Hemophagocytic lymphohistiocytosis and primary immune deficiency disorders

Faitelson, Yoram; Grunebaum, Eyal

doi:10.1016/j.clim.2014.09.008

Cited by 51Autoimmune and Inflammatory Disorders ResearchImmunodeficiency and Autoimmune DisordersInflammasome and immune disorders

Hemophagocytic lymphohistiocytosis and primary immune deficiency disorders

Faitelson, Yoram, Grunebaum, Eyal·2014·Clinical Immunology

DOI 10.1016/j.clim.2014.09.008

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Abstract

Hemophagocytic lymphohistiocytosis (HLH) is characterized by uncontrolled immune activation and is traditionally associated with inherited gene defects or acquired causes. In addition to abnormalities in cytotoxic granules and lysosomes, various primary immune deficiency disorders (PID) have been identified among patients suffering from HLH. Our purpose was twofold: to better characterize and detail the association between PID and HLH. We found that HLH occurs infrequently among patients with PID, particularly those suffering from abnormalities that impair T cell function. The prognosis of patients suffering from PID and HLH is poor, emphasizing the need for rapid clinical and genetic diagnosis of the PID as well as initiation of appropriate management of the HLH, including allogeneic hematopoietic stem cell transplantations. The association of HLH and PID implicates abnormal T cell function as an important factor in HLH development. It also suggests that the partition of HLH into genetic versus acquired forms might be misleading.

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Patients with genetic immunodeficiency syndromes such as chronic granulomatous disease and STAT1 gain-of-function disease can paradoxically present with cytokine storm from overwhelming infections.
“Patients with genetic immunodeficiency syndromes such as chronic granulomatous disease and STAT1 gain-of-function disease can, paradoxically, present with cytokine storm from overwhelming infections.78”
From Cytokine storm (2020) · cites this paper as [78]

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Authors: Faitelson, Y., & Grunebaum, E.
Affiliations: University of Toronto (CA)
Corresponding author: Grunebaum, Eyal
ORCID: Faitelson, Yoram
Grunebaum, Eyal
Venue: Clinical Immunology
Publisher: Elsevier BV
Published: 2014-11
Volume: 155
Issue: 1
Pages: 118-125
ISSN: 1521-6616 (ISSN-L)
DOI: 10.1016/j.clim.2014.09.008
OpenAlex: W2064270136
Cited by: 51
References: 70
Citations by year: 2026: 3 · 2025: 1 · 2024: 3 · 2023: 3 · 2022: 3 · 2021: 3 · 2020: 6 · 2019: 1 · 2018: 2 · 2017: 4 · 2016: 13 · 2015: 8 · 2014: 1
FWCI: 1.33
Type: journal article
Language: en
Open access: closed
Licence: www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations: Clinical Immunology (publishedVersion)
PubMed (publishedVersion)
Funders: Jeffrey Modell Foundation
Donald and Audrey Campbell Chair of Immunology
Topics: Health Sciences › Medicine › Hematology › Autoimmune and Inflammatory Disorders Research
Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Inflammasome and immune disorders
Keywords: hemophagocytic lymphohistiocytosis, T cell dysfunction, allogeneic hematopoietic stem cell transplantation, prognosis, genetic diagnosis
MeSH terms: Humans, Immunologic Deficiency Syndromes, Lymphocytes, Macrophages, Lymphohistiocytosis, Hemophagocytic

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Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

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Hemophagocytic lymphohistiocytosis and primary immune deficiency disorders

Abstract

Integrity signals

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