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Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: Clinical Management
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Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: Clinical Management

A special issue of Journal of Clinical Medicine (ISSN 2077-0383). This special issue belongs to the section "Immunology".

Deadline for manuscript submissions: closed (15 January 2024) | Viewed by 9013

Special Issue Editors

Dr. Jamilloux Yvan

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Guest Editor
Department of Internal Medicine, Croix-Rousse University Hospital, Hospices Civils de Lyon, Faculty of Medicine and Maïeutics Lyon Sud-Charles Mérieux, Lyon, France
Interests: still's disease; juvenile idiopathic arthritis; macrophage activation syndrome
Special Issues, Collections and Topics in MDPI journals
Dr. Maël Heiblig

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Guest Editor
Department of Hematology, Lyon Sud University Hospital, Hospices Civils de Lyon, Faculty of Medicine and Maïeutics Lyon Sud-Charles Mérieux, Lyon, France
Interests: myeloid cells; VEXAS
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Cytokine storm syndromes (CSS) encompass a range of conditions characterized by uncontrolled inflammation and hypercytokinemia. Various mechanisms lead to different CSS, the form and nature of which are mainly governed by host-intrinsic factors, environmental triggers and the pathological context.

Two CSS have been recently highlighted: cytokine release syndrome associated with CAR T cell treatment and CSS associated with SARS-CoV-2 infection. However, the best known and most studied CSS remains lymphohistiocytic haemophagocytosis (HLH). HLH is classically divided into two categories: primary HLH due to genetic mutations, and secondary HLH (sHLH), which is termed MAS when it is associated with rheumatic underlying disease. sHLH is a life-threatening condition that may accompany sepsis (either viral, bacterial, fungal or parasitic), Still’s disease, systemic lupus or cancers. The overall mortality of HLH is about 30–40%, depending on the underlying condition.

Several CSS share the common feature of increased serum ferritin that can reach extreme values, and some authors have proposed to group them under the umbrella term “hyperferritinaemic syndromes”.

Advances in molecular biology, NGS, the rise of immunomonitoring and targeted therapies, as well as the increase in knowledge about CSS have greatly changed the way they are viewed and managed.

This Special Issue entitled “Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: From Basic Science to Patient Care” of the Journal of Clinical Medicine is now open for submissions. This issue welcomes all types of papers* on the broad spectrum of pathophysiology, epidemiology, diagnosis, prognosis (biomarkers), and management. This Special Issue aims to provide the reader with a comprehensive overview of current knowledge.

*Guest editors will not accept case reports. Some case series accompanied by high-level literature review will be considered. Viewpoints from recognized experts are welcome.

Dr. Jamilloux Yvan
Dr. Maël Heiblig
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Journal of Clinical Medicine is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cytokine storm syndrome
  • hemophagocytic lymphohistiocytosis
  • macrophage activation syndrome
  • cytokine relase syndrome
  • ferritin
  • hematological malignancy
  • interleukin-1
  • interleukin-6
  • interferon gamma

Published Papers (4 papers)

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Research

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11 pages, 1351 KiB  
Article
Mortality and Associated Causes in Hemophagocytic Lymphohistiocytosis: A Multiple-Cause-of-Death Analysis in France
by Solène La Marle, Gaëlle Richard-Colmant, Mathieu Fauvernier, Hervé Ghesquières, Arnaud Hot, Pascal Sève and Yvan Jamilloux
J. Clin. Med. 2023, 12(4), 1696; https://doi.org/10.3390/jcm12041696 - 20 Feb 2023
Cited by 5 | Viewed by 2457
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome with an overall mortality rate of 40%. A multiple-cause-of-death analysis allows for the characterization of mortality and associated causes over an extended period. Death certificates, collected between 2000 and 2016 by the French Epidemiological Centre [...] Read more.
Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome with an overall mortality rate of 40%. A multiple-cause-of-death analysis allows for the characterization of mortality and associated causes over an extended period. Death certificates, collected between 2000 and 2016 by the French Epidemiological Centre for the Medical Causes of Death (CepiDC, Inserm), containing the ICD10 codes for HLH (D76.1/2), were used to calculate HLH-related mortality rates and to compare them with the general population (observed/expected ratios, O/E). HLH was mentioned in 2072 death certificates as the underlying cause of death (UCD, n = 232) or as a non-underlying cause of death (NUCD, n = 1840). The mean age at death was 62.4 years. The age-standardized mortality rate was 1.93/million person-years and increased over the study period. When HLH was an NUCD, the most frequently associated UCDs were hematological diseases (42%), infections (39.4%), and solid tumors (10.4%). As compared to the general population, HLH decedents were more likely to have associated CMV infections or hematological diseases. The increase in mean age at death over the study period indicates progress in diagnostic and therapeutic management. This study suggests that the prognosis of HLH may be at least partially related to coexisting infections and hematological malignancies (either as causes of HLH or as complications). Full article
(This article belongs to the Special Issue Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: Clinical Management)
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15 pages, 2126 KiB  
Article
Differential Diagnosis of Hyperferritinemia in Critically Ill Patients
by Friederike S. Schuster, Peter Nyvlt, Patrick Heeren, Claudia Spies, Moritz F. Adam, Thomas Schenk, Frank M. Brunkhorst, Gritta Janka, Paul La Rosée, Cornelia Lachmann and Gunnar Lachmann
J. Clin. Med. 2023, 12(1), 192; https://doi.org/10.3390/jcm12010192 - 27 Dec 2022
Cited by 3 | Viewed by 3316
Abstract
Background: Elevated serum ferritin is a common condition in critically ill patients. It is well known that hyperferritinemia constitutes a good biomarker for hemophagocytic lymphohistiocytosis (HLH) in critically ill patients. However, further differential diagnoses of hyperferritinemia in adult critically ill patients remain poorly [...] Read more.
Background: Elevated serum ferritin is a common condition in critically ill patients. It is well known that hyperferritinemia constitutes a good biomarker for hemophagocytic lymphohistiocytosis (HLH) in critically ill patients. However, further differential diagnoses of hyperferritinemia in adult critically ill patients remain poorly investigated. We sought to systematically investigate hyperferritinemia in adult critically ill patients without HLH. Methods: In this secondary analysis of a retrospective observational study, patients ≥18 years admitted to at least one adult intensive care unit at Charité–Universitätsmedizin Berlin between January 2006 and August 2018, and with hyperferritinemia of ≥500 μg/L were included. Patients with HLH were excluded. All patients were categorized into non-sepsis, sepsis, and septic shock. They were also classified into 17 disease groups, based on their ICD-10 codes, and pre-existing immunosuppression was determined. Uni- and multivariable linear regression analyses were performed in all patients. Results: A total of 2583 patients were analyzed. Multivariable linear regression analysis revealed positive associations of maximum SOFA score, sepsis or septic shock, liver disease (except hepatitis), and hematological malignancy with maximum ferritin. T/NK cell lymphoma, acute myeloblastic leukemia, Kaposi’s sarcoma, acute or subacute liver failure, and hepatic veno-occlusive disease were positively associated with maximum ferritin in post-hoc multivariable linear regression analysis. Conclusions: Sepsis or septic shock, liver disease (except hepatitis) and hematological malignancy are important differential diagnoses in hyperferritinemic adult critically ill patients without HLH. Together with HLH, they complete the quartet of important differential diagnoses of hyperferritinemia in adult critically ill patients. As these conditions are also related to HLH, it is important to apply HLH-2004 criteria for exclusion of HLH in hyperferritinemic patients. Hyperferritinemic critically ill patients without HLH require quick investigation of differential diagnoses. Full article
(This article belongs to the Special Issue Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: Clinical Management)
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Review

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18 pages, 1877 KiB  
Review
Diagnostic Challenges in Hemophagocytic Lymphohistiocytosis, a Rare, Potentially Fatal Disease: Two Case Studies
by Marcela Daniela Ionescu, Bianca Prajescu, Roxana Taras, Nicoleta Popescu, Ruxandra Vidlescu, Mihaela Smarandoiu, Loredana-Elena Rosca, Augustina Enculescu, Elena Camelia Berghea and Claudia Lucia Toma
J. Clin. Med. 2024, 13(6), 1643; https://doi.org/10.3390/jcm13061643 - 13 Mar 2024
Viewed by 786
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a rare, and potentially fatal, syndrome, characterized by immune system dysregulation, with excessive activation of the macrophages and cytotoxic T cells. It can be classified into primary (genetic) and secondary (acquired) forms. HLH presents with fever, hepatosplenomegaly, cytopenia, and [...] Read more.
Hemophagocytic lymphohistiocytosis (HLH) is a rare, and potentially fatal, syndrome, characterized by immune system dysregulation, with excessive activation of the macrophages and cytotoxic T cells. It can be classified into primary (genetic) and secondary (acquired) forms. HLH presents with fever, hepatosplenomegaly, cytopenia, and hyperferritinemia, with involvement of various organs. The initial symptoms of HLH are non-specific, but as, if untreated, it can progress rapidly to multiorgan failure, timely diagnosis is essential. We present here two cases of HLH in infants that illustrate the importance of early diagnosis and appropriate treatment, along with a short review of HLH. Full article
(This article belongs to the Special Issue Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: Clinical Management)
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Other

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18 pages, 1754 KiB  
Systematic Review
Tuberculosis-Associated Hemophagocytic Lymphohistiocytosis: A Review of Current Literature
by Trym Fauchald, Bjørn Blomberg and Håkon Reikvam
J. Clin. Med. 2023, 12(16), 5366; https://doi.org/10.3390/jcm12165366 - 18 Aug 2023
Cited by 1 | Viewed by 1820
Abstract
Hemophagocytic lymphohistiocytosis (HLH) is a condition of immune dysregulation and hyperinflammation, leading to organ failure and death. Malignancy, autoimmune conditions, and infections, including Mycobacterium tuberculosis (TB), are all considered triggers of HLH. The aim of this study was to review all reported cases [...] Read more.
Hemophagocytic lymphohistiocytosis (HLH) is a condition of immune dysregulation and hyperinflammation, leading to organ failure and death. Malignancy, autoimmune conditions, and infections, including Mycobacterium tuberculosis (TB), are all considered triggers of HLH. The aim of this study was to review all reported cases of TB-associated HLH in English literature, and to summarize the epidemiology, diagnostics, treatment, and mortality in patients with concomitant HLH and TB. A systematic review of described cases with TB-associated HLH, via a structured literature search in the medical database PubMed, is presented. Additional articles were included through cross-referencing with existing review articles. Articles were reviewed based on a predetermined set of criteria. A total of 116 patients with TB-associated HLH were identified with a male:female ratio of about 3:2. The age at presentation ranged from 12 days to 83 years. Malignancy, autoimmunity, and renal failure were the most common comorbid conditions. Most patients received both tuberculostatic and specific immunomodulating treatment, which was associated with a 66% (48/73) survival rate compared to 56% (15/27) in those receiving only tuberculostatic treatment, and 0% (0/13) in those receiving only immunomodulating treatment. The survival rate was 55% overall. The overlapping presentation between disseminated TB and HLH poses challenging diagnostics and may delay diagnosis and treatment, leading to increased mortality. TB should be considered as a potential trigger of HLH; clinicians’ knowledge and awareness of this may result in the appropriate investigations needed to ensure diagnosis and proper treatment. Full article
(This article belongs to the Special Issue Cytokine Storms, Hemophagocytic Lymphohistiocytosis and Hyperferritinemic Syndromes: Clinical Management)
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