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Inherited ADAMTS13 deficiency: Unique presentation and treatment
Author(s) -
Born Hayley,
Peters Antoinette,
Ettinger Robert
Publication year - 2008
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.21474
Subject(s) - medicine , adamts13 , microangiopathic hemolytic anemia , fresh frozen plasma , therapeutic plasma exchange , presentation (obstetrics) , pediatrics , intensive care medicine , surgery , thrombotic thrombocytopenic purpura , platelet
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 relapse. It may be reasonable to consider observational and reactive care rather than prophylactic care in some cases of inherited ADAMTS13 deficiency. Pediatr Blood Cancer 2008;50:956–957. © 2008 Wiley‐Liss, Inc.