Advanced Pathophysiology Midterm exam 2024 Question and Answers

Advanced Pathophysiology Midterm exam 2024 Question and Answers Primary immunodeficiency -less common and occur in result of single gene defects (defect on the development of the immune system) -this could involve antibody deficiencies, B- and T- cell deficiencies, defects in the phagocytic cells and deficiency of complement -something is lacking with the immune system Ex: B-lymphocyte deficiency is one of the most common forms of primary immunodeficiency Examples of primary immunodeficiency -Chronic Granulomatous Disease of Childhood -DiGeorge Syndrome -Familial Mediterranean fever -Job Syndrome -Common Variable Immunodeficiency Secondary Immunodeficiency -conditions where the immune system becomes compromised because of a complication of some other physiological condition or disease -can be caused by cancer, effect from a drug (chemotherapeutic agents that suppress immune system), and infections that compromise the immune system Ex: Patient with HIV gets pneumocystis carinii What is is a predominant cause of secondary immune deficiencies worldwide? -malnutrition Examples of secondary immunodeficiency -Pneumocystis Carinii -HIV -PNA -Sinus infection -Lung cancer Hypersensitivity Type I - allergic reaction -mediated by IgE -mast cells are the primary effector cells involved -inflammation due to mast cell degranulation Hypersensitivity Type I symptoms Local: itching, rash Systemic: wheezing Hypersensitivity Type I example Most dangerous form: anaphylactic reaction -> systemic response -> hypertension -> severe bronchoconstriction Treatment: epinephrine reverses the effects Hypersensitivity Type II -cytotoxic reaction -tissue/organ specific -macrophages are primary effector cells involved -can cause tissue damage or alter function Mechanism: Tissue-specific destruction or impairment because of: 1. Antibody binding followed by lysis via complement 2. Antibody binding followed by macrophage phagocytosis 3. Antibody binding followed by neutrophil destruction 4. Antibody-dependent cell (NK)-mediated cytotoxicity 5. Antireceptor antibodies Hypersensitivity Type II examples 1. Grave's disease (hyperthyroidism): altering thyroid function, but does not destroy thyroid tissue 2. Incompatible blood type (ABO incompatibility): cell/tissue damage occurs -severe transfusion reaction -> transfused erythrocytes destroyed by agglutination or complementmediated lysis 3. Drug allergies 4. Hemolytic anemia Graves disease -Autoantibodies specific for thyroid tissue impair receptor for TSH ABO incompatibility -Complement damages RBC membrane and cells lyse Hypersensitivity Type III -NOT organ specific -antibody binds to soluble antigen outside the cell surface that was released into the blood of body fluids -> complex is then deposited in the tissues -organ rejection involved cytotoxicity -antigens from target cells stimulate T-cells to differentiate into cytotoxic T-cells -neutrophils are the primary effector cells Raynaud's phenomenon -Complex deposited in small peripheral vessels in cool temperatures leading to vasoconstriction and blocked circulation Hypersensitivity Type III examples 1. Rheumatoid arthritis: antigen/antibodies are deposited in the joints 2. Systemic Lupus Erythematosus (SLE): antigen/antibodies deposit in organs that cause tissue damage 3. Serum sickness 4. Raynaud's phenomenon Systemic Lupus Erythematosus (autoimmune response) -facial rash confined to cheeks (malar rash) -discoid rash (raised patches, scaling) -photosensitivity (rash developed as a result to light exposure) -oral or nasopharyngeal ulcers -hematologic disorders -immunologic disorders -non-erosive arthritis of at least 2 peripheral joints -serositis (pleurisy, pericarditis) -renal disorder -neurologic disorder -presence of antinuclear antibody (ANA) Autoimmunity -reaction of immune response to one's own tissues -can be familial Autoimmunity examples -Systemic Lupus Erthyematosus -Rheumatoid Arthritis