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Title of Journal: Eur J Pediatr

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Abbravation: European Journal of Pediatrics

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Springer-Verlag

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DOI

10.1002/chin.198912035

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1432-1076

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Simultaneous manifestation of fulminant infectious

Authors: Boris Hügle Itziar Astigarraga JanInge Henter Anna PorwitMacDonald Alfons Meindl Volker Schuster
Publish Date: 2006/10/21
Volume: 166, Issue: 6, Pages: 589-593
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Abstract

Xlinked lymphoproliferative disease is a rare T and NK cell immune deficiency which most frequently presents as fulminant infectious mononucleosis following infection with the EpsteinBarr virus EBV We report the case of a 4yearold boy from a Spanish family presenting with severe infectious mononucleosis In the course of the disease he developed hepatic failure pancytopenia and neurologic impairment leading to death after less than 2 months The results of bone marrow biopsy and autopsy indicated a histological diagnosis of both highgrade Bcell lymphoma and virusassociated haemophagocytic syndrome thereby confirming the simultaneous presence of two different manifestations of Xlinked lymphoproliferative disease XLP in this patient The family history revealed four close male relatives dying under similar circumstances one of whom died following a vaccination against measles Molecular genetic studies identified a novel mutation in the SH2D1A gene in several members of the family establishing the diagnosis of XLP Fatal EBV infection in male infants is highly indicative of XLP Virusassociated haemophagocytic syndrome and Bcell lymphoma can occur concomitantly and may be difficult to distinguish due to their similar histological pictures

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