Asthma Allergy Immunology

Asthma Allergy Immunology

Hepatic Involvement in Common Variable Immunodeficiency: a Single Center Experience

Recep EVCEN 1, Haci Hasan ESEN 2, Fatih COLKESEN 1, Filiz SADI AYKAN 1, Mehmet KILINC 1, Ummugulsum YILMAZ ERGUN 1, Fatma Arzu AKKUS 1, Tugba ONALAN 1, Selim KAHRAMAN 1, Mehmet Emin GEREK 1, Eray YILDIZ 3, Naile KOKBUDAK 2, Sevket ARSLAN 1,

1 Department of Clinical Immunology and Allergy, Necmettin Erbakan University, Faculty of Medicine, Konya, Turkey
2 Department of Pathology, Necmettin Erbakan University, Faculty of Medicine, Konya, Turkey
3 Department of Clinical Immunology and Allergy, Necip Fazıl City Hospital, Kahramanmaraş, Turkey

DOI: 10.21911/aai.2024.402
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Objective: Common Variable Immunodeficiency (CVID) is the most prevalent symptomatic primary immunodeficiency in adults. This study evaluates the clinical, immune, and immunohistological characteristics of hepatic involvement in CVID.

Materials and Methods: Medical records of 72 CVID patients, including 7 with liver biopsies, were retrospectively reviewed. Immunohistochemical staining was performed to identify immune cell infiltration.

Results: We enrolled 72 patients with a median age of 38 (22–77) years, and 47% (n = 34) were female. Elevated alkaline phosphatase (ALP) levels were found in 58.3% (n=42) of the patients. Hepatic involvement was more prevalent among females, and those with hepatic involvement displayed a higher incidence of autoimmune disease. Splenomegaly was identified in 85% of the patients with hepatic involvement. Notably, switched memory B cells (IgD- CD19+ CD27+) and the CD4+/CD8+ ratio were statistically lower in patients with hepatic involvement (p=0.031, p=0.047). Liver biopsies were performed on 7 patients, revealing minimal to severe chronic hepatitis. Immunohistologic analysis of these liver biopsies revealed varying degrees of positivity of CD8+, CD4+, and CD16+. However, staining for CD21, CD56, and CD138 yielded negative results.

Conclusion: Our findings suggest that immune cells, particularly CD8+, CD4+, and CD16+ cells, may play a role in developing hepatic involvement. Understanding the underlying causes and mechanisms of hepatic involvement in CVID patients can lead to early diagnosis and targeted treatments, ultimately improving the prognosis.

Keywords : Common variable immunodeficiency, nodular regenerative hyperplasia, hepatic involvement, T cells