Detection and Characterization of Specific Anti-Apolipoprotein E4 IgG Antibodies in Human Plasma and an Intravenous Immunoglobulin Preparation (Gammagard Liquid)

Abstract

Screening with T cell receptor incision circles (TREC) assay identifies newborns with severe T cell deficiency, including Severe Combined Immune Deficiency syndromes (SCID) and other causes. METHODS: A 3 week old Caucasian female born at 37 weeks gestation by vaginal delivery to a 28 year old mother with a history of myasthenia gravis post thyroidectomy requiring azathioprine 100 mg daily, prednisone 10 mg daily, and omeprazole during the pregnancy and with breastfeeding. Birth weight 2669 grams. She had a hip click, but did not have cardiac or any other malformation. Newborn SCID screen was positive. Breastfeeding was stopped to prevent further immunosuppression. RESULTS: At 3 weeks old she had very low total white blood cell count (0.8 TH/cmm), severe neutropenia (absolute 0.03 TH/cmm), severe lymphopenia (absolute 0.35 TH/cmm), anemia (hemoglobin 7.9 g/dL), and borderline low platelets (277 TH/cmm). Lymphocyte subsets showed profoundly low T cells (absolute CD3 206 cells/uL, absolute CD4 177 cells/uL, absolute CD8 83 cells/uL), no B cells (CD19 <20 cells/uL), and normal NK cells (171 cells/ uL). CBC and lymphocyte subsets were repeated weekly for one month and then monthly. At 3 months old, her total white blood cell count was 5.90 TH/ cmm, neutrophils 1.06 TH/cmm, and lymphocytes 3.95 TH/cmm. CD4 cells were normal (1540 cells/uL), and CD8 cells were moderately low (289 cells/ uL). CD19 B cells were normal (975 cells/uL). She remained healthy. She tolerated killed vaccines but has not had live viral vaccines. CONCLUSIONS: Taking azathioprine with prednisone during pregnancy may cause secondary, reversible immune suppression in the fetus.