So white cells migrate, but don’t adhere so can’t function.

3 types:

• Type 1= beta2 integrin defect (no adhesion)
• Type 2=selectin ligand (a fucose transporter; no rolling). Developmental delay and hepatosplenomegaly.
• Type 3= RAP1. Bleeding disorder can be seen too.

Classically delayed cord separation (but actually seen in any neutrophil disorder), skin ulcers esp perianal, periodontitis, raised white cell count (because they are stimulated but don’t move about normally).

Lymphocyte subsets may reveal low CD 11/18.

Irish Setters particularly prone to… Puppies rarely survive 6 months.

Chemotaxis (migration) disorders, interestingly, tend to produce pretty mild disease eg periodontitis only…