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LYMPHOHISTIOCYTOSE HEMOPHAGOCYTAIRE FAMILIALE [1 record]
Record 1 - internal organization data 2009-09-25
Record 1, English
Record 1, Subject field(s)
- Human Diseases - Various
- Blood
Record 1, Main entry term, English
- primary hemophagocytic lymphohistiocytosis
1, record 1, English, primary%20hemophagocytic%20lymphohistiocytosis
correct
Record 1, Abbreviations, English
Record 1, Synonyms, English
- familial hemophagocytic lymphohistiocytosis 2, record 1, English, familial%20hemophagocytic%20lymphohistiocytosis
correct
- FHLH 2, record 1, English, FHLH
correct
- FHLH 2, record 1, English, FHLH
- familial HLH 2, record 1, English, familial%20HLH
correct
- primary HLH 3, record 1, English, primary%20HLH
correct
Record 1, Textual support, English
Record number: 1, Textual support number: 1 CONT
Primary hemophagocytic lymphohistiocytosis [HLH] ... is a heterogeneous autosomal recessive disorder found to be more prevalent with parental consanguinity. [Like secondary hemophagocytic lymphohistiocytosis, it is] characterized by the overwhelming activation of normal T lymphocytes and macrophages, invariably leading to clinical and hematologic alterations and death in the absence of treatment. 1, record 1, English, - primary%20hemophagocytic%20lymphohistiocytosis
Record 1, French
Record 1, Domaine(s)
- Maladies humaines diverses
- Sang
Record 1, Main entry term, French
- lymphohistiocytose hémophagocytaire familiale
1, record 1, French, lymphohistiocytose%20h%C3%A9mophagocytaire%20familiale
correct, feminine noun
Record 1, Abbreviations, French
- LHF 1, record 1, French, LHF
correct, feminine noun
Record 1, Synonyms, French
- lymphohistiocytose hémophagocytaire primitive 2, record 1, French, lymphohistiocytose%20h%C3%A9mophagocytaire%20primitive
correct, feminine noun, less frequent
Record 1, Textual support, French
Record number: 1, Textual support number: 1 CONT
Lymphohistiocytose hémophagocytaire familiale. Cette affection autosomique récessive touche un enfant sur 50 000. Elle se traduit par l'apparition, chez de très jeunes enfants, d'une fièvre associée à une hépatosplénomégalie, une pancytopénie, une hypertriglycéridémie, une hypofibrinogènémie et des troubles neurologiques sévères. La concentration sérique de cytokines pro-inflammatoires [...] est augmentée. Une caractéristique histologique de l'affection est l'accumulation de macrophages et de lymphocytes activés [...], avec images d'hémophagocytose dans la moelle osseuse, la rate, le foie, les ganglions et le système nerveux central. 1, record 1, French, - lymphohistiocytose%20h%C3%A9mophagocytaire%20familiale
Record 1, Spanish
Record 1, Textual support, Spanish
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