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Hereditary haemochromatosis, haemophagocytic lymphohistiocytosis and COVID-19.
Riley, Matthew J; Hicks, Scott R; Irvine, Sharon; Blanchard, Tom J; Britton, Edward; Shawki, Howida; Sajid Pervaiz, Muhammad; Fletcher, Tom E.
  • Riley MJ; Tropical and Infectious Diseases Unit, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
  • Hicks SR; Tropical and Infectious Diseases Unit, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
  • Irvine S; Tropical and Infectious Diseases Unit, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
  • Blanchard TJ; Tropical and Infectious Diseases Unit, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
  • Britton E; Department of Gastroenterology and Hepatology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
  • Shawki H; Liverpool Clinical Laboratories, Duncan Building, Royal Liverpool University Hospital, Daulby Street, Liverpool L69 3GA, United Kingdom.
  • Sajid Pervaiz M; Department of Haematology, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
  • Fletcher TE; Tropical and Infectious Diseases Unit, Royal Liverpool University Hospital, Prescot Street, Liverpool L7 8XP, United Kingdom.
Clin Infect Pract ; 7: 100052, 2020 Oct.
Article in English | MEDLINE | ID: covidwho-893696
Semantic information from SemMedBD (by NLM)
1. Iron Overload PREDISPOSES Severe disease
Subject
Iron Overload
Predicate
PREDISPOSES
Object
Severe disease
2. Severe disease COEXISTS_WITH Virus Diseases
Subject
Severe disease
Predicate
COEXISTS_WITH
Object
Virus Diseases
3. Immune System Diseases COEXISTS_WITH Hereditary hemochromatosis
Subject
Immune System Diseases
Predicate
COEXISTS_WITH
Object
Hereditary hemochromatosis
4. Lymphohistiocytosi COMPLICATES C5203670
Subject
Lymphohistiocytosi
Predicate
COMPLICATES
Object
C5203670
5. COVID-19 PROCESS_OF Male population group
Subject
COVID-19
Predicate
PROCESS_OF
Object
Male population group
6. anakinra INHIBITS Receptor
Subject
anakinra
Predicate
INHIBITS
Object
Receptor
7. Cholangiopancreatograph DIAGNOSES C0268066
Subject
Cholangiopancreatograph
Predicate
DIAGNOSES
Object
C0268066
8. Uptake PROCESS_OF lymph nodes
Subject
Uptake
Predicate
PROCESS_OF
Object
lymph nodes
9. Demand (clinical) USES oxygen
Subject
Demand (clinical)
Predicate
USES
Object
oxygen
10. Autopsy DIAGNOSES Hemosiderin pigmentation
Subject
Autopsy
Predicate
DIAGNOSES
Object
Hemosiderin pigmentation
11. Liver LOCATION_OF Hemosiderin pigmentation
Subject
Liver
Predicate
LOCATION_OF
Object
Hemosiderin pigmentation
12. Immune COEXISTS_WITH Hereditary hemochromatosis
Subject
Immune
Predicate
COEXISTS_WITH
Object
Hereditary hemochromatosis
13. Underlying COEXISTS_WITH Hereditary hemochromatosis
Subject
Underlying
Predicate
COEXISTS_WITH
Object
Hereditary hemochromatosis
14. Iron Overload PREDISPOSES Severe disease
Subject
Iron Overload
Predicate
PREDISPOSES
Object
Severe disease
15. Severe disease COEXISTS_WITH Virus Diseases
Subject
Severe disease
Predicate
COEXISTS_WITH
Object
Virus Diseases
16. Immune System Diseases COEXISTS_WITH Hereditary hemochromatosis
Subject
Immune System Diseases
Predicate
COEXISTS_WITH
Object
Hereditary hemochromatosis
17. Lymphohistiocytosis, Hemophagocytic COMPLICATES COVID-19
Subject
Lymphohistiocytosis, Hemophagocytic
Predicate
COMPLICATES
Object
COVID-19
18. COVID-19 PROCESS_OF Male population group
Subject
COVID-19
Predicate
PROCESS_OF
Object
Male population group
19. anakinra INHIBITS Receptors, Interleukin-1
Subject
anakinra
Predicate
INHIBITS
Object
Receptors, Interleukin-1
20. Cholangiopancreatography, Magnetic Resonance DIAGNOSES Hepatic hemosiderosis
Subject
Cholangiopancreatography, Magnetic Resonance
Predicate
DIAGNOSES
Object
Hepatic hemosiderosis
21. Uptake PROCESS_OF lymph nodes
Subject
Uptake
Predicate
PROCESS_OF
Object
lymph nodes
22. Demand (clinical) USES oxygen
Subject
Demand (clinical)
Predicate
USES
Object
oxygen
23. Autopsy DIAGNOSES Hemosiderin pigmentation
Subject
Autopsy
Predicate
DIAGNOSES
Object
Hemosiderin pigmentation
24. Liver LOCATION_OF Hemosiderin pigmentation
Subject
Liver
Predicate
LOCATION_OF
Object
Hemosiderin pigmentation
25. Immune COEXISTS_WITH Hereditary hemochromatosis
Subject
Immune
Predicate
COEXISTS_WITH
Object
Hereditary hemochromatosis
26. Underlying COEXISTS_WITH Hereditary hemochromatosis
Subject
Underlying
Predicate
COEXISTS_WITH
Object
Hereditary hemochromatosis
ABSTRACT

BACKGROUND:

Syndromes of iron overload have been shown to increase the risk of severe clinical disease in viral infections. Immune dysfunction is similarly described in hereditary haemochromatosis (HH). We present here the case of a 51-year-old man who developed severe coronavirus disease 2019 (COVID-19) complicated by suspected haemophagocytic lymphohistiocytosis (HLH). He was found to have HH post-mortem and we propose a link between his iron overload and the development of severe COVID-19. CASE REPORT The initial clinical presentation consisted of cough, shortness of breath and fever. Pancytopenia, markedly elevated ferritin and d-dimer were present. Computed tomography (CT) showed bilateral ground glass changes consistent with COVID-19, widespread lymphadenopathy and splenomegaly. A subsequent combined nose and throat swab was positive for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). HLH was suspected based upon the H-score and Anakinra, an IL-1 receptor antagonist, was commenced. Liver function acutely worsened and magnetic resonance cholangiopancreatography (MRCP) revealed hepatic haemosiderosis. Intense splenic and cervical lymph node uptake were seen on a positron emission tomography (PET) scan and high doses of intravenous steroids were administered due to concerns over haematological malignancy.

RESULTS:

Day fourteen of admission heralded the start of progressive clinical deterioration with rapid increase in oxygen demands. Continuous positive airway pressure (CPAP) was trialled without success and the patient unfortunately died seventeen days into admission. Results returned after his death showed homozygous C282Y mutation of the HFE gene consistent with a diagnosis of HH. Post-mortem examination revealed widespread haemosiderin deposition in the liver along with lung pathology in keeping with severe COVID-19 and widespread splenic infarctions.

CONCLUSION:

An association between HH and COVID-19 is not currently described in the literature. What does exist, however, is an evidence base for the detrimental impacts iron overload has on viral infections in general and the negative effects of HH on the immune system. We therefore postulate that the underlying metabolic and immune disturbances seen in HH should be considered a potential risk factor for the development of severe COVID-19. This case also adds to the evidence that hyperinflammation appears to be a unique and interesting characteristic of this novel viral disease.
Keywords

Full text: Available Collection: International databases Database: MEDLINE Type of study: Case report / Prognostic study Language: English Journal: Clin Infect Pract Year: 2020 Document Type: Article Affiliation country: J.clinpr.2020.100052

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Full text: Available Collection: International databases Database: MEDLINE Type of study: Case report / Prognostic study Language: English Journal: Clin Infect Pract Year: 2020 Document Type: Article Affiliation country: J.clinpr.2020.100052