The DOI matches its Crossref record; 25 of the 26 references it cites were found in a registry or an index.
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.”
From Cytokine storm (2020) · cites this paper as [17]
Citations
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Cited by
Sorted by citations
HLH‐2004: Diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis
Henter, J., Horne, A., Aricò, M., Egeler, R. M., Filipovich, A. H., Imashuku, S., Ladisch, S., McClain, K. L., Webb, D. K. H., Winiarski, J. H., et al. (2006) · Pediatric Blood & Cancer · doi:10.1002/pbc.21039 · Cited by 5,396
“This causes the defect in the NK and T-cell cytotoxicity that has been recognized for long [15,16],with three causativegene defects known today; PRF [9], UNC13D [40], and STX11 [41].”
BackgroundFajgenbaum, D. C., & June, C. H. (2020) · New England Journal of Medicine · doi:10.1056/nejmra2026131 · Cited by 3,196
Familial hemophagocytic lymphohistiocytosis
Janka, G. (1983) · European Journal of Pediatrics · doi:10.1007/bf00443367 · Cited by 576
Masera, G., Chesler, M. A., Jankovic, M., Ablin, A. R., Arush, M. W. B., Breatnach, F., McDowell, H. P., Eden, T. O. B., Epelman, C. L., Bellani, F. F., et al. (1997) · Medical and Pediatric Oncology · doi:10.1002/(sici)1096-911x(199705)28:5 · Cited by 71
Familial and acquired hemophagocytic lymphohistiocytosis
Janka, G. (2006) · European Journal of Pediatrics · doi:10.1007/s00431-006-0258-1 · Cited by 534
“A hallmark of HLH, in genetic as well as in acquired cases, is impaired or absent function of natural killer (NK) cells and cytotoxic T lymphocytes (CTL) measured as lysis of K652 cells in a standard 4h chromium release assay [5, 17, 88, 102, 112].”
Background
Metadata
- 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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