OP0017 Blurring of the Distinction Between Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome

R Q Cron,M Zhang

doi:10.1136/annrheumdis-2013-eular.222

OP0017 Blurring of the Distinction Between Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome

R Q Cron, M Zhang

https://doi.org/10.1136/annrheumdis-2013-eular.222

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Journal: Annals of the Rheumatic Diseases

Publication Date: Jun 1, 2013

#Macrophage Activation Syndrome #NK Cell Lytic Activity #HLH Gene #HLH Genes #Intracellular Perforin #Macrophage Activation Syndrome Patients #Annu Rev Med #Lentiviral Expression Vectors #Canakinumab Use #Specific Gene Mutations

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BackgroundHemophagocytic lymphohistiocytosis (HLH) is an often fatal disorder of infancy resulting from homozygous mutations in proteins involved in cytolysis (e.g. MUNC13-4, RAB27a, Perforin 1, Syntaxin11, STXBP2) [1]. HLH treatment is...

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OP0017 Blurring of the Distinction Between Hemophagocytic Lymphohistiocytosis and Macrophage Activation Syndrome