritional support
Enteral or parenteral nutrition support to maintain adequate caloric intake during intensive treatment phases when oral intake is compromised.
dietaryWhen to seek medical help
Recognize when you need immediate medical attention.
Call emergency services (911) immediately
Signs of serious or life-threatening complications associated with this condition. Immediate medical evaluation is required.
Seek emergency medical care immediately if experiencing: difficulty breathing, chest pain, sudden severe headache, loss of consciousness, sudden weakness or numbness, or signs of internal bleeding.
Frequently asked questions
Common questions about this topic.
What is Systemic Epstein Barr Virus Positive T Cell Lymphoproliferative Disease Of Childhood C2699747?
Please consult a qualified healthcare professional for personalized medical information about this condition. [Content pending medical review.]
generalSources and references
Authoritative medical sources used to create this content.
Cohen JI, Kimura H, Nakamura S, Ko YH, Jaffe ES. Epstein-Barr virus-associated lymphoproliferative disease in non-immunocompromised hosts. Blood. 2019.
Kimura H, Ito Y, Kawabe S, et al. EBV-associated T/NK-cell lymphoproliferative diseases in non-immunocompromised hosts. J Clin Immunol. 2018.
Eladl MA, Sultan AS, Assaf R, et al. WHO classification of tumors of hematopoietic and lymphoid tissues, revised 4th edition. In: Swerdlow SH, Campo E, Harris NL, et al., editors. Lyon: IARC Press; 2017.
Luo X, Kikuchi Y, Kimura S, et al. Familial cases of systemic EBV-positive T-cell lymphoproliferative disease of childhood. Haematologica. 2020.
Medication Comparisons
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This content is for general informational and educational purposes only and is not a substitute for professional medical advice, diagnosis, or treatment. Always seek the guidance of a qualified healthcare provider with any questions you have about a medical condition, and never disregard professional medical advice or delay seeking it because of something you read here.
If you think you may have a medical emergency, call your local emergency number or go to the nearest emergency department immediately.
Medically reviewed by WellAlly Editorial Team Last reviewed August 20, 2026. · Read our editorial policy