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Am J Perinatol 2011; 28(5): 337-346
DOI: 10.1055/s-0030-1268710
© Thieme Medical Publishers

Neonatal Enteroviral Sepsis/Meningoencephalitis and Hemophagocytic Lymphohistiocytosis: Diagnostic Challenges

Kristen Earley Lindamood1 [*] , Patricia Fleck1 [*] , Anupama Narla2 , Jo-Anne Vergilio3 , Barbara A. Degar2 , Michael Baldwin4 , Pia Wintermark1 , 4 , 5
  • 1Division of Newborn Medicine, Children's Hospital Boston, Boston, Massachusetts
  • 2Division of Pediatric Hematology Oncology, Children's Hospital Boston, Boston, Massachusetts
  • 3Department of Pathology, Children's Hospital Boston, Boston, Massachusetts
  • 4Department of Radiology, Children's Hospital Boston, Boston, Massachusetts
  • 5Division of Newborn Medicine, Department of Pediatrics, Montreal Children's Hospital, Montreal, Canada
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Publication History

Publication Date:
18 November 2010 (online)

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ABSTRACT

We present the clinical course of three neonates with proven enteroviral infection and an initial clinical picture suggestive of hemophagocytic lymphohistiocytosis (HLH). After a complete workup, only one was treated for HLH. Of particular interest, the first newborn presented with hemophagocytic cells in the cerebrospinal fluid (CSF) and proved to have enteroviral meningoencephalitis but was ultimately not diagnosed with HLH. A fourth infant, who fulfilled the diagnostic criteria for HLH but did not have enteroviral infection, is included for comparison. We suggest that severe neonatal enteroviral infection and HLH are difficult to distinguish. Careful assessment is recommended, as prognosis and treatment differ between these two entities. Literature regarding neonatal enteroviral infection and HLH is reviewed, to demonstrate the continuum between the inflammation triggered by enteroviral infection and the occurrence of HLH, as well as their comparable CSF findings.

KEYWORDS

Newborn - hemophagocytic lymphohistiocytosis - enteroviral sepsis - enteroviral meningoencephalitis

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1 These authors contributed equally to this manuscript.

Pia WintermarkM.D. 

Division of Newborn Medicine, Montreal Children's Hospital

2300 rue Tupper, C-920, Montréal, QC H3H 1P3, Canada

Email: Pia.Wintermark@bluemail.ch