Chronic active Epstein-Barr virus disease: Difference between revisions

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== Background ==
==Background==


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


=== Pathophysiology ===
===Pathophysiology===


* EBV infection involving B, T, or NK cells
*EBV infection involving B, T, and/or NK cells


=== Epidemiology ===
===Epidemiology===


* Most cases reported in people of Asian and North American Indigenous descent
*Most cases reported in Japan and East Asia
* Most common in Japan
*In the Americas, more common in Indigenous populations
*However, can occur in people of all ethnicities


== Clinical Manifestations ==
==Clinical Manifestations==


*Symptoms include fever, liver dysfunction, [[splenomegaly]], [[lymphadenopathy]], and [[thrombocytopenia]]
*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]]
*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]]
*Occasionally has [[uveitis]], CNS disease, intestinal perforation, and [[myocarditis]]
*Can progress to frank [[lymphoma]] or [[hemophagocytic lymphohistiocytosis]]


== Management ==
=== Related Disorders ===


==== Severe mosquito bite allergy ====
⚫
* [[Hematopoietic stem cell transplantation]] is the only curative treatment

⚫
* Symptoms may be temporarily improved with [[corticosteroids]]
* 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

==== Hydroa vacciniforme ====

* Characterized by light-induced vesicles
* Can also involve systemic inflammation

== Diagnostic Criteria ==

* Sustained or recurrent [[Infectious mononucleosis|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<sup>2.5</sup> 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)

== Diagnosis ==

* 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<sup>2.5</sup> copies/μg DNA
** Detection of EBV infection of T or NK cells in affected tissues or peripheral blood

==Management==

⚫
*[[Hematopoietic stem cell transplantation]] is the only curative treatment
⚫
*Symptoms may be temporarily improved with [[corticosteroids]]

== Further Reading ==

* Advances in the Study of Chronic Active Epstein-Barr Virus Infection: Clinical Features Under the 2016 WHO Classification and Mechanisms of Development. ''Front Pediatr''. 2019;7:14. doi: [https://doi.org/10.3389/fped.2019.00014 10.3389/fped.2019.00014]


[[Category:Hematology]]
[[Category:Hematology]]

Revision as of 01:34, 16 October 2020

Background

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

Pathophysiology

  • EBV infection involving B, T, and/or NK cells

Epidemiology

  • Most cases reported in Japan and East Asia
  • In the Americas, more common in Indigenous populations
  • However, can occur in people of all ethnicities

Clinical Manifestations

Related Disorders

Severe mosquito bite allergy

  • 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

Hydroa vacciniforme

  • Characterized by light-induced vesicles
  • Can also involve systemic inflammation

Diagnostic Criteria

  • 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 (>102.5 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)

Diagnosis

  • 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 ≥102.5 copies/μg DNA
    • Detection of EBV infection of T or NK cells in affected tissues or peripheral blood

Management

Further Reading

  • Advances in the Study of Chronic Active Epstein-Barr Virus Infection: Clinical Features Under the 2016 WHO Classification and Mechanisms of Development. Front Pediatr. 2019;7:14. doi: 10.3389/fped.2019.00014

References

  1. ^  Hiroshi Kimura, Yo Hoshino, Hirokazu Kanegane, Ikuya Tsuge, Takayuki Okamura, Keisei Kawa, Tsuneo Morishima. Clinical and virologic characteristics of chronic active Epstein-Barr virus infection. Blood. 2001;98(2):280-286. doi:10.1182/blood.v98.2.280.
  2. ^  Jeffrey I. Cohen, Elaine S. Jaffe, Janet K. Dale, Stefania Pittaluga, Helen E. Heslop, Cliona M. Rooney, Stephen Gottschalk, Catherine M. Bollard, V. Koneti Rao, Adriana Marques, Peter D. Burbelo, Siu-Ping Turk, Rachael Fulton, Alan S. Wayne, Richard F. Little, Mitchell S. Cairo, Nader K. El-Mallawany, Daniel Fowler, Claude Sportes, Michael R. Bishop, Wyndham Wilson, Stephen E. Straus. Characterization and treatment of chronic active Epstein-Barr virus disease: a 28-year experience in the United States. Blood. 2011;117(22):5835-5849. doi:10.1182/blood-2010-11-316745.
  3. ^  Motohiko Okano, Keisei Kawa, Hiroshi Kimura, Akihiro Yachie, Hiroshi Wakiguchi, Akihiko Maeda, Shosuke Imai, Shouichi Ohga, Hirokazu Kanegane, Shigeru Tsuchiya, Tomohiro Morio, Masaaki Mori, Shumpei Yokota, Shinsaku Imashuku. Proposed guidelines for diagnosing chronic active Epstein-Barr virus infection. American Journal of Hematology. 2005;80(1):64-69. doi:10.1002/ajh.20398.

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