Impaired natural killer activity in lymphohistiocytosis syndrome
Abstract
In six patients with well-documented lymphohistiocytosis syndrome, natural killer activity was found to be profoundly impaired and could not be increased by incubation in vitro with interferon. This abnormality was not found in parents of the affected children. A clear correlation with the activity of the disease was observed, although a delay of a few weeks (possibly reflecting the life span of NK cells in blood) was seen in the disappearance of NK activity after the onset of the disease and its reappearance after remission. No absolute correlation was observed between NK activity and percentage of leukocytes detected by the Leu-7 monoclonal antibody. Our findings indicate that testing NK activity is useful for the diagnosis of lymphohistiocytosis syndrome and can be used as an index of activity of the disease, among other major clinical and biologic signs of this syndrome. Reversal of the NK activity defect (rather than detection of Leu-7 positive cells) appears to be a good criterion of complete remission.
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The cytolytic function of NK cells is diminished in some forms of cytokine storm, which can lead to prolonged antigenic stimulation and difficulty resolving inflammation.
“The cytolytic function of NK cells is diminished in some forms of cytokine storm, which can lead to prolonged antigenic stimulation and difficulty resolving inflammation.”
From Cytokine storm (2020) · cites this paper as [17]
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“In particular, demonstration of frequent association with common pathogens, together with evidence of impaired natural killer cytotoxic activity, provided the rationale for considering HLH as a selective immune deficiency.”
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“In the setting of active HLH, the functional activity of NK cells is usually impaired.(27) An inability to reconstitute NK functional activity in vitro using modified assay conditions, and the persistence of depressed NK cell function after treatment of HLH, are associated with FHL.”
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“Poorly functioning natural killer (NK) cells fail to induce apoptosis of activated cells resulting in a persistent inflammatory state characterized by increased interleukin 1 (IL-1), IL-6, and tumor necrosis factor > production.(2) Hemophagocytic lymphohistiocytosis is subdivided into primary and secondary forms.”
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“NK cell activity was noted to be severely impaired (3, 5, 6), but NK absolute cell number remained normal (5), or decreased (3) as in our case.”
BackgroundInfluentialNatural Killer Cells in Relation to Disease and Treatment
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Familial Hemophagocytic Lymphohistiocytosis: Diagnostic Problems and Differential Diagnosis
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Familial Hemophagocytic Lymphohistiocytosis: Too Little Cell Death Can Seriously Damage Your Health
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“The pathophysiologic progression of HLH begins with a predisposing immunodysfunction, which leads to impaired attenuation of immune cell activation, leading to hypercytokinemia [7,8].”
BackgroundEnterovirus-associated haemophagocytic syndrome in a neonate
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Treatment of familial erythrophagocytic lymphohistiocytosis with cyclosporine A
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Electrical stimulation of the brain for relief of intractable pain due to cancer
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“In addition, the number and function of natural killer cells is reduced.(6) All three patients described here had hypoxemia and transaminitis suggestive of multiorgan dysfunction (Table 1).”
BackgroundNonneoplastic Histiocytic Proliferations of Lymph Nodes and Bone Marrow
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CD8+ T Cell Biology in Cytokine Storm Syndromes
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- Authors
- Perez, N., Virelizier, J.-L., Arenzana-Seisdedos, F., Fischer, A., & Griscelli, C.
- ORCID
- Fischer, Alain
- Venue
- The Journal of Pediatrics
- Publisher
- Elsevier BV
- Published
- 1984-04
- Volume
- 104
- Issue
- 4
- Pages
- 569-573
- ISSN
- 0022-3476 (ISSN-L)
- OpenAlex
- W2059566117
- Cited by
- 121
- References
- 26
- Citations by year
- 2026: 1 · 2025: 1 · 2024: 2 · 2023: 2 · 2022: 2 · 2021: 5 · 2020: 1 · 2019: 2 · 2018: 3 · 2017: 1 · 2016: 2 · 2015: 1 · 2014: 1 · 2013: 3 · 2012: 3
- FWCI
- 1.17
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- The Journal of Pediatrics (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Life Sciences › Immunology and Microbiology › Immunology › Immune Cell Function and Interaction
- Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
- Keywords
- natural killer cells, NK cell dysfunction, interferon, disease activity monitoring, remission, NK cell depletion
- MeSH terms
- Antibodies, Monoclonal, Cytotoxicity, Immunologic, Fluorescent Antibody Technique, Humans, Killer Cells, Natural, Leukocytes, Lymphatic Diseases
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