Background <p>Good Syndrome (GS) is a rare immunodeficiency disorder classically defined by the triad of thymoma, hypogammaglobulinemia, and recurrent infections. Cytomegalovirus (CMV) infection is the most frequently reported viral infection in patients with GS. This study aimed to characterize the clinical features and immunological alterations in patients with GS complicated by CMV infection.</p> Methods <p>We conducted a retrospective cohort study of GS patients with or without CMV infection at Peking Union Medical College Hospital from January 2015 to December 2024. Logistic regression and COX proportional hazards regression model were employed to identify potential risk factors for CMV infection and prognostic factors of overall survival among GS patients.</p> Results <p>Among the 27 enrolled patients, 15 developed CMV infection: 1 presented with CMV pneumonia, 6 was clinically diagnosed with CMV pneumonia, 6 with isolated CMV DNAemia, 1 with CMV colitis, and 1 with CMV retinitis. Peripheral blood CD4 + T-cell counts were significantly lower in the CMV group than non-CMV group (156 [116–210] vs. 374 [200–443] cells/µL; <i>P</i> &lt; 0.05). The CMV group exhibited a higher rate of glucocorticoid (GC) use, greater cumulative GC exposure prior to diagnosis, and higher frequencies of respiratory pathogen isolation and fungal infection compared with the non-CMV group. Multivariable logistic regression identified lower serum albumin (<i>P</i> = 0.012; OR, 0.743; 95% CI: 0.58–0.938) and lower serum IgG (<i>P</i> = 0.041; OR, 0.565; 95% CI: 0.327–0.978) as potential risk factors for CMV infection in GS patients.</p> Conclusion <p>GS patients with CMV infection exhibited lower lymphocyte counts, higher glucocorticoid administration rate and increased susceptibility to respiratory and opportunistic fungal infections. During active CMV infection, these patients consistently displayed CD8<sup>+</sup> T-cell lymphocytosis. Hypoalbuminemia and low serum IgG were associated with an increased risk of CMV infection. However, it is important to emphasize that the factors identified in our study apply to the broad definition of CMV infection and may not specifically predict end-organ disease.</p>

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Clinical characteristics and risk factors of cytomegalovirus infection in patients with good syndrome

  • Qi Chen,
  • Ruxuan Chen,
  • Yujie Shi,
  • Shiyang Wang,
  • Zhiyi Li,
  • Yupei Zhang,
  • Hui Huang,
  • Chi Shao

摘要

Background

Good Syndrome (GS) is a rare immunodeficiency disorder classically defined by the triad of thymoma, hypogammaglobulinemia, and recurrent infections. Cytomegalovirus (CMV) infection is the most frequently reported viral infection in patients with GS. This study aimed to characterize the clinical features and immunological alterations in patients with GS complicated by CMV infection.

Methods

We conducted a retrospective cohort study of GS patients with or without CMV infection at Peking Union Medical College Hospital from January 2015 to December 2024. Logistic regression and COX proportional hazards regression model were employed to identify potential risk factors for CMV infection and prognostic factors of overall survival among GS patients.

Results

Among the 27 enrolled patients, 15 developed CMV infection: 1 presented with CMV pneumonia, 6 was clinically diagnosed with CMV pneumonia, 6 with isolated CMV DNAemia, 1 with CMV colitis, and 1 with CMV retinitis. Peripheral blood CD4 + T-cell counts were significantly lower in the CMV group than non-CMV group (156 [116–210] vs. 374 [200–443] cells/µL; P < 0.05). The CMV group exhibited a higher rate of glucocorticoid (GC) use, greater cumulative GC exposure prior to diagnosis, and higher frequencies of respiratory pathogen isolation and fungal infection compared with the non-CMV group. Multivariable logistic regression identified lower serum albumin (P = 0.012; OR, 0.743; 95% CI: 0.58–0.938) and lower serum IgG (P = 0.041; OR, 0.565; 95% CI: 0.327–0.978) as potential risk factors for CMV infection in GS patients.

Conclusion

GS patients with CMV infection exhibited lower lymphocyte counts, higher glucocorticoid administration rate and increased susceptibility to respiratory and opportunistic fungal infections. During active CMV infection, these patients consistently displayed CD8+ T-cell lymphocytosis. Hypoalbuminemia and low serum IgG were associated with an increased risk of CMV infection. However, it is important to emphasize that the factors identified in our study apply to the broad definition of CMV infection and may not specifically predict end-organ disease.