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Indian J Pediatr. 2014 Mar;81(3):288-91. doi: 10.1007/s12098-013-1145-1. Epub 2013 Aug 3.

Use of recombinant thrombomodulin in disseminated intravascular coagulation complicated hemophagocytic lymphohistiocytosis.

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  • 1Department of Pediatrics, Sapporo Medical University School of Medicine, S1W16 Chuo-ku, Sapporo, 060-8543, Japan,


Hemophagocytic lymphohistiocytosis (HLH) is frequently lethal in its early phase due to complicating disseminated intravascular coagulation (DIC). The authors report a 14-mo-old girl with severe DIC complicating Epstein-Barr virus associated HLH. She was successfully treated with immunochemotherapy consisting mainly of etoposide and additional recombinant thrombomodulin (r-TM), a newly developed anticoagulant. Although the efficacy of r-TM cannot be proven in a single case report, additional anticoagulation therapy with r-TM is safe and may reduce early deaths in patients with DIC-complicated severe HLH. More clinical experience is required, although r-TM is currently licensed only in Japan.

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