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Lack of adhesion of leukocytes: causes, symptoms, diagnosis, treatment
Last reviewed: 23.04.2024
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Lack of adhesion of leukocytes is a consequence of a defect in adhesion molecules, which leads to dysfunction of granulocytes and lymphocytes and the development of recurrent infections of soft tissues.
It is inherited by autosomal recessive type. Lack of adhesion of leukocytes is a consequence of insufficiency of adhesion 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. Such deficiency disrupts the ability of granulocytes (and lymphocytes) to migrate through the walls of blood vessels to tissues, to participate in cytotoxic reactions and phagocytosis of the bacterium. The severity of the disease correlates with the degree of failure.
In children with severe leukocyte adhesion deficiency, recurrent or progressive necrotic soft tissue infections caused by staphylococcal or gram-negative flora, periodontitis, poor wound healing, leukocytosis and prolonged healing (> 3 weeks) of umbilical cord injury are noted. The number of leukocytes is high, even during periods of remission. Over time, infection control becomes more difficult.
The diagnosis is confirmed with monoclonal antibodies (eg, anti-CD11 or anti-CDI 8) and flow cytometry, which indicate the absence or severe disruption of adhesion glycoproteins on the surface of leukocytes. Leukocytosis in a general blood test is not specific. Most patients die before the age of 5, with the exception of those who have successfully undergone bone marrow transplantation, but patients with moderate leukocyte adhesion failure survive to adulthood. The appointment of antibiotics is often prolonged. Transfusion of granulocytes also helps. The only effective treatment is bone marrow transplantation.
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