Atypical presentation of Good syndrome: acute hepatitis from hepatitis B virus reactivation

Abstract

Good syndrome (GS) is a primary immunodeficiency (PID) that presents in middle aged to older adults with features of thymoma, hypogammaglobulinemia, CD4 T lymphopenia, inverted CD4/CD8+ ratio, and impaired T-cell mitogen proliferative responses. We present a patient, a 62-year-old female, who first presented with disease manifestation of acute hepatitis from hepatitis B virus (HBV) reactivation, which was subsequently complicated by recurrent hospitalizations for recurrent pneumonia and concomitant Helicobacter pylori and cytomegalovirus enteritis. She was later found to have thymoma and hypogammaglobulinemia and was diagnosed with GS. Although the well-known importance of T cell is in directing B-cell responses in the immunopathology of thymoma, low levels of natural killer and CD4+ γδ T cells may also be the cause of both low immune surveillance of tumor development and weak clearance of viral infection. Hence, the temporal sequence of opportunistic infections following HBV reactivation and thymoma discovery may reflect a loss of immune surveillance as the first manifestation of PID.

Hypogammaglobulinemia, a new risk factor for hepatitis B virus reactivation : about two cases.

“…In these two cases the loss of protective anti-HBs antibodies due to hypogammablobulinemia is probably the trigger of HBV recurrence. Interestingly, a case of HBV reactivation in a patient with hypogammaglobulinemia in the context of Good syndrome has recently been described and may support our hypothesis (5). In our first patient, hypogammaglobulinemia is chronic and affects all Ig subclasses.…”

Pathophysiology of Virus Infections: The Protein-Homeostasis-System Hypothesis

“…Lymphopenia may be a characteristic of patients with severe pneumonia infected with respiratory pathogens, including influenza viruses, coronaviruses, measles virus, and MP [88][89][90][91]. The severity of lymphopenia correlates with target cell injury in these diseases and chronic viral infections, including AIDS and hepatitis virus infections, when relapsed patients are severely affected [92,93]. Earlier experimental animal studies on respiratory pathogens, including influenza viruses and MP, have reported that extensive infiltration of small lymphocytes is observed in early lung lesions within 2-3 days after inoculation, before the appearance of virus-specific antibodies, irrespective of inoculation sites, such as intraperitoneal, oral, or intranasal routes [1].…”