Hematopoietic stem cell transplantation in treating patients with chronic granulomatous disease at the National Children’s Hospital
Main Article Content
Abstract
Chronic Granulomatous Disease (CGD) is an inborn error immunity, characterized by recurrent infections with bacteria and fungi, which can be fatal due to impaired phagocytic function of the innate immune system cells. Without hematopoietic stem cell transplantation (HSCT), patients with CGD have recurrent life-threatening infections and may develop inflammatory diseases, autoimmune diseases and granulomas. We describe two Vietnamese children presented with many recurrent, difficult to treat lungs, gastrointestinal and cutaneous infections since infancy. Dihydrorhodamine (DHR) test showed absent oxidative burst activity by the patient's neutrophils, and genetic testing revealed mutations are associated with CGD. The patients were successfully treated with HSCT from full-matched sibling donors. It is important to consider CGD for any patients presented with severe infection from infancy. HSCT should be considered as standard of care and should be considered early after diagnosis.
Article Details
Keywords
Inborn error immunity, Chronic granulomatous disease, Hematopoietic stem cells transplantation, CYBB, CYBA
References
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