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31 Cards in this Set

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Immunodeficiency diseases show a deficiency in what four areas?
1. B cell response
2. T cell response
3. Phagocytic response
4. Complement system
XLA inhibits maturation of what into what?
Inhibits pre-B cell into becoming immature B-cell
What is an important clue in diagnosing XLA.
Failure of infections to respond completely or promptly in proper antibiotic therapy.
Diagnosis of XLA in terms of:
T-cells
B-cells
Ig
T cells --> normal
B cells --> nonexistant in periphery
Ig --> none or marked decrease in all classes
Molecular cause of XLA
Nonfunctional tyrosine kinase (Burton's tyrosine kinase - btk)
Describe the Ig makeup of XLA.
Low or absent IgD, IgE, IgA
What do you see in XLA pt. biopsy?
No plasma cells in lamina propria of gut
Treatment of XLA.
Infusion of fresh plasma or gammaglobulin
Acquired hypogammaglobulinemia diagnosis in terms of:

B cells
T cells
Ig
B cells --> nl
T cells --> nl
Ig --> Decreased, but IgM and IgA may be normal
Pyogenic infections are susceptible to those with what disease?
Acquired hypogammaglobulinemia
Treatment of acquired hypogammaglobulinemia.
Infusion of plasma and gammaglobulin
Possible reason for acquired hypogammaglobulinemia. x2
1. Problems with T cell activating B cells
2. B cells does not properly secrete.
What is the most common immunodeficiency disease?
Selective IgA Deficiency
Why can't you treat Selective IgA Deficiency with fresh frozen plasma?
IgA will be seen as foreign
Common characteristics of selective IgA deficiency. x2
1. Recurrent sinopulmonary infections.
2. GI diseases
Hyper IgM syndrome has what major B-cell problem?
Defect in B cells to convert IgM to IgA (or IgG)
Test Diagnosis of Hyper IgM syndrome.
Normal or elevated IgM

Low IgA
Low IgG
Patients with hyper IgM syndrome manifests with what symptoms? x3
1. Tonsilitis
2. Otitis Media
3. Pneumonia
Congenital Thymic Aplasia determination based on:

T-cells
B-cells
NK cells
T cell deficiency
B cell function decrease
NK cells normal
T/F - Bone transplantation is effective in treatment of Congenital Thymic Aplasia.
False
What type of tranplantation is best for DiGeorge's?
Thymic Epithelium transplant

or

Fetal thymus within 14 wks of gestational age
What cells are absent or depressed in SCID?
B cells
What is the ADA (adenosine deanimase) catalyze?
Adenosine to Inosine
What does PNP (Purine Nucleoside Phosphorylase) catalyze?
Inosine to Hypoxanthine
For X-linked SCID with B cells, what is the gene mutation that is responsible?
Gene encoding gamma chain of IL-2 Receptor
What is the condition associated with the defect in tyrosine kinase ZAP70J?
Non-linked SCID with B-cells.
What is the best treatment with SCID patients?
Bone marrow transplant
Which severe disease has normal levels of B-cells (with possible normal function) AND normal levels of CD4 T-cells (but NO CD8 T-cells)
Non-X-linked SCID with B cells
X-linked SCID inhibits what developmental cells?
Progenitor T cells from becoming Pre-T cells
DiGeorge Syndrome inhibits what developmental cells?
Progenitor T cells from becoming Pre-T cells
A Tap1 and Tap2 deficiency (MHC Class II deficiency) inhibits what developmentally?
Double positive from going to single positive T cells.