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49 Cards in this Set

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what is the defect in Bruton's agammaglobulinemia?
X-linked recessive defect in a tyrosine kinase gene associated with low levels of all classes of Igs
this disorder occurs in boys and is associated with recurrent bacterial infections after 6 months of age, when levels of maternal IgG antibody decline
Brutons agammaglobulinemia (B cells)
this immune deficiency presents with tetany owing to hypocalcemia and recurrent viral and fungal infections
DiGeorge syndrome/thymic aplasia (T cells)
this immune deficiency is associated with congenital defects of heart and great vessels
DiGeorge syndrome
what ist eh chromosomal abnormality associated with DiGeorge syndrome?
22q11 deletion
this is a defect in early stem cell differentiation and presents with recurrent viral, bacterial, fungal, and protozoal infections
SCID - B and T cell deficiency
failure to synthesize MHC II antigens, defective IL-2 receptors, and adenosine deaminase deficiency can all lead to what immune deficiency?
SCID
what does IL-12 receptor deficiency (decreased activation of T cells) present with?
disseminated mycobacterial infections
defect in CD40 ligand on CD4 T helper cells leads to inability to class switch; presents early in life with severe pyogenic infections
hyper-IgM syndrome (decreased activation of B cells)
this disorder is characterized by high levels of IgM and very low levels of IgG, IgA, and IgE
hyper-IgM syndrome
this syndrome is characterized by an X-linked defect in the ability to mount an IgM response to capsular polysaccharides of bacteria
Wiskott-Aldrich syndrome (decreased activation of B cells)
what is the triad of symptoms associated with Wiskott-Aldrich syndrome?
infections, thrombocytopenic purpura, eczema (WIPE)
this syndrome is associated with elevated IgA levels, normal IgE levels, and low IgM levels
Wiskott-Aldrich syndrome
this syndrome presents with recurrent 'cold' (noninflamed) staphylococcal abscesses, eczema, coarse facies, retained primary teeth, and high IgE levels
Job's syndrome (decreased activation of macrophages)
this syndrome is characterized by failure of gamma-interferon production by helper T cells; neutrophils fail to respond to chemotactic stimuli
JOb's syndrome
what is the defect in leukocyte adhesion deficiency syndrome?
defect in LFA-1 adhesion proteins on phagocytes
this syndrome presents early with severe pyogenic and fungal infections and delayed separation of umbilicus
leukocyte adhesion deficiency syndrome
how is Chediak-Higashi inherited?
autosomal recessive
this disease is marked by a defect in microtubular function and lysosomal emptying of phagocytic cells
Chediak-Higashi disease
this disease presents with recurrent pyogenic infections by staph and strep, partial albinism, and peripheral neuropathy
Chediak-Higashi disease
defect in phagocytosis of neutrophils owing to lack of NADPH oxidase activity or similar enzymes
chronic granulomatous disease
how is the diagnosis of chronic granulomatous disease confirmed?
negative nitroblue tetrazolium dye reduction test
this disease presents with marked susceptibility to opportunistic infections with bacteria, especially S. aureus, E. coli, and Aspergillus
chronic granulomatous disease
what is the defect in chronic mucocutaneous candidiasis present?
idiopathic dysfunction of T cells specifically against candida albicans
what is the most comon selective immunoglobulin deficiency? how does it present?
selective IgA deficiency - presents with sinus and lung infections; milk allergies and diarrhea are common
ataxia-telangiectasia is an idiopathic dysfunction of what type of cells?
B cells
defect in DNA repair enzymes with associated IgA deficiency; presents with cerebellar problems and spider angiomas
ataxia-telangiectasia
deficiency of what leads to hereditary angioedema?
C1 esterase inhibitor
deficiency of which complement protein leads to severe, recurrent, pyogenic sinus and RT infections?
C3
deficiency of what complement proteins leads to Neisseria bacteremia?
C6-C8
deficiency of what leads to paroxysmal nocturnal hemoglobinuria?
decay-accelerating factor
patients with what deficiency have an increased susceptibility to recurrent bacterial infections, especially with encapsulated bacteria
C3 deficiency; not detected until later in life
what is the most important immunological protective mechanism against blood-borne encapsulated organisms?
IgG-mediated opsonization in the spleen
Warthin-Finkeldy giant cell is pathognomonic for what?
measles or the live attenuated measles vaccine
Lyme arthritis is associated with what HLA?
HLA-DR4
patients with Wiskott-Aldrich syndrome have a 12% chance of developing what?
non-Hodgkin's lymphoma
test of choice to determine presence of circulating anti-Rh antibody?
indirect Coombs test to measure IgG anti-Rh antibody
low levels of all antibody classes
common variable immunodeficiency
spleen is important for removing what type of organisms? list 3
strep pneumo, H. flu, Neisseria
what is defective in leukocyte adhesion deficiency?
integrins - function both in adhesion of leukocytes to other cells and in the phagocytosis of complement-coated material
HLA types associated with SLE?
HLA-DR2 and HLA-DR3
most likely sequelae of rheumatic fever?
mitral valve disease
antitopoisomerase antibodies?
scleroderma/systemic fibrosis - likely to develop diffuse systemic fibrosis & death from consequences of systemic disease such as pulmonary fibrosis or malignant hypertension
what is responsible for strong binding between monocytes, T lymphocytes, macrophages, neutrophils, and dendritic cells, and injured epithelium?
LFA-1; interacts with ICAM-1
an increased level of what cytokine would decrease the likelihood of a delayed-type hypersensitivity reaction?
IL-10
what 2 molecules exert the most powerful chemotactic effect on neutrophils?
C5a and C8
what are the best markers for identification of B cells?
CD19, CD20, CD21
IgG subclass deficiency is associated with a deficiency with what other substrate?
IgA
what is C-reactive protein a marker of?
non-specific inflammation - one of the most commonly measured acute-phase reactants