Author(s):
Taipa, R. 
; Moreira, B. ; França, M. ; Maia, L.
Date: 2010
Persistent ID: http://hdl.handle.net/10400.16/717
Origin: Repositório Científico do Centro Hospitalar do Porto
Document details
A case of haemophagocytic syndrome presenting with oculogyric crises
Description
Haemophagocytic lymphohistiocytosis (HLH), also called haemophagocytic syndrome (HPS), is a rare disorder resulting in abnormal proliferation of histiocytes in tissues and organs, including the CNS. HLH can present as a primary disease or occur as a secondary reactive disease. Clinical features are high fever, splenomegaly, cytopenia of two or more cell lines, hypertriglyceridaemia and haemophagocytosis. CNS involvement varies between 10% and 73%, and clinical manifestations include seizures, decreased sensorium, brainstem symptoms, ataxia or demyelinating peripheral neuropathy.
Haemophagocytic lymphohistiocytosis (HLH), also called haemophagocytic syndrome (HPS), is a rare disorder resulting in abnormal proliferation of histiocytes in tissues and organs, including the CNS. HLH can present as a primary disease or occur as a secondary reactive disease. Clinical features are high fever, splenomegaly, cytopenia of two or more cell lines, hypertriglyceridaemia and haemophagocytosis. CNS involvement varies between 10% and 73%, and clinical manifestations include seizures, decreased sensorium, brainstem symptoms, ataxia or demyelinating peripheral neuropathy.
Document Type
Article
Language English
Language English
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