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5 Facts you should know about
APDS
1
Characterized by chronic benign lymphoproliferation
2
Recurrent sinopulmonary infections (98%) and nonneoplastic lymphoproliferation (75%) are common manifestations
3
Autoimmune disease symptoms include immune cytopenias, juvenile arthritis, glomerulonephritis, and sclerosing cholangitis
4
There are varying degrees of increased IgM levels, increased circulating transitional B cells, decreased naïve CD4 and CD8 T-cells, with increased CD8 effector/memory cells
5
APDS appears to be caused by hyperactive phosphoinositide 3-kinase delta (PI3Kδ) signaling
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