PATHOLOGY OF THE LIVER IN SEVERE COMBINED IMMUNODEFICIENCY AND DIGEORGE-SYNDROME

Review of liver biopsy or autopsy material from 33 patients with sever e combined immunodeficiency or combined immunodeficiency and four pati ents with DiGeorge syndrome revealed a wide range of hepatic pathology . The most common abnormality was graft-versus-host disease (16 patien ts), followed by viral infection (4 patients) had adenovirus hepatitis , 3 had cytomegalovirus hepatitis. Centrilobular fibrosis with or with out veno-occlusive disease was seen in five patients. Three patients h ad nonspecific hepatitis, four had changes attributed to total parente ral nutrition, and two had lymphoproliferative disorders involving the liver. Both patients with lymphoproliferative disorders had received transplants. Two patients had resolving necrosis probably secondary to non-A, non-B hepatitis. One had ayptical mycobacterial infection. Hem osiderosis was a common nonspecific abnormality, seen in nine patients . All patients with hepatic graft-versus-host diseases had received tr ansplants or nonirradiated blood products. Hepatic graft-versus-host d isease varied in severity from hepatic necrosis with destruction of bo th large and small bile ducts in a transfusion-associated case to subt le damage to interlobular bile ducts. Even minimal bile duct changes c orrelated with the clinical impression of graft-versus host disease in these patients. Late chronic graft-versus-host disease was not seen i n any patient, although acute graft-versus-host disease sometimes occu rred late after transplant.