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7 Cards in this Set
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decreased B cells, IG's, BTK mutation:
decreased B cells, IG's, AR, IG-alpha, gamma-5 or BLNK mutations: Major morbidity in X-linked AG-emia? Treatment? What is contraindicated in these patients? |
XL agammaglobulinemia
AR agammaglobulinemia pulmonary disease/infections First treatment - replace IG's; don't give any live vaccines |
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B-cells normal or decreased, IGG decreased, no plasma cells, variable inheritance:
What pathogens commonly infect these patients? What is the defect in IGA deficiency? Are B-cells normal? |
Common Variable Immune Deficiency
S. pneumo, H. flu, S. aureus, susceptible to GI-replicating viruses (echovirus, polio); also HSV B-cells normal, but decreased terminal IGA+ differentiation |
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How are IG's affected in HIGM-CD40 deficiency? Type of inheritance?
Most common infections? In THI, what is affected? Why are there usually no serious infections? |
IGM, IGD, B-cells OK, rest are absent - AR inheritance
pulmonary - Pseudomonas, P. carinii, CMV THI - decreased IGG, IGA; AB-Ag response is still intact, so no serious infections |
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decreased IGM, IGG OK, increased IGA/IGE; XL, WASP gene; usually bleeding, bruising:
Decreased IG's, AR inheritance, 22q11 chromosome deletion, patients have abnormal facial features, lack of thymus fxn and decreased T-cells: X-linked, decreased T-cells, normal or increased B-cells, decreased IG's, NK cells: |
Wiskott-Aldrich syndrome
DiGeorge syndrome Common gamma chain deficiency |
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Progressively decreasing T and B cells, IG's; AR inheritance, ADA gene:
X-linked recessive, defect in NADP oxidase, decreased neuts and macrophages: Patients with complement deficiencies often get what infection? What autoimmune diseases are they are risk for? |
Adenosine deaminase deficiency
Chronic granulomatous disease Neisseria species - rehumatoid, scleroderma, SLE-like syndrome |
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Patients with _____ have a higher risk of graft vs. host disease.
What are some clinical features of SCID? Most common form of SCID? What type of inheritance? Why are there no functional B-cells? |
SCID
severe lymphopenia, decreased T, B cells, FTT, infections (CMV, Candida, protozoa, bacteria), chronic diarrhea Common gamma-chain deficiency - XLR No T-cells to help |
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Why do T-cells and B-cells die in ADA deficiency?
What is the difference between IL7-R-alpha and Jak 3 deficiencies? Most common PID? |
ADA helps protect against toxic metabolites
Jak3 has no NK cells, IL7-R-alpha does Chronic granulomatous disease |
