title: Clinical features of severe acquired ADAMTS13 deficiency in thrombotic thrombocytopenic purpura: the Korean TTP registry experience.
abstract: The clinical significance of ADAMTS13 activity for response to treatment, mortality rate, recurrence, and prognosis is unclear. Therefore, we investigated the characteristics of severe ADAMTS13 deficiency and evaluated its prognostic features in Thrombotic thrombocytopenic purpura (TTP). The Korean TTP Registry includes 66 patients from 13 teaching hospitals in Korea who received the diagnosis of TTP from January 2005 to December 2008. Blood samples obtained upon admission were sent for ADAMTS13 analysis (multimer analysis by sodium dodecyl sulfate electrophoresis) to a central laboratory along with patient clinical information. Patients with severe ADAMTS13 deficiency had lower serum creatinine levels (P = 0.001) than patients with non-severe ADAMTS13 deficiency. Although severe ADAMTS13 deficiency was associated with better response rate (75 vs. 53%, P = 0.145), remission rate (81 vs. 61%, P = 0.209), and mortality rate (19 vs. 31%, P = 0.508) than non-severe ADAMTS13 deficiency, treatment outcomes did not differ significantly between groups. After adjusting for clinical and laboratory features, multivariate analysis did not reveal any independent risk factors for TTP-associated mortality. Patients with severe ADAMTS13 deficiency had lower serum creatinine levels at presentation, but severe ADAMTS13 activity deficiency at TTP diagnosis does not appear to have prognostic significance.
authors.affiliation: Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea.
authors.forename: Moon Ju
authors.initials: MJ
authors.lastname: Jang
authors.affiliation: Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea.
authors.forename: So Young
authors.initials: SY
authors.lastname: Chong
authors.affiliation: Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: In-Ho
authors.initials: IH
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Seoul National University College of Medicine, Seoul, Korea.
authors.forename: Jee-Hyun
authors.initials: JH
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Chul-Won
authors.initials: CW
authors.lastname: Jung
authors.affiliation: Department of Laboratory Medicine, College of Medicine, Catholic University of Korea, Seoul, Korea.
authors.forename: Ja Young
authors.initials: JY
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, College of Medicine, Catholic University of Korea, Seoul, Korea.
authors.forename: Ji-Chan
authors.initials: JC
authors.lastname: Park
authors.affiliation: Department of Internal Medicine, Busan National University College of Medicine, Pusan, Korea.
authors.forename: Sun Min
authors.initials: SM
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, Chonnam National University College of Medicine, Gwangju, Korea.
authors.forename: Yeo-Kyeoung
authors.initials: YK
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Korea University College of Medicine, Seoul, Korea.
authors.forename: Ji-Eun
authors.initials: JE
authors.lastname: Lee
authors.affiliation: Department of Laboratory Medicine, University of Ulsan College of Medicine, Seoul, Korea.
authors.forename: Sung-Su
authors.initials: SS
authors.lastname: Jang
authors.affiliation: Department of Internal Medicine, Yonsei University College of Medicine, Seoul, Korea.
authors.forename: Jin-Seok
authors.initials: JS
authors.lastname: Kim
authors.affiliation: Department of Internal Medicine, Chungnam National University College of Medicine, Daejeon, Korea.
authors.forename: Deog-Yeon
authors.initials: DY
authors.lastname: Jo
authors.affiliation: Department of Internal Medicine, Hallym University Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Korea.
authors.forename: Dae-Young
authors.initials: DY
authors.lastname: Zang
authors.affiliation: Department of Internal Medicine, Hanyang University College of Medicine, Seoul, Korea.
authors.forename: Young-Yiul
authors.initials: YY
authors.lastname: Lee
authors.affiliation: Department of Internal Medicine, Chonbuk National University Medical School, Jeonju, Korea.
authors.forename: Ho-Young
authors.initials: HY
authors.lastname: Yhim
authors.affiliation: Department of Internal Medicine, School of Medicine, CHA University, Seongnam, Korea. doh@cha.ac.kr.
authors.forename: Doyeun
authors.initials: D
authors.lastname: Oh
mesh_terms: D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D006801:Humans; D011697:Purpura, Thrombotic Thrombocytopenic / Q000097:blood* / Q000503:physiopathology*; D012042:Registries; D056910:Republic of Korea; D012189:Retrospective Studies
chemical_list: D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human