Article
Mutations of the hemophagocytic lymphohistiocytosis-associated gene UNC13D in a patient with systemic juvenile idiopathic arthritis.
Arthritis and rheumatism - 1 Feb 2008
Hazen Melissa M, Woodward Amy L, Hofmann Inga, Degar Barbara A, Grom Alexei, Filipovich Alexandra H, Binstadt Bryce A
Abstract excerpt
The clinical syndromes of hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) are both characterized by dysregulated inflammation with prolonged fever, hepatosplenomegaly, coagulopathy, hematologic cytopenias, and evidence of hemophagocytosis in the bone marrow or liver. While HLH is either inherited or acquired, children with severe rheumatic diseases, most notably systemic juvenile...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.