Leucocyte Adhesion Deficiency in Harrison 17th and 18th Editions : Operation Harri Capsule

Leucocyte Adhesion Deficiency in Harrison 17th and 18th Editions : Operation Harri Capsule

What Does Harrison 17th Edition and 18th Edition Tell about Leucocyte Adhesion Deficiency

LEUCOCYTE ADHESION DEFICIENCY

Leucocyte Adhesion Deficiency in Harrison 17th and 18th Editions : Operation Harri Capsule

17TH EDITION : CHAPTER-Disorders of Granulocytes and Monocytes

Disorders of Adhesion

• Two main types of leukocyte adhesion deficiency (LAD) have been described, LAD 1 and LAD 2. Both are autosomal recessive traits.
• Patients with LAD 1 have mutations in CD18
• The CD18 gene is located on distal chromosome 21q.
• Patients with LAD 1 have recurrent bacterial infections involving the skin, oral and genital mucosa, and respiratory and intestinal tracts; persistent leukocytosis (neutrophil counts of 15,000–20,000/L).
• The most common bacteria are Staphylococcus aureus and enteric gram-negative bacteria.
• LAD 2 is caused by an abnormality of fucosylation of SLex (CD15s), the ligand on neutrophils that interacts with selectins on endothelial cells and is responsible for neutrophil rolling along the endothelium.
• Infection susceptibility in LAD 2 appears to be less severe than in LAD 1. LAD 2 is also known as congenital disorder of glycosylation IIc (CDGIIc).

18TH EDITION : CHAPTER-Disorders of Granulocytes and Monocytes

• Three main types of leukocyte adhesion deficiency (LAD) have been described.
• All are autosomal recessive and result in the inability of neutrophils to exit the circulation to sites of infection, leading to leukocytosis and increased susceptibility to infection.
• Patients with LAD 1 have mutations in CD18.
• The CD18 gene is located on distal chromosome 21q.
• Patients with LAD 1 have recurrent bacterial infections involving the skin, oral and genital mucosa, and respiratory and intestinal tracts; persistent leukocytosis (resting neutrophil counts of 15,000–20,000/L) because cells do not marginate; and, in severe cases, a history of delayed separation of the umbilical stump.
• The most common bacteria are Staphylococcus aureus.
• Infection susceptibility in LAD 2 appears to be less severe than in LAD 1.
• LAD 2 is also known as congenital disorder of glycosylation IIc (CDGIIc) due to mutation in a GDP-fucose transporter (SLC35C1).
• LAD 3 is characterized by infection susceptibility, leukocytosis, and petechial hemorrhage due to impaired integrin activation caused by mutations in the gene FERMT3.

Type 1: Delayed separation of umbilical cord, sustained neutrophilia, recurrent infections of skin and mucosa, gingivitis, periodontal disease
Type 2: Mental retardation, short stature, Bombay (hh) blood phenotype, recurrent infections, neutrophilia
Type 3: Petechial hemorrhage, recurrent infections

What is in store for tomorrow?
Retro virus has changed it’s priorities towards the human heart.What is that-to know from the 18th edition of HARRISON-Kindly log on tomorrow
Warm regards,
Dr.R.Manorajan
POSITIVE

PS : This is an extract from Operation Harri Capsule. This is a crash course for AIIMS, PGI and AIPG. For more details See OHC