Hello Maciej
it is most likely that your physician was querying a diagnosis of
"Leukocyte Adhesion Deficiency Type-1". This is a disorder caused in every
case identified to date by mutations in the Beta chain (CD18). Mutations
in CD18 lead to the abnormal expression of all of the beta 2 leukocyte
integrins (CD11a, CD11b and CD11c), since the proper expression of CD18 is
required for their normal expression. Clinically patients suffer from
severe difficult to treat non-purulent skin lesions, gingivitis and
periondontitis. The common clinical condition cited is delayed umbilical
cord separation. The most facile method for detecting LAD-1 is to gate on
neutrophils and look for the expression of CD11b on both resting and in
vitro activated whole blood. Yes this can be done in whole blood. We have
developed a diagnostic test which is extrely easy and can be performed in
only a few hours. Please refer to "O'Gorman, McNally, Andeson and Myones.
A rapid whole blood lysis technique for the diagnosis of moderate or severe
Leukocyte Adhesion Deficiency Type-1. Annal. NY Acad. Sci.. 677:427-430,
1993. We offer this test on a routine basis in our laboratory and would be
happy to provide you with a NCCLS formatted laboratory procedure.
Regards
Mo.
>Greetings once again,
>
>We have recently received a blood specimen and were asked to stain
>for CD18/11b ( I love it how doctors don't explain the condition of
>the patient ahead of time, so we know what to look for )
>
>We found, that when compared to our NC's (N=300+ for this "tube" and
>growing) this patient had less +/+ lymphoctes (~30% vs. 12%). The
>expression of CD18 was not reduced - all of his leukocytes had >97%.
>
>A question came up: what is the significance of CD18+/CD11b-
>lymphocytes? what is the significance of a decrease in the +/+
>population?
>
>We do not routinely do CD11a or CD11c - are these mutually exclusive?
>I.e. it's either a, b or c but never any of them together?
>
>And finally, is the ligand for CD11b soluble and may have interfered
>with our staning?
>
>This staining was done in whole blood (which I just found is a
>no-no).
>
>I would appreciate if anyone could share any thoughts on this topic.
>
>Maciej
>
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Dr. Maurice (Mo) R.G. O'Gorman
Associate Professor Pediatrics
Northwestern Univ. Med. School
Director Diagnostic Immunology and Flow Cytometry Laboratories
The Children's Memorial Hospital
2300 Children's Plaza
Chicago, IL 60614
Ph. 773 880 3070 office
773 880 4361 laboratory
Fax 773 880 3739
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