Journal
JOURNAL OF PEDIATRIC HEMATOLOGY ONCOLOGY
Volume 33, Issue 1, Pages 74-78Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/MPH.0b013e3181fd2aae
Keywords
heme oxygenase; nephritis; hemolysis
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Heme oxygenase-1 (HO-1) is a stress-induced enzyme that catalyses the oxidation of heme to biliverdin. The primary deficiency of this enzyme has been shown in HO-1 knockout mice, and is characterized by intrauterine death and chronic inflammation. The first case of human HO-1 deficiency was reported in 1999. Human HO-1 deficiency has been observed to involve the endothelial cells more severely, resulting in hemolysis and disseminated intravascular coagulation. We report another case of human HO-1 deficiency in a young girl with congenital asplenia, who presented with severe hemolysis, inflammation, nephritis, which was refractory to therapy with corticosteroids, cyclophosphamide, and rituximab.
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