Background Hepatitis-associated aplastic anemia is a variantof aplastic anemia in which aplastic anemia follows an acuteattack of hepatitis. The aplastic anemia, however, is oftenfatal if untreated. To characterize the illness, investigatethe role of hepatitis viruses, and assess the response to immunosuppressivetreatment, we studied patients with the syndrome who were referredto the National Institutes of Health (NIH).
Methods Standard hematologic and biochemical tests and measurementsof bone marrow cellularity were used to monitor the patients'response to treatment. Serum was assayed for antibodies andantigens related to hepatitis A, B, and C viruses and for theRNA of hepatitis C and GB virus C by the polymerase chain reaction.All patients were treated with antithymocyte globulin and cyclosporine.
Results Ten patients with hepatitis-associated aplastic anemiawere referred to the NIH between 1990 and 1996; all had thetypical features of this syndrome. There was evidence of activatedCD8 T lymphocytes in the blood. Serologic tests for hepatitisA, B, and C viruses were negative; RNA of hepatitis C viruswas undetectable in all patients, but RNA of GB virus C wasdetected in three patients. Seven of the patients respondedto intensive immunosuppressive treatment; the three who didnot respond all died within one year of treatment, two fromcomplications of stem-cell or marrow transplantation.
Conclusions The hepatitis of the hepatitis-associated aplasticanemia does not appear to be caused by any of the known hepatitisviruses. We recommend immunosuppressive treatment for patientswho do not have an HLA-matched related donor available for bonemarrow transplantation. Several features of the syndrome suggestthat it is mediated by immunopathologic mechanisms.
Source Information
From the Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, Md.
Address reprint requests to Dr. Brown at Bldg. 10, Rm. 7C218, National Institutes of Health, 9000 Rockville Pike, Bethesda, MD 20892-1652.
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