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DOI: 10.1055/s-2004-815791
Georg Thieme Verlag Stuttgart · New York
CNS Disease as the Main Manifestation of Hemophagocytic Lymphohistiocytosis in Two Children
Publication History
Received: July 22, 2003
Accepted after Revision: November 30, 2003
Publication Date:
04 March 2004 (online)
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