Acute Hepatitic Onset of Liver GVHD Occurring 9 Months Post-transplant

The least common presentation of liver GVHD develops as an acute hepatitic onset which develops following tapering or cessation of immunosuppressive therapy (IS) or following donor lymphocyte infusions for a graft-versus-leukemia effect. The clinical features differ from the usual hepatic GVHD, arising concurrently with skin and gut aGVHD. Instead, the clinical presentation features rapidly rising aminotransferases (>20x normal) followed by hyperbilirubinemia. The progression of changes is an initial lobular hepatitis followed by marked cholestasis and bile duct damage. The differential diagnosis of acute hepatitic onset of GVHD includes a number of other viral infections and an unusual late post-HSCT chronic inflammatory and fibrosing hepatitis with many plasma cells and damaged bile ducts without attendent GVHD or infection. Since it resembles autoimmune hepatitis, we thus propose calling this condition “chronic alloimmnune hepatitis” (“CAIH”). Sporadic cases of cirrhosis that occur 5-15 years post-HSCT and are attributed to GVHD isolated to the liver may well be a late manifestation of CAIH.