Chronic active Epstein-Barr virus infection of natural killer cells presenting as severe skin reaction to mosquito bites

Pacheco, Susan E.; Gresik, Mary V.; Dishop, Megan K.; Okmaura, Takayuki; McCormick, Theron G.

doi:10.1016/j.jaci.2005.04.044

Cited by 16 publications

(9 citation statements)

References 5 publications

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“…30 Related to these observations in primary immunodeficiency is the Images in Allergy and Immunology article that pictures the case of a child who developed severe mosquito bite hypersensitivity, ulcerating skin lesions, enlarged and draining adjacent lymph nodes, and marked hepatosplenomegaly. 31 Studies reveal that this child developed proliferation of EBV-containing NK cells similar, if not identical, to that seen in the few reported cases of chronic active EBV infection of NK cells that result in NK cell leukemia and lymphoma. 32 It is possible that this child is an example of the atypical immunodeficiency that presents with a more common and less marked clinical phenotype that ultimately might be resolved by detection of causal genes, as proposed by Casanova 33 and reviewed by Bonilla and Geha 34 in an editorial in this issue.…”

supporting

confidence: 65%

Infection versus immunity: What's the balance?

Shearer

2005

Journal of Allergy and Clinical Immunology

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supporting

confidence: 65%

Infection versus immunity: What's the balance?

Shearer

2005

Journal of Allergy and Clinical Immunology

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“…Patients with an HMB episode had high fever, intense local erythematous responses to mosquito bites, lymphadenopathy, and splenomegaly [21], [22]. Those with fulminant HLH episode met the updated diagnostic criteria of the HLH Study Group of the Histiocyte Society [15] and all had hemophagocytosis in bone marrow aspirates.…”

Section: Methodsmentioning

confidence: 99%

“…Hypersensitivity to mosquito bite (HMB) is a unique feature characterized by bulla formation with intense erythema on mosquito-bitten sites, escar healing and systemic manifestations like fever, lymphadenopathy, and splenomegaly [21], [22]. Around 70% of CAEBV patients present as the HMB episode (HMB-CAEBV) and have the potential of developing fulminant HLH [23].…”

Section: Introductionmentioning

confidence: 99%

Immunologic Difference between Hypersensitivity to Mosquito Bite and Hemophagocytic Lymphohistiocytosis Associated with Epstein-Barr Virus Infection

et al. 2013

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Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening, virus-triggered immune disease. Hypersensitivity to mosquito bite (HMB), a presentation of Chronic Active Epstein-Barr Virus infection (CAEBV), may progress to HLH. This study aimed to investigate the immunologic difference between the HMB episodes and the HLH episodes associated with EBV infection. Immunologic changes of immunoglobulins, lymphocyte subsets, cytotoxicity, intracellular perforin and granzyme expressions, EBV virus load and known candidate genes for hereditary HLH were evaluated and compared. In 12 HLH episodes (12 patients) and 14 HMB episodes (4 patients), there were both decreased percentages of CD4+ and CD8+ and increased memory CD4+ and activated (CD2+HLADR+) lymphocytes. In contrast to HMB episodes that had higher IgE levels and EBV virus load predominantly in NK cells, those HLH episodes with virus load predominantly in CD3+ lymphocyte had decreased perforin expression and cytotoxicity that were recovered in the convalescence period. However, there was neither significant difference of total virus load in these episodes nor candidate genetic mutations responsible for hereditary HLH. In conclusion, decreased perforin expression in the HLH episodes with predominant-CD3+ EBV virus load is distinct from those HMB episodes with predominant-NK EBV virus load. Whether the presence of non-elevated memory CD4+ cells or activated lymphocytes (CD2+HLADR+) increases the mortality rate in the HLH episodes remains to be further warranted through larger-scale studies.

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“…While this disorder is very rare, some cases of HMB mainly reported from East Asia, primarily from Japan (2). However, recently a 7-yr-old Latin American case was reported in 2005 (3). It has been suggested that HMB is associated with chronic Epstein-Barr virus (EBV) infection and natural killer (NK) cell leukemia/lymphoma (1, 4).…”

Section: Introductionmentioning

confidence: 99%

A Case of Hypersensitivity to Mosquito Bite Associated with Epstein-Barr Viral Infection and Natural Killer Cell Lymphocytosis

et al. 2010

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Hypersensitivity to mosquito bites (HMB) is a disorder characterized by a necrotic skin reaction and generalized symptoms subsequent to mosquito bites. It has been suggested that HMB is associated with chronic Epstein-Barr virus (EBV) infection and natural killer cell leukemia/lymphoma. We describe here a Korean child who had HMB associated with chronic EBV infection and natural killer cell lymphocytosis. A 5-yr-old boy was suffered from necrotic skin lesions on the right ear lobe. Type A EB virus was detected from hlood cells and bone marrow biospy recognized hemophagocyrosis.

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Chronic active Epstein-Barr virus infection of natural killer cells presenting as severe skin reaction to mosquito bites

Cited by 16 publications

References 5 publications

Infection versus immunity: What's the balance?

Infection versus immunity: What's the balance?

Immunologic Difference between Hypersensitivity to Mosquito Bite and Hemophagocytic Lymphohistiocytosis Associated with Epstein-Barr Virus Infection

A Case of Hypersensitivity to Mosquito Bite Associated with Epstein-Barr Viral Infection and Natural Killer Cell Lymphocytosis

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