Unknown Facts in DIT Immunology

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CD Surface Marker:
found on all NK cells and macrophages

CD16

CD Surface Marker:
inhibits compliment C9 binding

CD55/59 aka decay accelerating factor
Mutation or absent in Paryoxysmal Nocturnal Hemoglobinuria

Cytokine:
growth and activation of eosinophils

IL-5

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

Immunoglobulin:
75% of the immuniglobulin pool

IgG

Immunoglobulin:
occurs as a dimer

IgA

Immunoglobulin:
Primarily intravascular, early Ig response

IgM

Immunoglobulin:
distributed evenly intravascular and extravascularly

IgG

Immunoglobulin:
can be a pentamer

IgM

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?

hemosiderinuria
chronic intravascular hemolysis
thrombosis
+ Ham's test (RBC's lyse at low pH)
Rx: Iron + warfarin, bone marrow transplant

Erythoroblastosis Fetalis?

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:
PSGN -
Asthma -
Polyarteritis nodosa -
ABO Blood incompatibility -
Eczema -

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

Arthus reaction?

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
Polymyalgia rheumatica
temporal arteritis
infection/inflammation
malignancy

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
Increased AFP

ataxia-telangiectasia

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 Eczema, cold s. aureus abscesses, coarse facial features, 2 rows of teeth

Job's Syndrome

Child with thrombocytopenia, eczema, recurrent pyogenic diseases

Wiscott Aldrich syndrome
also decreased IgM, increased IgA

NADPH Oxidase deficiency results in susceptibility to what diseases?

Catalase+ organisms
S. Aureus, pseudomonas, E. Coli
Aspergillus, Klebsiella, Candida

Patient suffers from recurrent neiserria infections
What is deficient?

C5-C9
no membrane attack complex (MAC)

After bone marrow transplant, patient suffers dermatitis, enteritis, hepatitis - diagnosis?

Graft vs. Host disease

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