title: Thrombotic microangiopathy-like disorder after living-donor liver transplantation: a single-center experience in Japan.
abstract: OBJECTIVE
  To investigate thrombotic microangiopathy (TMA) in liver transplantion, because TMA is an infrequent but life-threatening complication in the transplantation field.
  
  
  METHODS
  A total of 206 patients who underwent living-donor liver transplantation (LDLT) were evaluated, and the TMA-like disorder (TMALD) occurred in seven recipients.
  
  
  RESULTS
  These TMALD recipients showed poor outcomes in comparison with other 199 recipients. Although two TMALD recipients successfully recovered, the other five recipients finally died despite intensive treatments including repeated plasma exchange (PE) and re-transplantation. Histopathological analysis of liver biopsies after LDLT revealed obvious differences according to the outcomes. Qualitative analysis of antibodies against a disintegrin-like domain and metalloproteinase with thrombospondin type 1 motifs (ADAMTS-13) were negative in all patients. The fragmentation of red cells, the microhemorrhagic macules and the platelet counts were early markers for the suspicion of TMALD after LDLT. Although the absolute values of von Willebrand factor (vWF) and ADAMTS-13 did not necessarily reflect TMALD, the vWF/ADAMTS-13 ratio had a clear diagnostic value in all cases. The establishment of adequate treatments for TMALD, such as PE for ADAMTS-13 replenishment or treatments against inhibitory antibodies, must be decided according to each case.
  
  
  CONCLUSIONS
  The optimal induction of adequate therapies based on early recognition of TMALD by the reliable markers may confer a large advantage for TMALD after LDLT.
authors.affiliation: Department of Hepato-Pancreato-Biliary, Kyoto University Hospital, Kyoto 606-8507, Japan. horit@kuhp.kyoto-u.ac.jp
authors.forename: Tomohide
authors.initials: T
authors.lastname: Hori
authors.forename: Toshimi
authors.initials: T
authors.lastname: Kaido
authors.forename: Fumitaka
authors.initials: F
authors.lastname: Oike
authors.forename: Yasuhiro
authors.initials: Y
authors.lastname: Ogura
authors.forename: Kohei
authors.initials: K
authors.lastname: Ogawa
authors.forename: Yukihide
authors.initials: Y
authors.lastname: Yonekawa
authors.forename: Koichiro
authors.initials: K
authors.lastname: Hata
authors.forename: Yoshiya
authors.initials: Y
authors.lastname: Kawaguchi
authors.forename: Mikiko
authors.initials: M
authors.lastname: Ueda
authors.forename: Akira
authors.initials: A
authors.lastname: Mori
authors.forename: Hajime
authors.initials: H
authors.lastname: Segawa
authors.forename: Kimiko
authors.initials: K
authors.lastname: Yurugi
authors.forename: Yasutsugu
authors.initials: Y
authors.lastname: Takada
authors.forename: Hiroto
authors.initials: H
authors.lastname: Egawa
authors.forename: Atsushi
authors.initials: A
authors.lastname: Yoshizawa
authors.forename: Takuma
authors.initials: T
authors.lastname: Kato
authors.forename: Kanako
authors.initials: K
authors.lastname: Saito
authors.forename: Linan
authors.initials: L
authors.lastname: Wang
authors.forename: Mie
authors.initials: M
authors.lastname: Torii
authors.forename: Feng
authors.initials: F
authors.lastname: Chen
authors.forename: Ann-Marie T
authors.initials: AM
authors.lastname: Baine
authors.forename: Lindsay B
authors.initials: LB
authors.lastname: Gardner
authors.forename: Shinji
authors.initials: S
authors.lastname: Uemoto
mesh_terms: D051722:ADAM Proteins / Q000097:blood; D000071120:ADAMTS13 Protein; D000293:Adolescent; D000328:Adult; D000368:Aged; D015415:Biomarkers / Q000378:metabolism; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007564:Japan; D016031:Liver Transplantation / Q000009:adverse effects*; D019520:Living Donors; D008297:Male; D008875:Middle Aged; D011183:Postoperative Complications; D057049:Thrombotic Microangiopathies / Q000175:diagnosis / Q000209:etiology* / Q000473:pathology; D055815:Young Adult; D014841:von Willebrand Factor / Q000378:metabolism
chemical_list: D015415:Biomarkers; D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human