Dicembre 2016 - Volume XXXV - numero 10
Percorsi clinici
UOC di Pediatria e Neonatologia, Ospedale di Ravenna, AUSL della Romagna
*Universitŕ di Trieste
Indirizzo per corrispondenza: federico.marchetti@auslromagna.it
Key words: Epstein-Barr Virus, Haemophagocytic lymphohistiocytosis, Diagnosis
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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