| Gender/age | CD11a/CD18 expression (MFI) | % LFA-1 expression | Details |
---|---|---|---|---|
LAD-1 #1 | Male, 8 years | 4/8 | <1% of normal | Late detachment of umbilical cord, recurrent infections, BM transplantation planned. |
LAD-1 #2 | Male, 15 years | 4/8 | 5% of normal | Recurrent infections, no chemotaxis/adhesion of granulocytes. Received granulocyte transfusions, no BM donor available. |
LAD-1 #3 | Female, 3 years | 4/7 | n.d. | Mild symptoms, ready for BM transplantation. |
LAD-1 variant | Male, 12 years | 104/199 | normal | Late detachment of umbilical cord, mild nonpussing inflammatory responses, necrotic of nature (20). Granulocyte transfusions for life-threatening pneumonia. Recently BM transplanted. |