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Internal Medicine

Chronic active EBV (CAEBV)

Severe progressive infection characterized by duration of > 6 months, persistent elevated EBV titers, and evidence of EBV-related organ damage.

  • Characteristics:
    1. Severe progressive illness lasting > 6 months
    2. Lymphocytic infiltration of tissue (e.g., lymph nodes, lungs, liver, central nervous tissue, bone marrow, eye, and skin)
    3. ↑ EBV DNA & RNA in affected tissue
    4. Absence of any other immunosuppressive conditions

Classification

Chronic active EBV (CAEBV) type B cell:

CAEBV-type T/NK cell disease:

  • Category A1: Polymorphic LPD with polyclonal proliferation of EBV-infected T/NK cells
  • Category A2: Polymorphic LPD with monoclonal T/NK cells
  • Category A3: Monomorphic LPD of monoclonal T cells

Clinical features

  • Prolonged fever
  • Hepatosplenomegaly
  • Thrombocytopenia
  • Anaemia
  • Lymphadenopathy

Complications

Life-threatening complications:

  • Hemophagocytic syndrome
  • Interstitial pneumonia
  • Malignant lymphoma
  • Coronary aneurysms
  • CNS involvement

Diagnosis

Histopathology

jptm-2017-03-15f2
Chronic active Epstein-Barr virus (EBV) infection of a T-cell or natural killer cell type, systemic (Courtesy of Dr. Y.H. Ko). (A) A 21-yearold man presented with severe oral ulcer, recurrent pneumonia, thrombocytopenia, and elevated liver enzymes for 2 years. Liver biopsy reveals atypical T lymphocytes infiltrating the sinusoidal and hepatic lobules. (B) EBV-encoded small RNA (EBER) in-situ hybridization exhibits positive signals in these T cells. (C) Bone marrow biopsy shows small lymphocytic infiltrate. (D) CD3 is expressed in most lymphocytes. (E) EBER in-situ hybridization also shows positive signals in T cells. | Chung, J. S., Shin, H. J., Lee, E. Y., & Cho, G. J. (2003). Hypersensitivity to mosquito bites associated with natural killer cell-derived large granular lymphocyte lymphocytosis: a case report in Korea. The Korean Journal of Internal Medicine, 18(1), 50–52. https://doi.org/10.3904/kjim.2003.18.1.50

Lymph nodes:

  • Paracortical hyperplasia with polymorphic and polyclonal lymphoid proliferation and large numbers of EBER positive cells

Liver:

  • Portal/sinusoidal infiltration by small lymphocytes with no definite atypia

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