Long term outcome of eight patients with type 1 Leukocyte Adhesion Deficiency (LAD-1): Not only infections, but high risk of autoimmune complications
DU De Rose, S Giliani, LD Notarangelo, V Lougaris… - Clinical …, 2018 - Elsevier
Abstract Leukocyte Adhesion Deficiency type 1 (LAD-1) is a rare primary immunodeficiency
due to mutations in the gene encoding for the common β-chain of the β2 integrin family
(CD18). Herein, we describe clinical manifestations and long-term complications of eight
LAD-1 patients. Four LAD-1 patients were treated with hematopoietic stem cell
transplantation (HSCT), while the remaining four, including two with moderate LAD-1
deficiency, received continuous antibiotic prophylaxis. Untreated patients presented …
due to mutations in the gene encoding for the common β-chain of the β2 integrin family
(CD18). Herein, we describe clinical manifestations and long-term complications of eight
LAD-1 patients. Four LAD-1 patients were treated with hematopoietic stem cell
transplantation (HSCT), while the remaining four, including two with moderate LAD-1
deficiency, received continuous antibiotic prophylaxis. Untreated patients presented …