[CIS PIDD] Making Diagnosis of LAD -leukocyte adhesion defect- 1

[Oner Ozdemir]

Dear All,

We are dealing a patient suggestive of leukocyte adhesion defect -1.

The patient is borne with C/S, G3P3, pair of twins, with sterile cerebral
abscesses. And there is a sibling to this twins is 5-year-old and healthy.

CBC: normal, Ig GAME: normal, lymphocyte subpopulations: normal in the
patient.

On this patient, flow cytometry revealed that borderline low CD18
expression (80 %) on lymphocytes, but neutrophil and monocyte CD18
expression within normals (above 90 %).

And the lymphocyte expressions of CD11a /CD18: *19 %*; CD11b /CD18: *2 %*;
CD11c /CD18: *11 %* seemed to be low.


*My questions are:*

1- Even though total CD18 expression on lymphocytes is borderline low; can
we make a diagnosis of LAD with one of the lower expresions of CD11a
/CD18, CD11b /CD18, CD11c /CD18 on just lymphocytes?

2- Should these low expressions of CD11a /CD18, CD11b /CD18, CD11c /CD18
with CD18 epression be on the monocytes and neutrophils in addition to
lymphocytes at the same time to make a diagnosis?

3- Any normal reference values for CD11a /CD18, CD11b /CD18, CD11c /CD18
expressions in children?


Best Regards;

Oner Ozdemir, MD














-- 


Öner Özdemir, MD
Chief and Assoc. Prof. Pediatrics
Republic of Turkey, Ministry of Health, İstanbul Medeniyet University,
Göztepe Research and Training Hospital
Göztepe, Kadıköy, İSTANBUL -TÜRKİYE.
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