Autoimmune hepatitis following allogeneic PBSCT from an HLA-matched sibling

Abstract

Summary:A 7-year-old boy with acute lymphoblastic leukemia (ALL) in second remission received an allogeneic PBSCT from his HLA-matched sister. Acute grade II graftversus-host disease (GVHD) resolved with corticosteroids. Chronic GVHD in the skin and oral mucosa at around day 60 responded to corticosteroids and cyclosporin A. At 6 months after the transplant, he developed hepatic dysfunction with elevated serum transaminases and gamma-globulin. Liver biopsy revealed chronic inflammation with lymphocytes and plasma cells in portal areas without destruction of bile ducts, suggesting autoimmune hepatitis. While rare, autoimmune hepatitis should be considered a potential long-term complication in patients with hepatic dysfunction in the late post-transplant phase.

Autoimmune-like hepatitis following unrelated BMT successfully treated with rituximab

“…We summarized occasional examples of de novo autoimmune-like hepatitis following allo-SCT in which the target liver cells and effector cells are different in origin (Table 1). [2][3][4][5]8,9 Habib et al used the term 'alloimmune hepatitis' to describe this entity. The laboratory and histological findings and clinical course of our patient were very similar to these cases and she may be included in the same disease entity.…”

Alloimmune hepatitis following peripheral stem cell transplantation

“…1 Occasional examples of de novo autoimmune-like hepatitis have been reported following liver, bone marrow or stem cell transplantation. [3][4][5][6][7][8] Other etiologies including viral hepatitis, non-alcoholic steatohepatitis and metabolic liver disease were excluded. The diagnosis was distinguished from chronic GVHD as liver histopathology did not reveal any changes consistent with GVHD (Table 2).…”

Acute hepatic failure as onset of progressive sclerodermatous chronic graft-versus-host disease after donor lymphocyte infusion

“…6 A Th2 polarization with autoimmune aspects has been observed in some cases. [5][6][7][8] In our case, a Th2-type reaction could have been the trigger for hepatic and sclerodermic flare or a Th2 response could have been followed by the shift toward a Th1 response. The first immunosuppressive therapy could have stopped the Th2 reaction but not Th1 and/or the flow of cytokines and chemokines, which activated fibrosis through TGF-b.…”

“…[1][2][3] Unusual presentations of liver GVHD, such as the hepatitic variant or autoimmune hepatitis, have been reported in literature. [3][4][5] They can occur during the tapering of immunosuppressive therapy or off therapy. 3,5 The hepatitic variant seems to be more frequent after DLI than the classical cholestatic variant.…”

“…[3][4][5] They can occur during the tapering of immunosuppressive therapy or off therapy. 3,5 The hepatitic variant seems to be more frequent after DLI than the classical cholestatic variant. 3 Chronic GVHD rarely causes hepatic failure, which occurs only in longterm survivors after the development of cirrhosis.…”