Yineleyen Cilt İnfeksiyonlarıyla Seyreden Lökosit Adezyon Defekti Vakası

Yıl 2009, Cilt: 9 Sayı: 2, 96 - 99, 01.04.2009

Müferet Ergüven Sinem Karaca Atakan Asuman Kıral

Öz

Lökositlerin adezyonu yeterli inflamasyon oluşması için başlıca rolü oynar. Bu çok aşamalı bir süreçtir. Bilinen iki adezyon molekülünün eksikliği sonucunda lökosit adezyon defekti sendromları oluşur. Yara iyileşmesinde gecikme, yineleyen infeksiyonlar ve lökositoz ile karakterizedir. Perineal bölgede ve ağızda dirençli ülsere lezyonlarla baş- vuran dokuz aylık erkek hastada lökositozun uzun süredir varlığı saptanınca CD18 ve ilişkili CD11a, CD11b, CD11c düzeyinin düşüklüğü klinik bulgularla birleştirilerek LAD-1 tanısı konmuştur

Anahtar Kelimeler

Lökosit adezyonu, yineleyen infeksiyon, inflamasyon defekti

Leucocyte Adhesion Defect With Recurrent Skin Infections

Öz

A nine-month boy presented with resistant ulcerated lesi- ons at perineal and oral regions. The levels of CD18 and related CD11a, CD11b, CD11c were found to be low. Persistent leucocytosis, low levels of CD cells and the cli- nical findingsled to the diagnosis of LAD-1

Anahtar Kelimeler

Leukocyte adhesion, recurrent infections, inflammation defect

Kaynakça

• 1. Rozensweig SD, Holland SM. Phagocyte immunodeficiencies and their infections. J Allergy Clin Immunol 2004; 113:620-6.
• 2. Folds JD, Schmitz JL. Clinical and laboratory assessment of immunity. J Allergy Clin Immunol 2003; 111:702-11.
• 3. McIntyre TM, Prescott SM, Weyrich AS. Cell-cell interac- tions. Leukocyte-endothelial interactions. Curr Opin Hematol 2003; 10:150-8.
• 4. Roos D, Law SKA. Hemotologically important mutations: leucocyte adhesion deficiency. Blood Cells Mol Dis 2001; 27:1000-4.
• 5. Bunting M, Harris ES, McIntyre TM. Leukocyte adhesion deficiency syndromes: adhesion and tetherind defects involving β2 integrins and selectin ligands. Curr Opin Hematol 2002; 9:30-5.
• 6. Bonilla FA, Geha RS. Primary immunodefiency diseases. J Allergy Clin Immunol 2003; 111:571-81.
• 7. Puck JM. Primary immunodefiency diseases. JAMA 1997; 278:1835-41.
• 8. Buckley RH. Pulmonary complications of primary immunodeficiencies. Pediatr Respir Rev 2004; 5(Suppl A):225-33.
• 9. Report of the IUIS Scientific Committee. Primary Immunode-ficiency Diseases. Clin Exp Immunol 1999; 118 (Suppl 1):1-28.
• 10. Etzioni A, Harlan JM. Cell adhesion and leukocyte adhesion dfect. In: Ochs HD, Smith CIE, Puck JM. Primary Immunodeficiency Diseases: A Molecular and Genetic Approch. New York: Oxford University Press, 1999: 375-88.
• 11. Etzioni A, Doerschuk CM, Harlan JM. Of man and mouse: leukocyte and endothelial adhesionmoleculedeficiencies. Blood 1999; 94:3281-8.
• 12. Bauer TR, Miller AD, Hickstein DD. Improved transfer of the leukocyte integrin CD18 subunit into hematopoietic cell lines by using retroviral vectors having a gibbon ape leukemia virus envelope. Blood 1995; 86:2373-87.
• 13. Segal BH, Holland SM. Primary phagocyte disorders of childhood. Pediatr Clin North Am 2000; 47:1311-38.