Secondary hemophagocytic lymphohistiocytosis and severe liver injury induced by hepatic SARS-CoV-2 infection unmasking Wilson’s disease: Balancing immunosuppression

Lubnow, Matthias; Schmidt, Bárbara; Salzberger, Bernd; Müller, Thomas; Peschel, G; Schneckenpointner, Roland; Lange, Tobias; Hitzenbichler, Florian; Kieninger, Martin; Lunz, Dirk; Graf, Bernhard M.; Brochhausen, Christoph; Weber, Florian; Lüke, Florian; Peterhoff, David; Schuster, Philipp; Hiergeist, Andreas; Offner, Robert; Hehr, Ute; Wallner, Stefan; Hanses, Frank; Schmid, Stephan; Weigand, Kilian; Geismann, Florian; Poeck, Hendrik; Pukrop, Tobias; Evert, Matthias; Gessner, André; Burkhardt, Ralph; Herr, Wolfgang; Maier, Lars S.; Heudobler, Daniel

doi:10.1016/j.ijid.2020.12.047

Cited by 16 publications

(12 citation statements)

References 9 publications

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“…Liver injury is widely reported in the setting of COVID‐19, although ALF due to COVID‐19 seems to be exceedingly rare and reported cases have potential competing diagnoses for ALF 9,10 . Infections have been reported to trigger acute Wilsonian crisis, and more recently, one case of SARS‐CoV‐2 infection “unmasked” WD was published 15 …”

Section: Discussionmentioning

confidence: 99%

Liver transplantation for acute liver failure in a SARS-CoV-2 PCR-positive patient

Yohanathan

Campioli

Mousa

et al. 2021

American Journal of Transplantation

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Current guidelines recommend deferring liver transplantation (LT) in patients with severe acute respiratory syndrome coronavirus 2 (SARS‐CoV‐2) infection until clinical improvement occurs and two PCR tests collected at least 24 hours apart are negative. We report a case of an 18‐year‐old, previously healthy African‐American woman diagnosed with COVID‐19, who presents with acute liver failure (ALF) requiring urgent LT in the context of SARS‐CoV‐2 polymerase chain reaction (PCR) positivity. The patient was thought to have acute Wilsonian crisis on the basis of hemolytic anemia, alkaline phosphatase:bilirubin ratio <4, AST:ALT ratio >2.2, elevated serum copper, and low uric acid, although an unusual presentation of COVID‐19 causing ALF could not be excluded. After meeting criteria for status 1a listing, the patient underwent successful LT, despite ongoing SARS‐CoV‐2 PCR positivity. Remdesivir was given immediately posttransplant, and mycophenolate mofetil was withheld initially and the SARS‐CoV‐2 PCR test eventually became negative. Three months following transplantation, the patient has made a near‐complete recovery. This case highlights that COVID‐19 with SARS‐CoV‐2 PCR positivity may not be an absolute contraindication for transplantation in ALF. Criteria for patient selection and timing of LT amid the COVID‐19 pandemic need to be validated in future studies.

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Section: Discussionmentioning

confidence: 99%

Liver transplantation for acute liver failure in a SARS-CoV-2 PCR-positive patient

Yohanathan

Campioli

Mousa

et al. 2021

American Journal of Transplantation

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“…[ 44 , 45 ] One case report describes a COVID-19 patient with severe liver injury who developed secondary hemophagocytic lymphohistiocytosis, and in whom Wilson disease was unmasked. [ 46 ]…”

Section: Gastrointestinal and Hepatobiliary Manifestations Of Covid-19mentioning

confidence: 99%

COVID-19: gastrointestinal and hepatobiliary manifestations

Shih

Misdraji

2023

Human Pathology

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“…Many treatments of hematologic malignancies, such as the cornerstone of treatment (anti‐CD‐20 monoclonal antibodies), may lead to prolonged B‐cell depletion and impaired immune responses 43,44 . From 32 case reports 45–76 and 11 case series, 27,44,77–85 we identified 150 COVID‐19 patients with hematological malignancies treated with convalescent plasma. Among these 150 patients, the mortality rate was 20% (30 of 150 patients), 37 patients demonstrated rapid clinical improvement after convalescent plasma transfusion, and the average time between convalescent plasma transfusion and hospital discharge was 27 days.…”

Section: Secondary Immunodeficiencymentioning

confidence: 99%

Use of convalescent plasma inCOVID‐19 patients with immunosuppression

et al. 2021

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In the absence of effective countermeasures, human convalescent plasma has been widely used to treat severe acute respiratory syndrome coronavirus 2, the causative agent of novel coronavirus disease 19 (COVID‐19), including among patients with innate or acquired immunosuppression. However, the association between COVID‐19‐associated mortality in patients with immunosuppression and therapeutic use of convalescent plasma is unknown. We review 75 reports, including one large matched‐control registry study of 143 COVID‐19 patients with hematological malignancies, and 51 case reports and 23 case series representing 238 COVID‐19 patients with immunosuppression. We review clinical features and treatment protocols of COVID‐19 patients with immunosuppression after treatment with human convalescent plasma. We also discuss the time course and clinical features of recovery. The available data from case reports and case series provide evidence suggesting a mortality benefit and rapid clinical improvement in patients with several forms of immunosuppression following COVID‐19 convalescent plasma transfusion. The utility of convalescent plasma or other forms of antibody therapy in immune‐deficient and immune‐suppressed patients with COVID‐19 warrants further investigation.

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Secondary hemophagocytic lymphohistiocytosis and severe liver injury induced by hepatic SARS-CoV-2 infection unmasking Wilson’s disease: Balancing immunosuppression

Cited by 16 publications

References 9 publications

Liver transplantation for acute liver failure in a SARS-CoV-2 PCR-positive patient

Liver transplantation for acute liver failure in a SARS-CoV-2 PCR-positive patient

COVID-19: gastrointestinal and hepatobiliary manifestations

Use of convalescent plasma inCOVID‐19 patients with immunosuppression

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