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

2021 ◽  
Vol 9 ◽  
Author(s):  
Seraina Prader ◽  
Nicole Ritz ◽  
Frédéric Baleydier ◽  
Maya C. Andre ◽  
Noémie Stähli ◽  
...  

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.

2011 ◽  
Vol 29 (1) ◽  
pp. 96-100
Author(s):  
Yueping Zeng ◽  
Lanqin Fu ◽  
Hongzhong Jin ◽  
Qiuning Sun ◽  
Baoxi Wang

2004 ◽  
Vol 16 (1) ◽  
pp. 56-59
Author(s):  
Hisaya Yukawa ◽  
Nobuo Kubo ◽  
Kaori Nakae ◽  
Hiroyuki Tsuji ◽  
Sachiko Fujimoto ◽  
...  

1995 ◽  
Vol 26 (1) ◽  
pp. 84-87 ◽  
Author(s):  
Edith Renoult ◽  
Bernadette Aymard ◽  
Marie-José Grégoire ◽  
Abdel Bellou ◽  
Jacques Hubert ◽  
...  

2002 ◽  
Vol 69 (2) ◽  
pp. 220-224 ◽  
Author(s):  
Maria Angela Biasolo ◽  
Arianna Calistri ◽  
Simone Cesaro ◽  
Giuseppe Gentile ◽  
Carlo Mengoli ◽  
...  

Author(s):  
Simon-Pierre Harvey-Bolduc ◽  
Benoit Guay

<p>Lymphomatoïd granulomatosis is a rare lymphoproliferative disease caused by Epstein-Barr virus described in 1972 by Liebow. The most common primary localization is the lungs. Other systems are affected occasionally such as the skin, central nervous system, renal and gastrointestinal system. Some cases have been described at the laryngeal level in immunosuppressed patients. This is a case report of supraglottic lymphomatoid granulomatosis in an immunocompetent patient.  A literature review on lymphomatoid granulomatosis will be detailed and the clinical case will be described including investigations, treatment and follow-up. The differential diagnosis of this pathology as well as the importance of histopathology will also be discussed. Several radiological imaging and histopathology slides will be presented. Emphasis will be placed on the difficulty of diagnosis given the frequent multisystem involvement and on the importance of the multidisciplinary team approach including otolaryngology, haematology-oncology, nephrology, pathology and radiology. We will end with a discussion about the case with reference to the current literature on the subject.</p>


1996 ◽  
Vol 21 (2) ◽  
pp. 123-126
Author(s):  
U. BALDARI ◽  
A. ASCARI RACCAGNI ◽  
B. CELLI ◽  
M. GIOVANNA RIGHINI

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