Leukocyte Adhesion Deficiency (CD11a, CD11b, CD11c, CD18)
Test Code
FLOW LAD
Test Synonyms
Beta2 integrin
Associations
Leuocycte adhesion, Phagocyte aggregation, CD18 (beta2 integrin) gene
Methodology
Flow Cytometry
CPT Codes
88184 – Flow cytometry, cell surface, cytoplasmic, or nuclear marker, technical component only, first marker
88185 x4 – Each additional marker (multiple)
88187 – Flow cytometry, interpretation, 2 to 8 markers
88185 x4 – Each additional marker (multiple)
88187 – Flow cytometry, interpretation, 2 to 8 markers
Turnaround Time
1 day
Specimen Requirements
- 5.0 mL (min. 2.0 mL) heparin blood preferred, EDTA whole blood accepted
- 3.0 mL (min. 1.0 mL) heparin bone marrow preferred, EDTA bone marrow accepted
- 20 mm^3 bone marrow core biopsy in MPLN RPMI transport media
Specimen Stability
- Whole blood or bone marrow stable for 48 hours at 18-25°C
- Bone marrow biopsy in MPLN RPMI stable for 48 hours at 2-8°C
Storage & Handling
- Whole blood or bone marrow, ship ambient
- Bone marrow biopsy, ship in a Styrofoam container with an ice pack (Do not allow the ice pack to directly contact the sample)
Reference Range
See report
Related Content
Specimens stored at incorrect temperature; Non-viable specimens; Specimens in inappropriate anticoagulant; Too few cells; Hemolysis; Specimen clotted
Description
Leukocyte adhesion deficiency (LAD) is a rare disorder of leukocyte function. LAD has been classified into two major subtypes and rare variants.
LAD type 1 is the more common subtype and is characterized by decreased or absent expression of the CD11/CD18 complex, resulting in an inability of leukocytes to emigrate from the bloodstream to sites of inflammation. Severely affected infants present with delayed umbilical cord detachment, recurrent or progressive non-purulent infections, delayed wound healing and leukocytosis. Flow cytometry can be used to detect normal expression of the CD11/CD18 complex.
LAD type 2 is less common and is a disorder of fucosylation characterized by deficiency of sialyl Lewis X (sLex) and an inability of normal leukocyte rolling function on activated endothelium.
Other “variants” of LAD have been reported and include normal expression, but dysfunctional CD11/CD18; E selectin/CD62E expressed, but cleaved and present only as soluble form; and defective Rap-1 activator CalDAG-GEFI.
LAD type 1 is the more common subtype and is characterized by decreased or absent expression of the CD11/CD18 complex, resulting in an inability of leukocytes to emigrate from the bloodstream to sites of inflammation. Severely affected infants present with delayed umbilical cord detachment, recurrent or progressive non-purulent infections, delayed wound healing and leukocytosis. Flow cytometry can be used to detect normal expression of the CD11/CD18 complex.
LAD type 2 is less common and is a disorder of fucosylation characterized by deficiency of sialyl Lewis X (sLex) and an inability of normal leukocyte rolling function on activated endothelium.
Other “variants” of LAD have been reported and include normal expression, but dysfunctional CD11/CD18; E selectin/CD62E expressed, but cleaved and present only as soluble form; and defective Rap-1 activator CalDAG-GEFI.
References
- Nervi SJ et al. (2007). Leukocyte Adhesion Deficiency. eMedicine.
- Cox et al. (2007). Leukocyte adhesion deficiency type 1: an important consideration in the clinical differential diagnosis of prepubertal periodontitis. a case report and review of the literature. Oral Surgery, Oral Medicine, Oral Pathology, Oral Radiology and Endodontology.
- Hann et al. (2006). Pediatric Hematology. Blackwell Publishing.
- Lichtman et al. (2006). Hematology, 7th edition. McGraw-Hill Companies, Inc. 935-938.
- Nervi SJ (2006). Leukocyte Adhesion Deficiency. eMedicine.
- Etzioni A. (2005). Leukocyte Adhesion Deficiency (LAD) Syndromes. Orphanet Encyclopedia.
- Keren et al. (2001). Flow Cytometry in Clinical Diagnosis 3rd edition. ASCP Press.
