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X-Linked Lymphoproliferative Disease Mimicking Multisystem Inflammatory Syndrome in Children-A Case Report

Most children with a SARS-CoV-2 infection are asymptomatic or exhibit mild symptoms. However, a small number of children develop features of substantial inflammation temporarily related to the COVID-19 also called multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multis...

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Main Authors: Seraina Prader (Author), Nicole Ritz (Author), Frédéric Baleydier (Author), Maya C. Andre (Author), Noémie Stähli (Author), Kevin Schmid (Author), Hanna Schmid (Author), Andreas Woerner (Author), Tamara Diesch (Author), Patrick M. Meyer Sauteur (Author), Johannes Trück (Author), Fabienne Gebistorf (Author), Lennart Opitz (Author), Michael P. Killian (Author), Tommaso Marchetti (Author), Stefano Vavassori (Author), Géraldine Blanchard-Rohner (Author), Valerie Mc Lin (Author), Serge Grazioli (Author), Jana Pachlopnik Schmid (Author)
Format: Book
Published: Frontiers Media S.A., 2021-08-01T00:00:00Z.
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Summary:Most children with a SARS-CoV-2 infection are asymptomatic or exhibit mild symptoms. However, a small number of children develop features of substantial inflammation temporarily related to the COVID-19 also called multisystem inflammatory syndrome in children (MIS-C) or pediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS), clinically similar to Kawasaki disease, toxic shock syndrome and hemophagocytic lymphohistiocytosis (HLH). It is well-known that genetic pre-disposition plays an important role in virally-triggered diseases such as Epstein-Barr virus (EBV)-associated HLH, while this has not yet been established for patients with MIS-C. Here we describe a male patient fulfilling the diagnostic criteria of MIS-C, who was initially treated according to current consensus guidelines. Presence of hypofibrinogenemia, normal lymphocyte counts and C-reactive protein, but substantial hyperferritinemia distinguish this patient from others with MIS-C. The clinical course following initial presentation with acute respiratory distress syndrome was marked by fatal liver failure in the context of EBV-associated HLH despite treatment with steroids, intravenous immunoglobulins, interleukin (IL)-1 receptor blockade and eventually HLH-directed treatment. X-linked lymphoproliferative disease type 1 (XLP1), a subtype of primary HLH was diagnosed in this patient post-mortem. This case report highlights the importance of including HLH in the differential diagnosis in MIS-C with severe disease course to allow specific, risk-adapted treatment and genetic counseling.
Item Description:2296-2360
10.3389/fped.2021.691024

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