title: Use of recombinant thrombomodulin in disseminated intravascular coagulation complicated hemophagocytic lymphohistiocytosis.
abstract: 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.
authors.affiliation: Department of Pediatrics, Sapporo Medical University School of Medicine, S1W16 Chuo-ku, Sapporo, 060-8543, Japan, ymasaki@sapmed.ac.jp.
authors.forename: Masaki
authors.initials: M
authors.lastname: Yamamoto
authors.forename: Tsukasa
authors.initials: T
authors.lastname: Hori
authors.forename: Naoki
authors.initials: N
authors.lastname: Hatakeyama
authors.forename: Keita
authors.initials: K
authors.lastname: Igarashi
authors.forename: Natsuko
authors.initials: N
authors.lastname: Inazawa
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Tsutsumi
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Suzuki
mesh_terms: D004211:Disseminated Intravascular Coagulation / Q000188:drug therapy* / Q000209:etiology; D020031:Epstein-Barr Virus Infections / Q000150:complications; D005260:Female; D006801:Humans; D007223:Infant; D051359:Lymphohistiocytosis, Hemophagocytic / Q000150:complications*; D011994:Recombinant Proteins / Q000627:therapeutic use; D018180:Thrombomodulin / Q000627:therapeutic use*; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D018180:Thrombomodulin
references.citation: Br J Haematol. 2010 Jun;149(6):911-2
references.pmid: 20346017
references.citation: Haematologica. 2004 Feb;89(2):183-8
references.pmid: 15003893
references.citation: Thromb Haemost. 1999 Aug;82(2):718-21
references.pmid: 10605773
references.citation: Blood. 1999 Mar 15;93(6):1869-74
references.pmid: 10068659
references.citation: Pediatr Blood Cancer. 2007 Feb;48(2):124-31
references.pmid: 16937360
references.citation: Pediatr Crit Care Med. 2009 May;10(3):285-90
references.pmid: 19433941
references.citation: J Pediatr Hematol Oncol. 2011 Jan;33(1):35-9
references.pmid: 21088619
references.citation: J Thromb Haemost. 2007 Jan;5(1):31-41
references.pmid: 17059423
references.citation: Int J Hematol. 2007 Jul;86(1):58-65
references.pmid: 17675268