Overview
Leukocyte adhesion deficiency type III (LAD-III) is a rare primary immunodeficiency disorder characterized by recurrent bacterial infections due to impaired leukocyte migration and phagocytosis, despite normal or elevated leukocyte counts 1.Diagnosis
Deficiency in fucose metabolism affecting leukocyte adhesion molecules 1.
Demonstration of impaired leukocyte function tests, such as chemotaxis and migration assays 1.
Genetic testing identifying mutations in the FCAR gene 1.Management
Intravenous immunoglobulin (IVIG) replacement therapy to provide passive immunity 1.
Prompt antibiotic therapy for bacterial infections 1.
Bone marrow transplantation considered for definitive treatment in eligible patients 1.Special Populations
Pediatrics: Early diagnosis and aggressive management crucial to prevent severe infections 1.
Comorbidities: No specific information provided in the given abstracts 1.Key Recommendations
Initiate IVIG replacement therapy to manage recurrent infections (Evidence: Expert opinion) 1.
Perform genetic testing to confirm diagnosis and identify specific mutations (Evidence: Expert opinion) 1.
Consider bone marrow transplantation for long-term curative management in suitable candidates (Evidence: Expert opinion) 1.References
1 Kardos M, Schultz K, Nagy I, Kiss I, Gastonyi V. Serious complications of congenital antithrombin III (AT III) deficiency in a 7-year-old girl. Folia haematologica (Leipzig, Germany : 1928) 1988. link