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Hepatitis-associated aplastic anemia

K E Brown1, J Tisdale, A J Barrett

  • 1Hematology Branch, National Heart, Lung, and Blood Institute, Bethesda, MD 20892-1652, USA.

The New England Journal of Medicine
|April 10, 1997
PubMed
Summary
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Hepatitis-associated aplastic anemia, often fatal if untreated, appears to be immunopathologically mediated, not caused by known hepatitis viruses. Immunosuppressive treatment shows promise for patients lacking a bone marrow transplant donor.

Area of Science:

  • Hepatology
  • Hematology
  • Immunology

Background:

  • Hepatitis-associated aplastic anemia (HAAA) is a severe condition following hepatitis.
  • HAAA can be fatal without timely intervention.
  • The National Institutes of Health (NIH) investigated HAAA to understand its characteristics and treatment efficacy.

Purpose of the Study:

  • To characterize HAAA.
  • To investigate the role of hepatitis viruses in HAAA.
  • To assess the effectiveness of immunosuppressive treatment for HAAA.

Main Methods:

  • Hematologic and biochemical tests were used for monitoring.
  • Bone marrow cellularity was measured.
  • Serum analysis included hepatitis A, B, C virus assays, and PCR for hepatitis C and GB virus C RNA.

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  • Patients received antithymocyte globulin and cyclosporine.

    • Main Results:

    • Ten HAAA patients were studied; all exhibited typical syndrome features.
    • Evidence of activated CD8 T lymphocytes was found.
    • Hepatitis A, B, C viruses were negative; GB virus C RNA was detected in three patients.
    • Seven patients responded to immunosuppressive treatment; three non-responders died within a year.

    Conclusions:

    • HAAA hepatitis is unlikely caused by known hepatitis viruses.
    • Immunosuppressive therapy is recommended for HAAA patients without an HLA-matched donor.
    • The syndrome's features suggest an immunopathologic mechanism.