Immune System Flashcards
Post-splenectomy
Howell-Jolly bodies
target cells
thrombocytosis
Positive Selection
thymus, cortex
MHC restriction
Negative Selection
thymus, medulla
non reactive to self
HLA A3
Hemochromatosis
HLA-DQ2/8
Celiac Disease
HLA-DR5
pernicious anemia/b12 def
hashimoto’s thyroidits
NK cells
induce apoptosis of virally infected and tumor cells
Th1/Th2 secretes
Th1 - ifn gamma
Th2 - IL-4,5,10,13
Th1/Th2 activates
th1 - macrophages
th2 - eosinophil, Ige production
Th1 inhibited by
IL4 and IL10
th2 inhibited by
IFN-gamma
SCID
infant, agammaglobulenima, lymphopenia
SCID cause
defective IL-2 receptor
adenosine deaminase def
X-linked
SCID treatment
BMT
Wiskott-Aldrich Syndrome
Thrombocytopenia
infections
Eczema
combined B and T cell def
cause of Wiskott-Aldrich Syndrome
X linked, WAS gene
T cells unable to reorganize actin skeleton
Ataxia-telangiectasia
cerebellar defects - ataxia
spider angiomas - telangiectasia
IgA def
increased AFP
Ataxia-telangiectasia cause
defects in ATM gene, codes for DNA repair
Hyper IgM sydrome
severe pyogenic infections early in life defective CD40L on Helper T cells/inability to class switch
Leukocyte adhesion def
recurrent bacterial infections
perstistent neutrophilia
absent pus
delayed separation of umbilical cord
Cause of Leukocyte adhesion def
defect in LFA-1 integrin on phagocytes
Chediak-Higashi syndrome
recurrent infections - catalase positive
AR
microtubule dysfunction in phagosome-lyosome fusion
Chronic Ganulomatous disease
boy
catalase + infections
Abnormal DHR flow cyto test or nitroblue tetrazoliume
catalse poisitve organisms
S aeures, E coli
Serratia
Aspergillus
Chronic Granulomatous dz causes
lack of NADPH oxidase –> decrease of ROS
X-linked agammaglobulinemia
infant boy, 4-6 months
encapsulated organism sepsis
absent B cells
cause of X linked agammaglobulinemia
X linked recessive/defect in BTK/no B cell maturation