A Successful Liver Transplantation for Refractory Hepatic Veno‐Occlusive Disease Originating from Cord Blood Transplantation

Abstract

An 11-month-old boy with acute lymphoblastic leukemia (ALL) underwent umbilical cord blood transplantation (CBT) from an unrelated donor after a first complete remission. Despite the prophylactic use of low molecular weight heparin, prostaglandin E1 and ursodeoxycholic acid, hepatic veno-occlusive disease (VOD) occurred on the 29th day after CBT. Furthermore, neither defibrotide nor antithrombin-III improved the hepatic coma and coagulopathy due to the hepatic VOD. On the 42nd day after CBT, he underwent living related liver transplantation (LRLT) with a left lateral segment graft from his father. He received tacrolimus for the prevention of rejection and graft-vs.-host disease (GVHD) and also received aggressive antifungal and antiviral prophylaxis. Although he showed signs of acute rejection on postoperative days 5 and 10, the postoperative course was uneventful in general. At present, 17 months after LRLT, the patient shows stable liver function and no signs of either GVHD or a relapse of ALL. In conclusion, LRLT can be seen as a feasible option for the treatment of a hepatic VOD after CBT, though aggressive prophylaxis for infection and the anticipation of acute rejection are of importance.

Review article: updates in the pathogenesis and therapy of hepatic sinusoidal obstruction syndrome

“…Liver transplantation has been reported as a treatment of HSOS 172–174 . However, it should be considered only in patients with severe liver failure who are expected to have a good outcome in the absence of liver disease, and those who have undergone bone marrow transplantation for benign disease.…”

Sinusoidal Obstruction Syndrome (Hepatic Veno-Occlusive Disease)

“…[129][130][131] However, it should be considered only in patients with severe liver failure who are expected to have a good outcome in the absence of liver disease, and those who have undergone bone marrow transplantation for benign disease. Liver transplantation is usually contraindicated when malignancy is present because of the high rates of recurrence.…”

Two difficulty diagnosis cases of severe veno-occlusive disease

“…These events have been associated with abnormalities in factors such as VII, protein C levels, and plasminogen activator inhibitor. These findings indicate administration of infusion of low-dose heparin, recombinant tissue plasminogen activator (t-PA), and prostaglandin E1 (PGE1) as innovative therapies [16–18]. However, patients with severe SOS fail to respond to these therapies.…”