Decreased ADAMTS13 levels in patients after living donor liver transplantation.
BACKGROUND
Thrombotic microangiopathy (TMA) is a complication occurring after liver transplantation (LT), and an unusually large multimer (ULM) of Von Willebrand factor (VWF) and ADAMTS13 may play an important role in the onset of TMA during LT.


METHODS
Eight-one patients underwent living donor LT (LDLT). Seventeen of those patients had both severe thrombocytopenia and hemolytic anemia with fragmented red cells and were diagnosed as TMA- like syndrome (TMALS).


CONCLUSIONS
A significant reduction of ADAMTS13 and an increase of VWF were observed in the patients with TMALS. The ADAMTS13 activity in patients after LDLT was significantly reduced from day 1 to day 21, and it was significantly low in those with TMALS at day 14 and 28. The VWF levels in patients with LDLT were significantly high, and the VWF/ADAMTS13 ratio was significantly increased in patients at 7, 14 and 28 days after LDLT, especially in patients with TMALS at day 14 and 28 after LDLT. High molecular weight multimers of VWF were observed to have increased in patients with LDLT, and the high molecular weight multimers of VWF were further increased in those with mild TMALS but they decreased in those with severe TMA. These findings suggest that ULM- VWF and ADAMTS13 might be associated with the onset of TMA after LT.
Kobayashi
Toshihiko
T
Departments of Hematology, Mie University Graduate School of Medicine, Mie, Japan.
Wada
Hideo
H
Usui
Masanobu
M
Sakurai
Hiroyuki
H
Matsumoto
Takeshi
T
Nobori
Tsutomu
T
Katayama
Naoyuki
N
Uemoto
Sinji
S
Ishizashi
Hiromichi
H
Matsumoto
Masanori
M
Fujimura
Yoshihiro
Y
Isaji
Shuji
S
D051722:ADAM Proteins / Q000097:blood*; D000071120:ADAMTS13 Protein; D000368:Aged; D002648:Child; D005260:Female; D006801:Humans; D016031:Liver Transplantation / Q000009:adverse effects*; D008297:Male; D008875:Middle Aged; D057049:Thrombotic Microangiopathies / Q000097:blood* / Q000209:etiology*; D014841:von Willebrand Factor / Q000378:metabolism
D014841:von Willebrand Factor; D051722:ADAM Proteins; D000071120:ADAMTS13 Protein; C099604:ADAMTS13 protein, human