Recurring Fibro-Obliterative Venopathy in Liver Allografts

Abstract

Recurrent diseases in liver allografts are not uncommon. These occur most frequently in those transplanted for viral hepatitis B and C. We report an unusual case of recurrent process in two consecutive liver allografts received by a 37-year-old woman, who previously had an unremarkable past medical history but developed a rapidly progressive cholestatic liver failure. Histopathologic examination of the native liver showed fibroocclusive lesions of both terminal hepatic venules and portal vein branches. The exuberant fibroobliterative process created dense fibrosis with whorled appearance, and broad fibrous septa connecting adjacent central areas, and sometimes bridging portal to central areas. Dense portal fibrosis resulted in compression atrophy and loss of bile ducts. The first allograft, which failed within 3 months, showed histopathologic findings similar to that of the native liver. A liver biopsy that was performed 20 months after the second liver transplant again showed similar histopathology. The histopathologic features and clinical presentation of this patient suggest an unusual form of recurring progressive fibroobliterative venopathy causing liver failure.

Significance of isolated hepatic veno-occlusive disease/sinusoidal obstruction syndrome after liver transplantation

“…In this study, the overall prevalence of VOD/SOS after LT was 2.3% (31/1364), which is statistically similar to the overall prevalence in our previous study (1.9% or 19/1023) 9. This value differs from literature values, with previous studies (even those from centers performing protocol biopsy) mainly reporting sporadic cases 2‐11. The difference is probably related to the histological description of VOD/SOS and AR when they coexist.…”

“…Despite a similar overall prevalence of VOD/SOS over the last 2 decades, the proportion of iVOD/SOS has significantly increased (15/31 versus 2/19, P = 0.006)9 and is similar to the proportion reported in the literature (10/20) 2, 3, 5, 7, 11. None of our patients received azathioprine as part of their immunosuppression.…”

“…VOD/SOS is a rare cause of liver graft dysfunction with a poor prognosis 2‐11. VOD/SOS has been reported to be related initially to azathioprine use11 and subsequently to AR.…”

Sinusoidal Obstruction Syndrome (Hepatic Veno-Occlusive Disease)

“…90 This is to be distinguished from post-transplant perivenulitis, as part of graft rejection. 91 If present, SOS is more likely to occur in living-related transplant grafts 92 or in patients receiving azothiaprine as part of their immunosuppressive regime.…”

Hepatic veno-occlusive disease after liver transplantation: an unusual case report

“…In a previous study [10], 58% of the patients (11/19) were re‐transplanted but only three survived. None of these re‐transplants was diagnosed with a recurrence of VOD, making our case an extremely rare situation, with only one previous report in the literature [14].…”