In Autoimmune Lymphoproliferative Syndrome (ALPS), all three of the lymphocyte subsets (alpha-beta positive, CD4 negative, and CD8 negative; CD5 positive and CD20 positive; and CD3 positive and HLA-DR positive) are elevated above the reference intervals. CD5 and HLA-DR may be found normally on B-cells and T-cells, respectively, as activation markers. Autoantibody production from CD5 positive B-cells is likely responsible for the autoimmune phenomena seen in ALPS. Increased levels of TCR alpha-beta positive, CD4 negative, and CD8 negative T-cells are the hallmark of ALPS and play a significant role in the pathophysiology of the disease.
Compliance Statement A
: Analyte specific reagents (ASR) are used in many laboratory tests necessary for standard medical care and generally do not require U.S. Food and Drug Administration (FDA) approval or clearance. This test was developed and its performance characteristics determined by ARUP Laboratories. The U.S. Food and Drug Administration has not approved or cleared this test; however, FDA clearance or approval is not currently required for clinical use. The results are not intended to be used as the sole means for clinical diagnosis or patient management decisions. This test should not be regarded as investigational or for research use.