Primary haemophagocytic syndrome in a young girl.
Article
in English
| IMSEAR
| ID: sea-139168
ABSTRACT
Haemophagocytic syndrome is a life-threatening systemic illness characterized by an uncontrolled inflammatory response. Patients present with fever, hepatosplenomegaly, jaundice and liver dysfunction, neurological manifestations and often pancytopenia. Bone marrow, lymph node, hepatic or splenic biopsy shows macrophages with ingested blood cells or their precursors. Laboratory markers include elevated triglycerides and ferritin, low fibrinogen with normal or low erythrocyte sedimentation rate (ESR). Familial haemophagocytic lymphohistiocytosis (HLH) is an autosomal recessive disorder. Secondary haemophagocytic syndrome results from infections, malignancy and collagen vascular disorders. We describe a young girl with primary haemophagocytic syndrome.
Full text:
Available
Index:
IMSEAR (South-East Asia)
Main subject:
Female
/
Humans
/
Dexamethasone
/
Amphotericin B
/
Cyclosporine
/
Fatal Outcome
/
Adult
/
Drug Therapy, Combination
/
Lymphohistiocytosis, Hemophagocytic
/
Young Adult
Language:
English
Year:
2011
Type:
Article
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