Article
Hemophagocytic lymphohistiocytosis and congenital factor VII deficiency: a case report.
BMC medical genetics - 12 Sept 2018
Wang Xiong, Tang Ning, Chang Wei, Lu Yanjun, Li Dengju
Abstract excerpt
BACKGROUND: Hemophagocytic lymfohistiocytosis (HLH) is a rare, life-threatening hyperinflammation, characterized by immune system over-activation resulting in hemophagocytosis. HLH could appear as a primary disease caused by mutations of immune-regulatory genes, or develop as a result of viral or bacterial infections, or malignancy. Congenital factor VII (FVII) deficiency is a rare autosomal recessive disorder...
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