Is COVID-19-associated cytokine storm distinct from non-COVID-19 secondary hemophagocytic lymphohistiocytosis?

Liu, Johnson M; Chi, Jeffrey

doi:10.1177/15353702211068840

Cited by 12 publications

(12 citation statements)

References 48 publications

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“…Our patient had a significant increase in serum urea and creatinine levels when he was admitted to the hospital, but an improvement was seen with no need for dialysis. We suspected an immune-mediated mechanism or hemophagocytic syndrome; 27 however, the investigation was unremarkable, and the true etiology of renal damage was unclear.…”

Section: Discussionmentioning

confidence: 96%

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

et al. 2023

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COVID-19 is commonly associated with high serum levels of pro-inflammatory cytokines, and the post-infection status can disturb self-tolerance and trigger autoimmune responses. We are reporting a 45-year-old male who was admitted with fatigue, jaundice, elevated liver enzymes (with cholestatic pattern), and acute kidney injury two weeks after recovering from a mild SARS-CoV-2 infection. Serologies for viral hepatitis and anti-mitochondrial antibody were negative, while anti-nuclear and anti-smooth muscle antibodies were positive. There were no signs of chronic liver disease, and a magnetic resonance cholangiography showed no dilatation of biliary ducts. Histologic evaluation of the liver evidenced numerous foci of lobular necrosis without ductopenia or portal biliary reaction. Considering the autoantibody profile and histologic changes, the medical team started oral prednisone, but there was a suboptimal biochemical response in the outpatient follow-up. Two months later, a second liver biopsy was performed and revealed non-suppurative destructive chronic cholangitis, extensive areas of confluent necrosis with hepatocytes regenerating into pseudorosettes, and numerous plasma cells. According to the Paris Criteria, the patient was then diagnosed with an autoimmune hepatitis-primary biliary cholangitis overlap syndrome (AIH-PBC-OS). After adding azathioprine and ursodeoxycholic acid to the treatment, there was a satisfactory response. This is the second worldwide report of an AIH-PBC-OS triggered by COVID-19, but the first case with a negative anti-mitochondrial antibody. In this setting, histologic evaluation of the liver by an experienced pathologist is a hallmark of achieving the diagnosis and correctly treat the patient.

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Section: Discussionmentioning

confidence: 96%

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

et al. 2023

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“…In recent years, since the start of the COVID-19 pandemic, cases of SARS-CoV-2-induced secondary HLH during and post-infection have been sparsely reported [10][11][12][13][14][15]. In these case reports, the cause of HLH has been postulated to be secondary to subclinical inflammation and dysregulation of the immune system in patients of a wide age range with PCR-proven recovery [15][16][17].…”

Section: Discussionmentioning

confidence: 99%

Coronavirus Does It Again: Post-COVID-19 Hemophagocytic Lymphohistiocytosis (HLH)

Jeyakanthan¹,

Ladel²,

Khandpur³

et al. 2023

Cureus

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Hemophagocytic lymphohistiocytosis (HLH) is a hematological disorder that results from an uncontrolled activation of the immune system, which can then lead to multisystem organ failure. Given the nonspecific nature of this illness, it can go undetected for too long, thereby causing permanent damage to organ systems. In adults, HLH has been associated with a number of infectious etiologies, particularly viral infections. Coronavirus disease 2019 (COVID-19), caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), has led to a global pandemic and has been associated with acute respiratory distress syndrome (ARDS). Among its other manifestations, COVID-19 has also been linked to HLH. In this report, we describe a case of a male patient who presented with multisystem organ failure and was found to have HLH. Since no clear etiology for his HLH could be elicited, it was determined to be a result of his recent COVID-19 infection.

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“…В качестве основной причины такого несоответствия критериям выделяют неполноценное обследование (например, трепанобиопсия костного мозга, пункционная биопсия печени была доступна к проведению только в 30 % случаев) [17]. В связи с этим ряд авторов рекомендует воздержаться от применения H-score в диагностике ГЛГ у пациентов после перенесенной инфекции COVID-19 ввиду недостаточной чувствительности метода и в первую очередь опираться на клиническую картину [13,17,25].…”

Section: Discussionunclassified

Patient with Jaundice, Dyspnea and Hyperferritinemia after COVID-19

Grechishnikova

Tkachenko

Zharkova

et al. 2022

Rossijskij žurnal gastroènterologii, gepatologii, koloproktolog

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The aim: to highlight the importance of considering hemophagocytic lymphohistiocytosis in patients with jaundice of unclear origin and systemic inflammatory manifestations after coronavirus infection.Key points. A 64-y.o. patient was admitted to the hospital with jaundice, pruritus, fatigue, weight loss. The complaints occurred 2 weeks after discharge from the hospital for treatment of patients with coronavirus infection. Laboratory tests revealed signs of hepatic insufficiency, markers of cholestasis and inflammation persisted in time. Upon instrumental examination no signs of hepatosplenomegaly, biliary tree changes, intra- and extrahepatic obstruction were found. S. aureus was identified in blood cultures, CT scan of the facial skull bones showcased the focus of infection in the area of the roots of teeth 2.4 and 2.5. Therefore, antibiotics were prescribed. Subsequently, the patient's condition was complicated by the development of two episodes of acute respiratory distress syndrome, which occurred during the withdrawal of glucocorticosteroid therapy. Liver biopsy was performed, morphological study revealed signs of “vanishing bile duct” syndrome, excessive activation of macrophages and hemosiderosis of sinusoidal cells. Identified lesions can be found in hemophagocytic lymphohistiocytosis (HLH), a life-threatening complication of coronavirus infection. Glucocorticosteroids therapy, transfusions of human immunoglobulin, albumin, and parenteral nutrition have led to patient's condition improvement.Conclusion. COVID-19 provokes the development of secondary HLH 10 times more often than other respiratory viral infections. The possibility of hemophagocytic syndrome development should be considered, including cases of overlap syndrome with sepsis, in patients with unresolved jaundice, hyperferritinemia after coronavirus infection. Routinely used scales and criteria for diagnosis of HLH (H-score, HLH 2004) in such cases lacks sensitivity, therefore, careful analysis of clinical picture and exclusion of other causes of jaundice are required.

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Is COVID-19-associated cytokine storm distinct from non-COVID-19 secondary hemophagocytic lymphohistiocytosis?

Cited by 12 publications

References 48 publications

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

Autoimmune hepatitis and primary biliary cholangitis overlap syndrome after COVID-19

Coronavirus Does It Again: Post-COVID-19 Hemophagocytic Lymphohistiocytosis (HLH)

Patient with Jaundice, Dyspnea and Hyperferritinemia after COVID-19

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