Article
Familial haemophagocytosis lymphohisticytosis type 3: A case report.
Archives de pediatrie : organe officiel de la Societe francaise de pediatrie - 1 Jan 2017
Kamoun F, Hsairi M, Grandin V, Ben Ameur S, De Saint Basile G, Hachicha M
Abstract excerpt
Familial hemophagocytic lymphohistiocytosis (FHL) is a rare autosomal recessive disorder of immune regulation. Here, we report on a fatal case of type 3 FHL (FHL3) in a 45-day-old boy. Clinically, the infant presented with fever and hepatosplenomegaly. Biology showed pancytopenia, elevated ferritin, and decreased fibrinogen. Images of hemophagocytosis were found at the bone morrow examination. The diagnosis of...
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