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CD Surface Marker:
inhibits compliment C9 binding
CD55/59 aka decay accelerating factor
Mutation or absent in Paryoxysmal Nocturnal Hemoglobinuria
VDJ recombination is initiated by what gene?
Recombination activating gene complex (RAG)
RAG-1 and RAG-2
Mutations in RAG arrest B and T cell development
What happens when you are deficient in CD55/59?
paroxysmal nocturnal hemoglobinuria
no DAF, meaning complement lyses own red blood cell
Results in iron deficiency anemia
Symptoms of paroxysmal nocturnal hemoglobinuria
What is Ham's Test?
Treatment for PNH?
chronic intravascular hemolysis
+ Ham's test (RBC's lyse at low pH)
Rx: Iron + warfarin, bone marrow transplant
Maternal anti Rh-D antigen to baby
Results in hemolysis of the newborn
Can lead to hydrops fetalis, IU death
Tx: Anti-Rh-D immunoglobulin
Type of hypersensitivity:
Polyarteritis nodosa -
ABO Blood incompatibility -
Type of hypersensitivity:
PSGN - 3
Asthma - 1
Polyarteritis nodosa - 3
ABO Blood incompatibility - 2
Eczema - 1
What is serum sickness?
Immune complex disease type III
antibodies to foreign proteins produced (takes 5 days)
Immune complex deposit into membranes and fix complement leading to tissue damage
Sx - fever, urticaria, arthralgia, proteinuria, lymphadenopathy
Local type III hypersensitivity
Intradermal injection of antigen induces antibodies
Sx - edema, necrosis, activation of complement
Often following vaccinations
Conditions with elevated ESR
Disease activity in RA/SLE
Young child with tetany from hypocalcemia, candidiacsis from immunosuppression
What cell type is deficient?
Digeorge - T cells
Child has immune disorder -> repeated staph. abcesses -> neutrophils fail to respond to chemotactic stimuli
Job Syndrome (Hyper IgE)
Deficiency in IFN-g because PMNs fail to respond to C5a/LTB4
IgA deficiency, poor smooth pursuit with eyes
telangiectasias of face
Increased lymphoma leukemia risk
Chediak Higashi Disease
Defective LYST gene (lysosomal transport)
Giant cytoplasmic granules in PMNs diagnostic
Triad - partial albinism, neurologic disorders, recurrent respiratory tract and skin infections
Child with thrombocytopenia, eczema, recurrent pyogenic diseases
Wiscott Aldrich syndrome
also decreased IgM, increased IgA
NADPH Oxidase deficiency results in susceptibility to what diseases?
S. Aureus, pseudomonas, E. Coli
Aspergillus, Klebsiella, Candida
Patient suffers from recurrent neiserria infections
What is deficient?
no membrane attack complex (MAC)
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