Relevance of ADAMTS13 to liver transplantation and surgery

Figure 2 Clinical course of 27-year-old male with Budd-Chiari syndrome after liver transplantation. Serum ALT level mildly increased on days 1 and 2 because of ischemia-reperfusion injury, decreased thereafter, but rapidly increased again on day 7 due to acute rejection (left panel). The platelet count decreased gradually and reached a nadir on day 7, when ADAMTS13 activity decreased markedly to less than 3% from 108% before surgery. After the administration of fresh frozen plasma and bolus injection of methylprednisolone (arrow), ALT level decreased and the platelet count gradually increased. The ADAMTS13 activity increased to 22% on day 14. After the first episode of acute rejection, VWF:Ag increased further and reached 368% on day 21, when ALT again increased due to a second episode of acute rejection. Bolus injection of methylprednisolone (arrow) led to a rapid decrease of ALT and a gradual increase in the platelet count. VWF:Ag decreased gradually, and ADAMTS13 activity finally recovered to 50% until day 98. Plasma UL-VWFM was detectable on day 1 at the time of ischemia-reperfusion injury, thereafter diminishing gradually during days 2 to 4, and again becoming evident on day 7 when acute rejection developed (right panel). The UL-VWFM disappeared transiently on day 9, but reappeared on day 11, coinciding with a mild increase of transaminase. UL-VWFM tended to diminish on day 15, but again became prominent on day 22 during the second episode of acute rejection. Pre: Before transplantation; NP: Normal plasma control; ALT: Alanine transaminase; LDH: Lactate dehydrogenase; FFP: Fresh frozen plasma; VWF: von Willebrand factor; UL-VWFM: Unusually large VWF multimers; ADAMTS13: A disintegrin-like and metalloproteinase with thrombospondin type-1 motifs 13.