Chronic active Epstein-Barr virus disease - Revision history

Aidan at 23:20, 18 February 2022

2022-02-18T23:20:45Z

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 *Requires all of the criteria
 *Persistent or recurrent [[Infectious mononucleosis|IM‐like]] symptoms
 * Unusual pattern of anti-EBV antibodies with raised anti-VCA and anti-EA, and/or detection of increased EBV genomes in affected tissues, including the peripheral blood
-** EBV infection of T or NK cells in the affected tissues or peripheral blood
+***PCR, which typically shows >10<sup>2.5</sup> copies/µg DNA in the peripheral blood
 * Chronic illness which cannot be explained by other known disease processes at diagnosis, including:
 ** Primary EBV infection ([[infectious mononucleosis]])

Aidan at 23:11, 18 February 2022

2022-02-18T23:11:50Z

← Older revision		Revision as of 23:11, 18 February 2022
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	== Diagnostic Criteria ==		== Diagnostic Criteria ==

			* Criteria were proposed in 2005 based on Japanese cases[[CiteRef::okano2005pr]]
⚫	* ~~Sustained~~ or recurrent [[Infectious mononucleosis\|IM‐like]] symptoms ~~for greater than 3 months~~
			*Requires all of the criteria
		⚫	*Persistent or recurrent [[Infectious mononucleosis\|IM‐like]] symptoms
			**For greater than 3 months
	** Symptoms include fever, lymphadenopathy, and hepatosplenomegaly, and possibly other symptoms		** Symptoms include fever, lymphadenopathy, and hepatosplenomegaly, and possibly other symptoms
			* Unusual pattern of anti-EBV antibodies with raised anti-VCA and anti-EA, and/or detection of increased EBV genomes in affected tissues, including the peripheral blood
	* Elevated EBV genome load in the peripheral blood (>10<sup>2.5</sup> copies/µg DNA)		**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		** EBV infection of T or NK cells in the affected tissues or peripheral blood
	* Exclusion of other possible diagnoses including the following:
			* Chronic illness which cannot be explained by other known disease processes at diagnosis, including:
	** Primary EBV infection (infectious mononucleosis)		** Primary EBV infection ([[infectious mononucleosis]])
	** [[Primary immunodeficiencies]]		** [[Primary immunodeficiencies]]
	** [[HIV]]		** [[HIV]]
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	** Autoimmune or collagen vascular diseases		** 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)		** Other malignant [[lymphoma]] (classic [[Hodgkin lymphoma]], extranodal NK/T cell lymphoma, including nasal type, peripheral T cell lymphomas, and aggressive NK‐cell leukemia)
			**Note, however, that an EBV-associated disease such as [[HLH]] or [[lymphoma]]/[[LPD]] often develops in the course of illness

	== Diagnosis ==		== Diagnosis ==

Aidan at 16:19, 18 February 2022

2022-02-18T16:19:11Z

← Older revision		Revision as of 16:19, 18 February 2022
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	==Background==		==Background==

	*Life-threatening ~~inflammatory~~ ~~disorder~~ ~~and lymphoid neoplasm~~ caused by infection with [[Epstein-Barr virus]] involving NK and T cells		*Life-threatening EBV-associated lymphoproliferative disorder caused by infection with [[Epstein-Barr virus]] involving primarily NK and T cells
			*Classified as: polymorphic polyclonal/oligoclonal LPD, polymorphic monoclonal LPD, and monomorphic monoclonal LPD (which is similar to [[PTLD]])
			**Related disorders of exclusion include aggressive NK-cell leukemia (ANKL) and extranodal NK/T-cell lymphoma (ENKTL), though there may be some overlap

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

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

	===Epidemiology===		===Epidemiology===
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	==Clinical Manifestations==		==Clinical Manifestations==

	*~~Symptoms~~ include fever, ~~liver dysfunction~~, [[splenomegaly]], [[lymphadenopathy]], and [[thrombocytopenia]]		*The most common symptoms include fever, [[hepatitis]], [[splenomegaly]], [[lymphadenopathy]], and [[thrombocytopenia]][[CiteRef::kimura2001cl]][[CiteRef::cohen2011ch]]
			**[[Hypogammaglobulinemia]] and [[pancytopenia]] often seen
	*~~May~~ ~~also~~ have [[hepatomegaly]], [[anemia]], mosquito bite hypersensitivity, rashes, oral ulcers~~, [[hemophagocytic lymphohistiocytosis]]~~, coronary artery aneurysm, liver failure, [[lymphoma]], and [[interstitial ~~pneumonia~~]]		*Also commonly have [[hepatomegaly]], [[anemia]], mosquito bite hypersensitivity (see below), rashes (such as hydroa vacciniforme), oral ulcers, coronary artery aneurysm, liver failure, [[lymphoma]], [[hemophagocytosis]], and [[interstitial pneumonitis]]
	*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]]		*Can progress to frank [[lymphoma]] or [[hemophagocytic lymphohistiocytosis]]
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	=== Related Disorders ===		=== Related Disorders ===

	==== Severe Mosquito Bite ~~Sllergy~~ ====		==== Severe Mosquito Bite Allergy ====

	* A severe hypersensitivity reaction to saliva in the bite of [[Aedes albopictus]] mosquitoes		* A severe hypersensitivity reaction to saliva in the bite of [[Aedes albopictus]] mosquitoes
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	* Characterized by light-induced vesicles		* Characterized by light-induced vesicles
	* Can also involve systemic inflammation		* Can also involve systemic inflammation

			== Differential Diagnosis ==

			* [[Kawasaki disease]]
			* [[Behçet disease]]

	== Diagnostic Criteria ==		== Diagnostic Criteria ==
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	** Anti-EBV antibodies demonstrating anti-VCA-IgG (necessary for diagnosis), anti-EA-IgG, and anti-VCA-IgA or anti-EA-IgA antibodies		** 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		*** Anti-EBNA antibodies may be negative
	** EBV DNA viral load ≥10<sup>2.5</sup> copies/μg DNA		** EBV DNA viral load ≥10<sup>2.5</sup> copies/μg DNA (i.e. 2.5 log) in peripheral blood mononuclear cells
	** Detection of EBV infection of T or NK cells in affected tissues or peripheral blood		** Detection of EBV infection of T or NK cells in affected tissues or peripheral blood
			*The diagnosis requires a combination of identifying EBV-infected T/NK cells in PB or affected tissues/organs and having compatible symptomatology

	==Management==		==Management==

Aidan at 15:42, 18 February 2022

2022-02-18T15:42:08Z

← Older revision		Revision as of 15:42, 18 February 2022
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	=== Related Disorders ===		=== Related Disorders ===

	==== Severe ~~mosquito~~ ~~bite~~ ~~allergy~~ ====		==== Severe Mosquito Bite Sllergy ====

	* A severe hypersensitivity reaction to saliva in the bite of [[Aedes albopictus]] mosquitoes		* A severe hypersensitivity reaction to saliva in the bite of [[Aedes albopictus]] mosquitoes
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	* Resoves within a month		* Resoves within a month

	==== Hydroa ~~vacciniforme~~ ====		==== Hydroa Vacciniforme ====

	* Characterized by light-induced vesicles		* Characterized by light-induced vesicles

Aidan at 01:34, 16 October 2020

2020-10-16T01:34:00Z

← Older revision		Revision as of 01:34, 16 October 2020
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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]]

Aidan: Created page with "== Background == * Life-threatening infection with Epstein-Barr virus involving NK and T cells === Pathophysiology === * EBV infection involving B, T, or NK cells ===..."

2020-10-16T00:43:51Z

Created page with "== Background == * Life-threatening infection with Epstein-Barr virus involving NK and T cells === Pathophysiology === * EBV infection involving B, T, or NK cells ===..."

New page

== Background ==

* Life-threatening infection with [[Epstein-Barr virus]] involving NK and T cells

=== Pathophysiology ===

* EBV infection involving B, T, or NK cells

=== Epidemiology ===

* Most cases reported in people of Asian and North American Indigenous descent
* Most common in Japan

== Clinical Manifestations ==

*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]]

== Management ==

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

[[Category:Hematology]]
[[Category:Infectious diseases]]