Abstract:

Objective To investigate the clinical characteristics, immunological characteristics and therapeutic effects of disseminated BCG complicated with primary immunodeficiency disease. Methods Twenty-three patients with disseminated BCG complicated with primary immunodeficiency disease were retrospectively collected from January 2015 to December 2019 in Children’s Hospital Affiliated to Chongqing Medical University. Among them, there were 18 males and 5 females with disseminated BCG disease within 6 months after inoculation. The clinical data and immunological characteristics were analyzed, and the therapeutic effect was followed up. Results Among the 23 children with disseminated BCG disease after BCG vaccination, there were 16 cases of chronic granulomatosis, 3 cases of primary immunodeficiency disease, 2 cases of severe combined immunodeficiency disease, 1 case of X-linked severe combined immunodeficiency disease and 1 case of X-linked high IgM syndrome. Among the 23 cases, 23 cases had pulmonary dissemination, 3 cases had liver spread, 1 case had abdominal dissemination, 1 case had left femur spread and 1 case had intracranial spread. Sixteen cases of children with chronic granulomatosis were abnormal in leukocyte phagocytosis test. One case of X-linked high IgM syndrome had significantly increased IgM level and decreased complement C3 level; two cases of severe combined immunodeficiency disease showed that CD3⁺ CD4⁺ T cells and CD3⁺ CD8⁺ T cells were significantly decreased, with the decrease of immunoglobulin level. Among the 23 cases, 6 cases died, the mortality rate was 26.1%; seventeen cases of survival children were followed up for 1.0-2.8 years and were in intermittent hospitalization. Conclusion Disseminated BCG complicated with primary immunodeficiency has an early onset, and chronic granulomatous disease is more common in these children; this disease has a high mortality rate and poor prognosis, so it is necessary to clarify the type of immunodeficiency and carry out targeted treatment as early as possible.

Key words: Child, Disseminated BCG disease, Immunologic deficiency syndromes, Disease attributes, Treatment outcome