Abstract
Hemophagocytic lymphohistiocytosis is a rare and fatal disorder of early infancy,
which affects predominantly the mononuclear phagocyte system and is characterized
by the presence of fever, hepatosplenomegaly and cytopenia. Neurological symptoms
can be extremely variable, ranging from irritability, and convulsions to focal neurological
signs. They often develop during disease progression, but can also be the leading
initial symptoms. Early diagnosis is mandatory, because new treatments, including
bone marrow transplantation, appear to be promising. Here we present the clinical,
neuroradiological and histopathological findings from two children with progressive
CNS disease as the main clinical manifestation of hemophagocytic lymphohistiocytosis.
Both children died and diagnosis was only obtained in retrospect after careful review
of the histopathological material.
Key words
Hemophagocytic lymphohistiocytosis - children - neurological symptoms
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Kevin Rostasy
Pediatric Neurology · University of Göttingen
Robert-Koch-Straße 40
37075 Göttingen
Germany
Email: krostasy@excite.com
