Reading...
Play button
Play button
Use LEFT and RIGHT arrow keys to navigate between flashcards;
Use UP and DOWN arrow keys to flip the card;
H to show hint;
A reads text to speech;
51 Cards in this Set
- Front
- Back
|
What immunodeficiency is present in all infants at 3-6 months?
|
Transient Hypogammaglobulinemia due to catbolism of placentally transferred IgG
|
|
|
B cell tyrosine kinase (btk) lesion
|
X-linked Agammaglobulinemia of Bruton
|
|
|
Failure of B-cell precursors to mature into B cells
|
X-linked Agammaglobulinemia of Bruton
|
|
|
Heavy chains, but NOT light chains of Ab are formed
|
X-linked Agammaglobulinemia of Bruton
|
|
|
When does X-linked Agammaglobulinemia of Bruton manifest?
|
6 months (maternal Igs have been depleted)
|
|
|
Bacterial infections of the URT/LRT of what are common in X-linked Agammaglobulinemia of Bruton?
|
H. influenzae, S. pneumoniae, S. aureus
|
|
|
Why do H. influenzae, S. pneumoniae, S. aureus cause infections in X-linked Agammaglobulinemia of Bruton?
|
They are encapsulated bacteria that need to be opsonized by Ab to be removed. Without Ab, no opsonization.
|
|
|
What viruses, not handled well by T cells, may cause a problem in X-linked Agammaglobulinemia of Bruton?
|
Enteroviruses (echo, polio, coxsackie). They require Ab to neutralize them.
|
|
|
In what diseases may Giardia lamblia cause persistent infections, and why?
|
X-linked Agammaglobulinemia of Bruton because there is an absence of secreted IgA and in CVID
|
|
|
Mycoplasma arthritis in kids
|
X-linked Agammaglobulinemia of Bruton
|
|
|
Normal CD19, but no surface Igs
|
X-linked Agammaglobulinemia of Bruton
|
|
|
Rudimentary or underdeveloped germinal centers
|
X-linked Agammaglobulinemia of Bruton
|
|
|
ZERO plasma cells
|
X-linked Agammaglobulinemia of Bruton
|
|
|
What makes CVID different from Bruton?
|
CVID is a later onset, normal # of B cells, and affects both sexes =
|
|
|
B cells can proliferate in response to antigen, but do not produce antibodies
|
CVID
|
|
|
Infections similar to Bruton, with herpetic also
|
CVID
|
|
|
Enteroviral infections causing meningoecncephalitis
|
CVID
|
|
|
50 fold > in gastric carcinoma
|
CVID
|
|
|
Fairly common ID
|
Isolated IgA
|
|
|
Post-infectious (measles or toxoplasmosis)
|
Isolated IgA
|
|
|
What are the sx in Isolated IgA?
|
Most are asymptomatic
|
|
|
Where do you see infections in Isolated IgA?
|
tubes
|
|
|
High frequency of respiratory allergies
|
Isolated IgA (lack of IgA in mucous secretions)
|
|
|
CD40 is on what cell?
|
B cell
|
|
|
What Igs are on all naive B cells?
|
M and D
|
|
|
What is the cause for Hyper IgM
|
Abnormal T cells fail to induce B cells to make Ab other than IgM
|
|
|
What is the mutation in Hyper IgM?
|
CD40L on Xq26
|
|
|
Why are there pyogenic infections in Hyper IgM?
|
opsonizing IgG is low
|
|
|
P. jiroveci pneumonia
|
Hyper IgM and SCID
|
|
|
DiGeorge Syndrome is the failure of what to develop?
|
3rd, 4th pharyngeal pouches
|
|
|
What is the consequence of thymic agenesis in DiGeorge Syndrome?
|
poor defense against fungal and viral infections
|
|
|
Tetany
|
DiGeorge Syndrome (parathyroid agenesis)
|
|
|
Facial anomalies (big nose, cleft palate, retrognathia)
|
DiGeorge Syndrome
|
|
|
Is DiGeorge Syndrome a familial disorder?
|
NO
|
|
|
Where is the deletion in DiGeorge Syndrome?
|
22q11
|
|
|
CATCH (DiGeorge)
|
Cardiac abnormality
Abnormal facies Thymic/T cell deficit Cleft Palate Hypocalcemia |
|
|
What would the plasma cell level in lymphoid tissue be in DiGeorge Syndrome?
|
Normal
|
|
|
What would the Ig level be in DiGeorge Syndrome?
|
Normal to reduced (depending upon the severity of T cell (CD4) deficiency
|
|
|
Where would you see lymphoid depletion in DiGeorge Syndrome?
|
Paractorical region of LN, periarteriolar sheaths of spleen
|
|
|
Where is there always hypoplasia in SCID?
|
T cell zone (may be B cell zone too)
|
|
|
Defects in both humoral and cell-mediated immune responses
|
SCID
|
|
|
oral thrush, diaper rashes, failure to thrive
|
SCID
|
|
|
Rash on a newborn due to GVH
|
SCID
|
|
|
Gamma-c SCID has a defect in a receptor for what? (Most important)
|
IL-7 (required for the lymphoid progenitors, particularily T cells)
|
|
|
What does an ADA deficiency produce?
|
accumulation of deoxyadenosine which is toxic to immature lymphocytes, esp T cells
|
|
|
What is the Dx triad of Wiskott-Aldrich?
|
Thrombocytopenia, eczema and recurrent infections (early death)
|
|
|
What would the serum Ig level in WA?
|
Low IgM, normal IgG, IgA and IgE > some
|
|
|
What is the significance of a low IgM in WA?
|
poor response to bacterial polysaccharides
|
|
|
Do not make abys to bacterial polysaccharide antigens?
|
WA
|
|
|
Where is the lesion in WA?
|
Xp 11.23 --> No WASP (CD43)
|
|
|
Those with WA have a tendency to develop what?
|
lymphomas
|
