About Leukocyte adhesion deficiency type II

What is Leukocyte adhesion deficiency type II?

Leukocyte adhesion deficiency type II (LAD-II) is a rare, inherited disorder that affects the body's ability to fight infection. It is caused by a mutation in the ITGB2 gene, which is responsible for producing a protein called beta-2 integrin. This protein helps white blood cells (leukocytes) stick to the walls of blood vessels, allowing them to move to the site of an infection and fight it. Without this protein, the white blood cells cannot move to the site of infection, leading to recurrent and severe infections.

What are the symptoms of Leukocyte adhesion deficiency type II?

The symptoms of Leukocyte adhesion deficiency type II (LAD-II) include recurrent bacterial infections, especially of the skin, lungs, and gastrointestinal tract. Other symptoms may include delayed healing of wounds, poor growth, and recurrent fever.

What are the causes of Leukocyte adhesion deficiency type II?

Leukocyte adhesion deficiency type II (LAD-II) is a rare inherited disorder caused by mutations in the ITGB2 gene. This gene provides instructions for making a protein called integrin beta-2, which is found on the surface of white blood cells called leukocytes. This protein helps leukocytes stick to other cells and to the walls of blood vessels, a process known as adhesion. Mutations in the ITGB2 gene reduce or eliminate the production of integrin beta-2, which prevents leukocytes from adhering to other cells and the walls of blood vessels. As a result, leukocytes are unable to move to the sites of infection, leading to recurrent and severe bacterial infections.

What are the treatments for Leukocyte adhesion deficiency type II?

1. Hematopoietic stem cell transplantation: This is the most effective treatment for Leukocyte adhesion deficiency type II. It involves replacing the defective stem cells with healthy ones from a donor.

2. Antibiotic therapy: Antibiotics can be used to treat infections caused by Leukocyte adhesion deficiency type II.

3. Immunoglobulin therapy: Immunoglobulin therapy can help to boost the immune system and reduce the risk of infection.

4. Bone marrow transplantation: This is a more complex procedure than hematopoietic stem cell transplantation, but it can be used to treat Leukocyte adhesion deficiency type II.

5. Gene therapy: This is a relatively new treatment option that involves introducing a healthy gene into the patient’s cells to replace the

What are the risk factors for Leukocyte adhesion deficiency type II?

1. Genetic mutation in the CD18 gene
2. Inherited in an autosomal recessive pattern
3. Defects in the expression of the CD11/CD18 integrin family
4. Defects in the expression of the CD14 and CD16 molecules
5. Defects in the expression of the CD62L and CD11b molecules
6. Defects in the expression of the CD54 molecule
7. Defects in the expression of the CD66b molecule
8. Defects in the expression of the CD11a molecule
9. Defects in the expression of the CD18 molecule
10. Defects in the expression of the CD11c molecule

Is there a cure/medications for Leukocyte adhesion deficiency type II?

Yes, there is a cure for Leukocyte adhesion deficiency type II. The only available treatment is a bone marrow transplant, which can be successful in restoring normal immune system function. Additionally, medications such as antibiotics, antifungals, and antivirals may be prescribed to help prevent and treat infections.