Common variant immunodeficiency: a case series and literature review / 中华全科医师杂志

ABSTRACT

Objective:To analyze the clinical manifestation, laboratory examination, treatment and prognosis of common variable immunodeficiency (CVID) in single center of Chinese population.Methods:The clinical data of 75 cases of CVID, diagnosed according to European Society for Immunodeficiency (ESID) criteria and admitted in Peking Union Medical College Hospital from January 1983 to May 2021 were retrospectively analyzed.Results:The main clinical manifestations of CVID were respiratory abnormality (68.0%,51/75), blood system abnormality (66.7%,50/75), liver and spleen involvement (66.7%,50/75), gastrointestinal abnormality (46.7%,35/75), autoimmune abnormality (29.3%,22/75). Immunoglobulin decreased significantly (median IgG 2.4 g/L, median IgA 0.1 g/L, median IgM 0.1 g/L). Lymphocyte subsets indicated that CD4 +T cells decreased (median 471/μl), CD8 +T cells increased (median 620/μl), CD4 +/CD8 +T calls proportion inverted (median 0.7), and NK cells and B cells decreased (median 44/μl, 115/μl, respectively). During hospitalization, their conditions were improved after IgG replacement therapy, supplemented with anti-infection and nutritional support therapy. Forty seven discharged patients were followed up, and only 16 patients insisted on regular IgG replacement therapy after discharge. Conclusions:The clinical manifestations of CVID are varied, and multiple systems may be involved, including autoimmune abnormalities. The treatment based on IgG replacement has a certain curative effect.