Elevated antibody titers to Epstein-Barr virus and low natural killer cell activity in patients with Chediak-Higashi syndrome

Merino, Fernando; Klein, George; Henle, Werner; Ramı́rez-Duque, Pedro; Forsgren, Marianne; Amesty, Carmen

doi:10.1016/0090-1229(83)90085-5

Cited by 46 publications

(12 citation statements)

References 36 publications

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“…This contention could find support in our recent studies in Chediak-Higashi patients as it has been shown these patients have low or nondemonstrable NK cell activity in peripheral blood cells [18]. A re cent description of cases from a rural community of Venezuela [19] did not evidence any increase in the frequency of parasitic diseases in these children des pite the fact that they live in a village and are exposed to different common parasites.…”

Section: Discussionsupporting

confidence: 61%

Natural Killer Activity in Experimental Cutaneous Leishmaniasis

Merino¹,

Cruz²

1984

Int Arch Allergy Immunol

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Studies were performed to determine the role of natural killer (NK) cell cytotoxicity in experimental cutaneous leishmaniasis. Analysis of a possible correlation between in vitro NK cell activity and in vivo susceptibility to Leishmania mexicana infection showed that there is no relationship between the degree of NK reactivity to YAC-1 lymphoma cells and in vivo leishmania growth. It was also observed that spleen lymphoid cells from mice with high NK activity did not cause an increase in isotope release by the macrophage permanent cell line J774G8-1 previously infected with the parasite and supporting its growth. Mice infected with L. mexicana manifested an increased NK activity to YAC-1 cells but not to leishmania-infected J774G8-1 tumor macrophages. The lack of effect of NK cell activity is discussed with regard to the role of NK cells in immune mechanisms to intracellular parasites.

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Section: Discussionsupporting

confidence: 61%

Natural Killer Activity in Experimental Cutaneous Leishmaniasis

Merino¹,

Cruz²

1984

Int Arch Allergy Immunol

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“…Thus, the clinical picture of XLP may be explained in part by a failure of NK cell control of primary EBV infection. Likewise, low NK cell activity has been identified in other rare disorders in which uncontrolled EBV infection may occur: EBV related haemaophagocytic syndrome, Chediak–Higashi syndrome, and chronic active EBV (Merino et al , 1983; Joncas et al , 1989; Kogawa et al , 2002).…”

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confidence: 99%

The immune response to primary EBV infection: a role for natural killer cells

et al. 2005

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SummaryThe role of antigen-specific CD3 + CD8 + cytotoxic T cells in the control of primary Epstein-Barr Virus (EBV) infection is well established. However, time is required for the antigen-specific immune response to develop and expand. In contrast, innate immune responses, such as natural killer (NK) cells, are considered vital early in the infection process. We analysed the scale, phenotype and function of the NK cell response during symptomatic primary EBV infection, infectious mononucleosis (IM) and showed that NK cell numbers were significantly elevated both at diagnosis of IM and in the first month following diagnosis. There were also significant changes in cell phenotype and function, an increase in the proportion of CD56 bright cells at diagnosis, and freshly isolated cells showing an enhanced ability to kill EBVinfected cell lines. Moreover, in our cohort of IM patients higher NK cell counts were associated with significantly lower viral load in peripheral blood. Our results suggest NK cells have an important role in the control of primary EBV infection by eliminating infected B cells and augmenting the antigenspecific T cell response via release of immunomodulatory cytokines. The magnitude of the NK cell response may ultimately determine whether primary EBV infection has a clinical outcome.

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“…Though the common organism associated with infections in the chronic stable phase of the disease is Staphylococcus aureus, the Epstein-Barr virus (EBV) is implicated in the accelerated phase. High-liter scropositivity for the VCA and other antigens has been described in patients with accelerated disease [4]. Inability to clear the infection by EBV is believed to lead to a state of contstant lymphoproliferation, seen in the phase of disease acceleration [6].…”

Section: Discussionmentioning

confidence: 99%

“…The accelerated phase of CHS is characterized by high fever, pancytopenia, and lymphohistiocytic infiltration of liver, spleen, and lymph nodes [3]. Defective natural killer activity, and episodes of macrophage activation are known in the accelerated phase [4]. Most patients undergo a variable period of recurrent infections before going into the accelerated phase.…”

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confidence: 99%

Accelerated Phase at Initial Presentation: An Uncommon Occurrence in Chédiak-Higashi Syndrome

Ahluwalia

Pattari

Trehan

et al. 2003

Pediatric Hematology and Oncology

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The authors describe an Indian child, who presented in the accelerated phase of the Chédiak-Higashi syndrome. The disease usually presents in early childhood with recurrent skin and mucosal infections. This patient had subtle pigmentary abnormalities and no family history of the disease, which made the clinical diagnosis difficult. The cytopenias, hepatosplenomegaly, lymphohistiocytic infiltrate in the bone marrow, and the characteristic granules in the leucocytes clinched the diagnosis. This case underscores the importance of a bone marrow examination in patients with unusual presentations of rare disorders.

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Elevated antibody titers to Epstein-Barr virus and low natural killer cell activity in patients with Chediak-Higashi syndrome

Cited by 46 publications

References 36 publications

Natural Killer Activity in Experimental Cutaneous Leishmaniasis

Natural Killer Activity in Experimental Cutaneous Leishmaniasis

The immune response to primary EBV infection: a role for natural killer cells

Accelerated Phase at Initial Presentation: An Uncommon Occurrence in Chédiak-Higashi Syndrome

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