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Aspergillosis after liver transplantation in the context of common variable immunodeficiency: case report
1Johns Hopkins School of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.
Insights
Common variable immunodeficiency (CVID) can lead to autoimmune hepatitis and cirrhosis. A CVID patient undergoing liver transplant developed severe complications, highlighting risks in managing these complex cases.
Area of Science:
- Immunology
- Hepatology
- Transplantation
Background:
- Common variable immunodeficiency (CVID) is a primary immune defect causing hypogammaglobulinemia and impaired cell-mediated immunity.
- CVID is associated with various autoimmune phenomena, including autoimmune hepatitis (AIH).
- AIH is a serious CVID complication that can lead to cirrhosis, ascites, and hepatocellular carcinoma.
Observation:
- This report details a case of liver transplantation for cirrhosis secondary to AIH in a patient with CVID.
- The transplant surgery was initially uncomplicated.
- Post-transplant, the patient experienced rapid-onset alveolar hemorrhage and seizures.
Findings:
- The patient succumbed to disseminated neuroaspergillosis following the transplant.
- This case underscores the severe, potentially fatal complications that can arise in CVID patients with AIH undergoing transplantation.
Implications:
- Management of CVID patients with autoimmune complications requires careful consideration of transplant risks.
- Further research is needed to understand and mitigate post-transplant complications in this vulnerable population.
- This case highlights the critical interplay between immunodeficiency, autoimmunity, and infectious complications in advanced liver disease.
Abstract:
Common variable immunodeficiency (CVID) is the most common primary immune defect, resulting in hypogammaglobulinemia as well as deficits in cell-mediated immunity. Although it mainly manifests in immunodeficiency and related infection, CVID can also be associated with autoimmune phenomena such as immune thrombocytopenic purpura, hemolytic anemia, rheumatoid arthritis, lupus, primary biliary cirrhosis, and autoimmune hepatitis (AIH). AIH is a less common but serious complication of CVID, which can result in early cirrhosis, ascites, and even hepatocellular carcinoma. Here, we discuss a recent case of transplantation for cirrhosis secondary to AIH in the context of CVID. Although the patient's surgery occurred without complication, he rapidly developed fulminant alveolar hemorrhage and seizures, and died secondary to disseminated neuroaspergillosis.
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