Deficiencia de adhesión leucocitaria: detección de los primeros casos en Paraguay

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Keywords

Leukocyte Adhesion Deficiency Syndrome; Cell Adhesion Molecules; Flow Cytometry; Leukocytosis

Abstract

Background: Leukocyte adhesion deficiency (LAD) is a primary immunodeficiency with low suspicion and underdiagnosed. There are no detected cases in Paraguay, and the diagnostic methodology is not available. Objective: To implement the diagnostic technique for LAD by evaluating the expression of CD18 and CD15 in healthy patients and in a group with clinical suspicion. Methods: Twenty healthy pediatric patients who attended the Instituto de Investigaciones en Ciencias de la Salud (IICS), and 40 patients from public hospitals with clinical suspicion of LAD were studied, period 2017-2019. The expression (%) of CD18 and CD15 in peripheral blood leukocytes was evaluated by flow cytometry, establishing a normal range in healthy patients. The presence of LAD was established by decreased expression of CD18 or CD15. Results: Twelve out of twenty healthy patients were male (median age: 14 years) and 27/40 patients with clinical suspicion were female (median age: 2 years). Persistent leukocytosis and respiratory tract (32%) and skin (25%) infections predominated. The expression range of CD18 and CD15 in healthy patients was 95%-100%, and in patients with clinical suspicion 0%-100%. One patient with 0% expression of CD18 (LAD-1) and one patient with 0% CD15 (LAD-2) were detected. Conclusions: The implementation of a new diagnostic technique allowed to establish a normal range of CD18 and CD15 by flow cytometry and it was possible to detect the first two cases of LAD in Paraguay.

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References

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