Chronic active Epstein-Barr virus disease

Background

Life-threatening inflammatory disorder and lymphoid neoplasm caused by infection with Epstein-Barr virus involving NK and T cells

Symptoms include fever, liver dysfunction, splenomegaly, lymphadenopathy, and thrombocytopenia
May also have hepatomegaly, anemia, mosquito bite hypersensitivity, rashes, oral ulcers, hemophagocytic lymphohistiocytosis, coronary artery aneurysm, liver failure, lymphoma, and interstitial pneumonia
Occasionally has uveitis, CNS disease, intestinal perforation, and myocarditis
Can progress to frank lymphoma or hemophagocytic lymphohistiocytosis

A severe hypersensitivity reaction to saliva in the bite of Aedes albopictus mosquitoes
Characterized by local skin inflammation followed by high fever, lymphadenopathy, and liver dysfunction
The bite can ulcerate and scar
Resoves within a month

Sustained or recurrent IM‐like symptoms for greater than 3 months
- Symptoms include fever, lymphadenopathy, and hepatosplenomegaly, and possibly other symptoms
Elevated EBV genome load in the peripheral blood (>10^2.5 copies/µg DNA)
EBV infection of T or NK cells in the affected tissues or peripheral blood
Exclusion of other possible diagnoses including the following:
- Primary EBV infection (infectious mononucleosis)
- Primary immunodeficiencies
- HIV
- Iatrogenic immunosuppression
- Autoimmune or collagen vascular diseases
- Other malignant lymphoma (classic Hodgkin lymphoma, extranodal NK/T cell lymphoma, including nasal type, peripheral T cell lymphomas, and aggressive NK‐cell leukemia)

Can follow a series of stepwise diagnostic tests:
- Anti-EBV antibodies demonstrating anti-VCA-IgG (necessary for diagnosis), anti-EA-IgG, and anti-VCA-IgA or anti-EA-IgA antibodies
  - Anti-EBNA antibodies may be negative
- EBV DNA viral load ≥10^2.5 copies/μg DNA
- Detection of EBV infection of T or NK cells in affected tissues or peripheral blood