Immunodeficiency Flashcards
What is the presenting pattern of Chronic Granulomatous Disease?
What test would you obtain
Invasive Skin Infections
Dihydrorhodamine ( DHR) oxidation test
What is the presenting pattern of a patient who has a Congenital defect in cell mediated immunity?
What test would you obtain
Benign or intracellular viral or fungal infections
CBC ( low Lymphocyte count) CD3,CD4 and CD8 lymphocyte markers
What is the presenting pattern of a patient with a congenital defect in producing Immunoglobulins? CVID
What test do you send
Repeated Sinopulmonary Infections with encapsulated bacteria ( pneumococcus/ haemophilus), infectious diarrhea esp Giardia and chronic diarrhea/ malabsorption
Quantitative serum immunoglobulins and response to tetanus and pneumococcal polysaccharide vaccines
How do you treat CVID
IVIG is first line therapy, most patients with selective IgA therapy do not require treatment
Standard IVIG is contraindicated in isolated igA deficiency because these patients may have IgG or IgE antibodies directed against transfused IgA
How does a patient with a congenital defect in the CFTR gene present? AKA patient with Cystic Fibrosis
What test do you send
Presents with Sinopulmonary infections, malabsorption, infertility, family history
Sweat Chloride test
How does a patient with a deficiency in terminal complement components ( C5-9, alternate and lectin pathways present?
Presents with recurrent Neisseria meningitidis infection and disseminated gonorrhea
Send a CH 50 assay. If this comes back negative and you have high suspicion, send alternate ( AH50 assay) and lectin pathway components
