Percorsi clinici
L’EBV che non mi aspetto: MASe ci penso...!
The Epstein-Barr virus that I do not expect: MAS… if I just think about it!
Alberto Di Mascio*, Caterina Redice, Sara Pusceddu, Vittoria Camelli, Federico Marchetti
UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna
*Università di Trieste
Dicembre 2016 - pagg. 637 -640
Abstract
The paper reports the case of a 14-year-old boy who presented to our attention because
for three days he had fever, laterocervical lymphadenopathy with limited movements of
the neck and cytopoenia. The clinical diagnostic process led to the diagnosis of Epstein-
Barr Virus haemophagocytic lymphohistiocytosis (HLH). In the presented case the strong
diagnostic suspicion was mostly driven by the presence of high ferritin level. HLH is a
life-threatening hyperinflammatory syndrome that is classified into primary and secondary
HLH. Both primary and secondary HLH are characterized by uncontrolled hypercytokinemia
that results in myelosuppression and vascular endothelium damage. The Epstein-
Barr virus (EBV) is the most common cause of secondary HLH. In male patients who
develop HLH during a first EBV infection the evaluation of the function of certain proteins
involved on immune response (SAP, XIAP, and perforin) and function of Natural Killer
(NK) cells are essential to exclude primitive forms (X-linked lymphoproliferative disorder -
XLP), which would benefit from a specific monoclonal antibody therapy (rituximab).
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Classificazione MeSH
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Bibliografia
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Corrispondenza: federico.marchetti@auslromagna.it