Primary haemophagocytic lymphohistiocytosis (Chédiak‐Higashi Syndrome) triggered by acute SARS‐CoV‐2 infection in a six‐week‐old infant

Lange, Matthias; Linden, Tobias; Müller, Hermann L.; Flasskuehler, Meera A; Koester, Holger; Lehmberg, Kai; Ledig, Svea; Ehl, Stephan; Heep, Axel; Beske, Florian

doi:10.1111/bjh.17669

Cited by 9 publications

(18 citation statements)

References 9 publications

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“…Therefore, the patient was considered to have acquired HLH secondary to COVID‐19. By contrast, the case described by Lange was a case of fHLH (Chediak‐Higashi syndrome) triggered by COVID‐19 [ 6 ].…”

Section: Casementioning

confidence: 99%

“…Secondary HLH (sHLH) can occur in patients with rheumatologic, oncologic, or infectious diseases [ 5 ]. A case of COVID‐19 inducing HLH has previously been reported in a 6‐week‐old infant with Chediak‐Higashi syndrome [ 6 ]. Here we report a case of a pediatric patient who developed refractory sHLH to COVID‐19 infection and required a hematopoietic cell transplant (HCT) for the cure.…”

Section: Introductionmentioning

confidence: 99%

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COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

Greenmyer

Wyatt

Milanovich

et al. 2022

eJHaem

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Coronavirus disease 2019 (COVID‐19) infection causes a variety of extrapulmonary complications in pediatric patients. Multisystem inflammatory syndrome and hemophagocytic lymphohistiocytosis (HLH) are related to hypercytokinemia in COVID‐19 patients. HLH is a disorder of exaggerated inflammation resulting in a cytokine storm and unrestricted hemophagocytosis. HLH can be primary (familial) or secondary (acquired). Secondary HLH (sHLH) can occur in patients with rheumatologic, oncologic, or infectious diseases. The link between COVID‐19 and HLH has been reported in pediatric patients. Here we report a case of a pediatric patient who developed refractory sHLH secondary to COVID‐19 infection and required a hematopoietic cell transplant for the cure.

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Section: Casementioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

Greenmyer

Wyatt

Milanovich

et al. 2022

eJHaem

View full text Add to dashboard Cite

show abstract

“…As for immune dysregulation disorders, COVID-19 has been reported in 62 patients (10%), the majority of whom were affected by autoimmune polyendocrine syndrome type 1 13,14,16,21,22,37,40,56,57,70]. The rate of ICU admission was 28% and CFR 15% (n ¼ 9).…”

Section: Immune Dysregulation Disordersmentioning

confidence: 99%

Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

Bucciol

Tangye

Meyts

2021

Current Opinion in Pediatrics

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Purpose of review The severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic has caused extreme concern for patients with inborn errors of immunity (IEIs). In the first 6 months of the pandemic, the case fatality rate among patients with IEIs resembled that of the general population (9%). This review aims at summarizing what we have learned about the course and outcome of coronavirus disease 2019 (COVID-19) in patients with different IEIs and what this can potentially teach us about the immune mechanisms that could confer protection or predisposition to severe disease. Recent findings A total of 649 patients with IEI and COVID-19 have been reported in the last year and a half, spanning all groups of the International Union of Immunological Societies classification of IEIs. For most patients, the underlying IEI does not represent an independent risk factor for severe COVID-19. In fact, some IEI may even be protective against the severe disease due to impaired inflammation resulting in less immune-mediated collateral tissue damage. Summary We review the characteristics of SARS-CoV-2 infection in a large number of patients with IEI. Overall, we found that combined immunodeficiencies, immune dysregulation disorders, and innate immune defects impairing type I interferon responses are associated with severe disease course.

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“…In terms of children or infants, one 6-week old with COVID-19 was identified as having familial HLH (homozygous nonsense LYST mutation, p.Gly1675) (Fig. 1) (Table 1) triggered by SARS-CoV-2 [54]. Likewise, a toddler with COVID-19 was noted to have familial HLH (homozygous frameshift mutation in STX11 , p.Gln230Alafs∗125) (Fig.…”

Section: Cytokine Storm Syndrome Genes In Covid-19 and Multisystem In...mentioning

confidence: 99%

Host genetics of pediatric SARS-CoV-2 COVID-19 and multisystem inflammatory syndrome in children

Schulert

Blum

Cron

2021

Current Opinion in Pediatrics

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Purpose of review This review is meant to describe the genetic associations with pediatric severe COVID-19 pneumonia and the postinfectious complication of the multisystem inflammatory syndrome in children (MIS-C). Multiple genetic approaches have been carried out, primarily in adults with extrapolation to children, including genome-wide association studies (GWAS), whole exome and whole genome sequencing (WES/WGS), and target gene analyses. Recent findings Data from adults with severe COVID-19 have identified genomic regions (human leukocyte antigen locus and 3p21.31) as potential risk factors. Genes related to viral entry into cells (ABO blood group locus, ACE2, TMPRS22 ) have been linked to severe COVID-19 patients by GWAS and target gene approaches. Type I interferon (e.g. IFNAR2 ) and antiviral gene (e.g. TLR7 ) associations have been identified by several genetic approaches in severe COVID-19. WES has noted associations with several immune regulatory genes (e.g. SOCS1 ). Target gene approaches have identified mutations in perforin-mediated cytolytic pathway genes in children and adults with severe COVID-19 and children with MIS-C. Summary Several genetic associations have been identified in individuals with severe COVID-19 and MIS-C via various genetic approaches. Broadly speaking, COVID-19 genetic associations include genes involved with antiviral functions, viral cell entry, immune regulation, chemotaxis of white blood cells, and lymphocyte cytolytic function.

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Primary haemophagocytic lymphohistiocytosis (Chédiak‐Higashi Syndrome) triggered by acute SARS‐CoV‐2 infection in a six‐week‐old infant

Cited by 9 publications

References 9 publications

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

COVID‐19‐associated secondary hemophagocytic lymphohistiocytosis requiring hematopoietic cell transplant

Coronavirus disease 2019 in patients with inborn errors of immunity: lessons learned

Host genetics of pediatric SARS-CoV-2 COVID-19 and multisystem inflammatory syndrome in children

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