Immunopathology Flashcards
(34 cards)
DiGeorge Syndrome
No Development of third and fourth pharyngeal pouches
22q11 microdeletion
T-cell deficiency–no thymus
Hypocalcemia– lack parathyroids
Abnormalities of heart/great vessels/face
Severe Combined immunodeficiency
Defective cell-medicated and humoral immunity Cytokine receptor defects Adenosine deaminase (ADA) deficiency-- build up of adenosine and deoxyadenosine MHC class II deficiency--necessary for CD4 helper T cell activation and cytokine production
X-Linked Agammaglobulinemia
Complete lack of immunoglobulin due to disordered B-cell maturation
Mutated Bruton tyrosine kinase
Presents after 6 months of life due to antibodies present during first 6 months from mother for protection
Common variable immunodeficiency
Low immunoglobulin due to B-cell or helper T-cell defects
B-cells- Igs
T-cells- IL-4 and 5
Increased risk for autoimmune dz and lymphoma
IgA deficiency
Low serum and mucosal IgA
increased risk for mucosal infection–especially viral
Wiskott-Aldrich syndrome
Thrombocytopenia/eczema/recurrent infections
Bleeding is a major cause of death
Mutation in the WASP gene
Complement deficiencies
C5-C9–increased risk for Neisseria infection
C1 inhibitor- angioedema–edema of the skin and mucosal surfaces
Hyper-IgM syndrome
Elevated IgM
Mutated CD40L (On helper T cell) or CD40 receptor (on B cells)
B-cells cannot be activated–cytokines for class switching are not pro ducted (IL-4 and 5)
Low IgA/IgG/IgE– result in recurrent pyogenic infections–poor opsonization
What activates Caspase-1 and what does this lead to?
NLRs which cause Caspase-1 to release IL-1 and IL-18
This attracts immune cells to site of infection and IL-1 causes Fever
What role does IL-6 play in innate immunity?
increase synthesis and release of hepcidin which decreases reabsorption of Fe in duodenum and prevents release from macrophages—-gets Fe away from bacteria
What is Type I immediate hypersensitivity?
Triggered by binding of an antigen to IgE antibody on surface of Mast cell/basophil
What is Type II hypersensitivity?
Antibody directed against antigens on cell membrane or in ECM
What is autoimmune hemolytic anemia?
Type II hypersensitivity
- -Target–RBC membrane protein
- -hemolysis and anemia
What is autoimmune thrombocytopenia purpura?
Type II hypersensitivity
- -Target– Platelet membrane proteins
- -bleeding
What is Pemphigue vulgaris?
Type II hypersensitivity
- -target– proteins in intercellular junctions
- -Skin vesicles
What is Vasculitis caused by ANCA?
Type II hypersensitivity
- -target–Neutrophil granule proteins
- -Vasculitis
What is Goodpasture syndrome?
Type II hypersensitivity
- -target– Noncollagenous protein in BMs of kidney glomeruli and lung alveoli
- -Nephritis/lung hemorrhage
What is acute rheumatic fever?
Type II hypersensitivity
- -target–streptococcal cell wall antigen–antibody cross-reacts with myocardial antigen
- -myocarditis/arthritis
What is Myasthenia gravis?
Type II hypersensitivity
- -target–acetylcholine receptor
- -muscle weakness/paralysis
What is graves dz?
Type II hypersensitivity
- -target–TSH receptor
- -hyperthyroidism
What is insulin-resistant diabetes?
Type II hypersensitivity
- -target–Insulin receptor
- -Hyperglycemia/ketoacidosis
What is Pernicious anemia?
Type II hypersensitivity
- -Target–intrinsic factor of gastric parietal cells–decreased absorption of B12
- -abnormal erythropoiesis/anemia
What is Systemic lupus erythematosus?
Type III hypersensitivity
Antigen–Nuclear–circulating or planted in kidney
Nephritis/skin lesions/arthritis
What is post streptococcal glomerulonephritis?
Type III hypersensitivity
Antigen–streptococcal cell wall–may be planted in glomerular basement membrane
Nephritis
