title: Low ADAMTS-13 activity during hemorrhagic events with disseminated intravascular coagulation.
abstract: Disseminated intravascular coagulation (DIC) is a life-threatening complication, and its control is essential for therapeutic success. Recombinant human soluble thrombomodulin alfa (rTM) is a novel therapeutic agent for DIC. The efficacy of rTM in the treatment of DIC is reportedly superior to that of conventional anti-DIC treatments, such as unfractionated heparin or low molecular weight heparin, but hemorrhagic events occasionally interfere with the therapeutic benefits of rTM. We assessed the clinical features of 20 consecutive patients who were given rTM for DIC associated with various hematologic disorders. Eight patients achieved remission of both primary disease and DIC, eight died due to progression of the primary disease, and four died of various hemorrhagic complications. Assessment of 16 biomarkers for coagulation showed that the four patients who died of hemorrhagic complications despite remission of their primary disease showed lower ADAMTS-13 (a disintegrin and metalloproteinase with a thrombospondin Type 1 motif, member 13) plasma activity than other patients (P = 0.016). The optimal cut-off level of ADAMTS-13 for predicting risk of hemorrhagic complications was 42 % (P = 0.007). Plasma ADAMTS-13 activity determined at diagnosis of DIC may help predict the risk of hemorrhagic events during and/or following DIC treatment with hematologic disorders.
authors.affiliation: Division of Hematology and Oncology, Department of Medicine, Kyoto Prefectural University of Medicine, 465 Kajii-cho, Kamigyo-ku, Kyoto, Japan.
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Chinen
authors.forename: Junya
authors.initials: J
authors.lastname: Kuroda
authors.forename: Muneo
authors.initials: M
authors.lastname: Ohshiro
authors.forename: Yuji
authors.initials: Y
authors.lastname: Shimura
authors.forename: Shinsuke
authors.initials: S
authors.lastname: Mizutani
authors.forename: Hisao
authors.initials: H
authors.lastname: Nagoshi
authors.forename: Nana
authors.initials: N
authors.lastname: Sasaki
authors.forename: Ryuko
authors.initials: R
authors.lastname: Nakayama
authors.forename: Miki
authors.initials: M
authors.lastname: Kiyota
authors.forename: Mio
authors.initials: M
authors.lastname: Yamamoto-Sugitani
authors.forename: Tsutomu
authors.initials: T
authors.lastname: Kobayashi
authors.forename: Yosuke
authors.initials: Y
authors.lastname: Matsumoto
authors.forename: Shigeo
authors.initials: S
authors.lastname: Horiike
authors.forename: Masafumi
authors.initials: M
authors.lastname: Taniwaki
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000368:Aged; D000369:Aged, 80 and over; D001777:Blood Coagulation; D004211:Disseminated Intravascular Coagulation / Q000097:blood* / Q000150:complications* / Q000175:diagnosis / Q000188:drug therapy; D004789:Enzyme Activation; D017809:Fatal Outcome; D005260:Female; D006470:Hemorrhage / Q000209:etiology*; D006801:Humans; D008297:Male; D008875:Middle Aged; D011994:Recombinant Proteins / Q000627:therapeutic use; D018180:Thrombomodulin / Q000627:therapeutic use; D016896:Treatment Outcome
chemical_list: D011994:Recombinant Proteins; D018180:Thrombomodulin; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human