Immunodeficiency Evaluation for Chronic Infections in Adults and Older Children Testing Click Here for Topics Associated with This Algorithm

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Immunodeficiency Evaluation for Chronic Infections in Adults and Older Children Testing Click here for topics associated with this algorithm

Rule out: 1. Physical/anatomic lesions, such as foreign body (bronchial), eustachian tube dysfunction, indwelling catheter, or other anatomic abnormalities 2. Cancer 3. Connective tissue diseases 4. Diabetes Adult or older child 5. Renal disease Recurrent upper and lower respiratory tract infections Then perform the following testing: AND/OR 1. Comprehensive Metabolic Panel (general health screening for immunodeficiency) Diarrhea, abscesses, sepsis, meningitis 2. CBC with platelet count (general health screening for immunodeficiency) 3. Human Immunodeficiency Virus (HIV) Combo Antigen/Antibody (HIV-1/O/2) by CIA, with Reflex to HIV-1 Antibody Confirmation by Western Blot 4. Protein Electrophoresis with Reflex to Immunofixation Electrophoresis Monoclonal Protein Detection, Quantitation and Characterization, IgA, IgG and IgM, Serum AND Immunofixation, Random, Urine OR Monoclonal Protein Study, Serum and Immunoglobulins (IgA, IgG, IgM), Quantitative 5. Sweat chloride (at accredited cystic fibrosis center) 6. If only recurrent sinopulmonary disease – pneumococcal antibody IgG titers pre and post vaccine (1 month)

Sweat Recurrent severe viral Recurrent severe sepsis Immunoglobulin No pneumococcal Abscesses, pneumonia, recurrent respiratory infections chloride HIV + infections, candidiasis, or infections (eg, Neisseria All testing normal evaluation abnormal antibodies with or without diarrhea abnormal herpes, etc. spp, S. pneumoniae)

ORDER Follow ORDER Rule out immunoglobulin Cystic Fibrosis (CFTR) 165 Specific ORDER ORDER • Lymphocyte Subset Panel abnormality or absent Pathogenic Variants with HIV algorithm antibody • Immunoglobulins (IgA, IgG, IgM), Quantitative Toll-Like Receptor 6 - Total Lymphocyte spleen Reflex to Sequencing deficiency • Immunoglobulin E Function Enumeration with CD45RA • Complement Activity Total, (CH50) and CD45RO • Neutrophil Oxidative Burst Assay (DHR) • Lymphocyte Subset Panel • Leukocyte Adhesion Deficiency Panel Variant present 7 - Congenital ORDER • Myeloperoxidase Stain Immunodeficiencies Complement Activity • CBC with platelet count and automated differential Enzyme Total (CH50) and Cystic fibrosis Complement Activity, confirmed Alternate Pathway (AH50) Abnormal Low Complement defect Low IgG, IgM, IgA Common variable complement ORDER ORDER OR B cells immune deficiency Consider Abnormal complement B Cell Subset Lymphocyte Antigen and Innate immune poor antibody present (CVID) or other genetic testing activity Analysis Mitogen Proliferation Panel deficiency response immune deficiency Low IgG or Hypogammaglobulinemia IgM suggests CVID

• Absent IgA Possible complement Abnormal Chronic granulomatous • Low IgG 2, 4 deficiency DHR disease Immunology consult • Pneumococcal vaccine Abnormal Consider genetic response low testing ORDER Decreased Leukocyte adhesion Immunoglobulin CD11b/ IgA low deficiency, type 1 G Subclasses Individual complement CD18 (1, 2, 3, 4) Warn of possible testing based on results of reaction to IgA- Symptomatic IgA absent Consider true T-cell CH50, AH50 Decreased Leukocyte adhesion containing blood treatment deficiency, HIV, CD4 CD15 products deficiency, type 2 deficiency, adenosine deaminase deficiency, Candida nucleoside phosphorylase Possible hyper IgE Monoclonal Consider monoclonal gammopathy of Follow If all normal, contact Increased specific IgE deficiency, chronic syndrome protein undetermined significance (MGUS), multiple Plasma Cell immunology director for IgE neutrophil mucocutaneous candidiasis (Job syndrome) present myelomas, or Waldenström macroglobulinemia Dyscrasia Algorithm further evaluation chemotaxis

Secondary protein losses Hematology consult Drug induced 1. Chronic renal disease Hypogammaglobulinemia May need bone marrow 1. Steroids Possible 2. Nephrotic syndrome secondary to malignancy Low exam 2. Anti-rheumatic secondary 3. Protein-losing 1. Chronic lymphocytic neutrophil dddrugs hypogammaglobulinemias ddenteropathy leukemia Contact immunology count 3. Phenytoin consultant or medical 4. Intestinal 2. Lymphoma Neutrophil antibody Autoimmune 4. Carbamazepine director ddlymphangiectasia positive neutropenia

ORDER High IgM Normal IgM cells, low Hyper IgM Consider B Cell Subset Absence of Myeloperoxidase low IgG, IgA IgG and IgA syndromes likely genetic testing Analysis myeloperoxidase deficiency