Immunology

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Investigation

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Notes

Useful for:

• Investigating Leukocyte Adhesion Deficiency in patients younger than 18 years old.

Clinical Information:

• The CD18 molecule is a member of the beta-2 integrin family.
• Autosomal mutations in the CD18 gene result in an inability to express CD11a, CD11b and CD11c correctly.
• This results in defective leukocyte adhesion, required for effective leukocyte accumulation in tissue.
• Characterisitcs of Leukocyte Adhesion Deficiency include delayed umbilical stump separation, infection of the umbilical stump, leukocytosis in the absence of infection, gingivitis with peridontitis and alveolar bone resorption.
• There are three immunodeficieny states associted with leukocyte adhesion defects; Leukocyte Adhesion Deficieicy Type 1, 2 and 3.
• Leukocyte Adhesion Deficieicy Type 1 results from the absence of CD18.
• Leukocyte Adhesion Deficieicy Type 2 is due to the absence of fucosylated carbohydrate ligands.
• Leukocyte Adhesion Deficieicy Type 3 is an extremely rare autosomal recessive syndrome.

Please Note:

• As this is a specialised test please contact the laboratory to arrange sample processing.
• Samples must be processed within 5 hours of collection.
• External Institiutions must transport samples immediately to the St. James's Hospital Immunology department.
• Absoulte lymphocyte levels and influenced by circadian variation, therefore consistency of timing when acquiring blood samples is required when monitoring patients serially.

Related links

Reference range

Reference ranges have been established in the Immunology Department, St. James's Hospital, using 15 normal adult donors.

Last updated

Wed, 09 Mar 2022 09:26:32 GMT