Article
Inherited ADAMTS13 deficiency: unique presentation and treatment.
Pediatric blood & cancer - 1 May 2008
Born Hayley, Peters Antoinette, Ettinger Robert
Abstract excerpt
A 3-year-old male presented with severe thrombocytopenia and microangiopathic hemolytic anemia in conjunction with severe bilateral otitis media. After laboratory analysis, a diagnosis of inherited ADAMTS13 deficiency was proven. Rather than treating with prophylactic fresh frozen plasma, to date the patient has been successfully treated with single-donor, directed plasma infusions in response to early signs of...
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