Postoperative thrombotic thrombocytopenic purpura in an infant: case report and literature review

Abstract Thrombotic thrombocytopenic purpura is caused by an imbalance of von Willebrand factor and its cleaving protease, which leads to the formation of microthrombi in end-organs. It rarely occurs in the pediatric population. Plasma exchange can significantly reduce mortality and morbidity. We pr...

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Bibliographic Details
Published in:Journal of pediatric surgery 2011-04, Vol.46 (4), p.764-766
Main Authors: Schiller, Ofer, Ash, Shifra, Schonfeld, Tommy, Kadmon, Gili, Nahum, Elhanan, Yacobovich, Joanne, Tamary, Hannah, Davidovits, Miriam
Format: Article
Language:English
Subjects:
Endodermal Sinus Tumor - surgery
Female
Hemolytic anemia
Hemolytic uremic syndrome
Humans
Infant
Laparotomy - adverse effects
Pediatrics
Pelvic Neoplasms - surgery
Plasma Exchange - methods
Plasmapheresis - methods
Postoperative
Postoperative Complications
Purpura, Thrombotic Thrombocytopenic - blood
Purpura, Thrombotic Thrombocytopenic - etiology
Purpura, Thrombotic Thrombocytopenic - therapy
Surgery
Thrombotic microangiopathy
Thrombotic thrombocytopenic purpura
von Willebrand Factor - metabolism
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Summary:Abstract Thrombotic thrombocytopenic purpura is caused by an imbalance of von Willebrand factor and its cleaving protease, which leads to the formation of microthrombi in end-organs. It rarely occurs in the pediatric population. Plasma exchange can significantly reduce mortality and morbidity. We present a 14-month-old infant in whom clinical and laboratory abnormalities compatible with thrombotic thrombocytopenic purpura were noted several days after resection of a large pelvic tumor. Treatment with double volume plasma exchange on postoperative day 5 led to complete resolution of the renal failure, thrombocytopenia, anemia, and neurological manifestations. ADAMTS13 inhibitors were negative and no mutations were found in factor H, factor I, membrane cofactor protein, and thrombomodulin to account for genetic predisposition to thrombotic thrombocytopenic purpura or atypical hemolytic uremic syndrome. Postoperative anemia, thrombocytopenia, fever, and neurological deficits in children should raise the suspicion of thrombotic thrombocytopenic purpura. Early diagnosis is important because the disorder is readily and efficiently treated with plasma exchange.
ISSN:0022-3468
1531-5037
DOI:10.1016/j.jpedsurg.2011.01.009