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Leukocyte adhesion insufficiency: causes, symptoms, diagnosis, treatment
Last reviewed: 07.07.2025
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Leukocyte adhesion deficiency is a consequence of a defect in adhesion molecules, which leads to dysfunction of granulocytes and lymphocytes and the development of recurrent soft tissue infections.
Inherited in an autosomal recessive manner. Leukocyte adhesion deficiency is a consequence of the deficiency of adhesive glycoproteins on the surface of leukocytes, which leads to disruption of intercellular interactions, cell adhesion to the walls of blood vessels, cell migration and interaction with components of the complement system. This deficiency impairs the ability of granulocytes (and lymphocytes) to migrate through the walls of blood vessels into tissues, participate in cytotoxic reactions and phagocytosis of bacteria. The severity of the disease correlates with the degree of deficiency.
Children with severe leukocyte adhesion deficiency have recurrent or progressive necrotizing soft tissue infections caused by staphylococcal or gram-negative bacteria, periodontitis, poor wound healing, leukocytosis, and prolonged healing (>3 weeks) of the umbilical cord. The white blood cell count is high, even during periods of remission. Infections become increasingly difficult to control over time.
Diagnosis is confirmed by monoclonal antibodies (eg, anti-CD11 or anti-CDI 8) and flow cytometry, which show absent or severely impaired adhesion glycoproteins on the surface of leukocytes. Leukocytosis on complete blood count is nonspecific. Most patients die before age 5 years, except in those who have successfully undergone bone marrow transplantation, but patients with mild leukocyte adhesion deficiency survive into young adulthood. Antibiotics are often given for a long time. Granulocyte transfusions also help. The only effective treatment is bone marrow transplantation.
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