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Matthew Shared "BFOM Test 3 - Immunodeficiency" - 10 Picmonics

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BFOM Test 3 - Immunodeficiency

Chronic Granulomatous Disease
Pathophysiology
Most Commonly X-linked Recessive
Defective NADPH Oxidase (Enzyme Complex)
Decreased Hydrogen Peroxide
Impaired Intracellular Killing in Phagocytes
Signs and Symptoms
Recurrent Purulent Skin and Lung Infections
Catalase Positive Organisms
Diagnosis
Negative Nitroblue Tetrazolium Test
DHR (Dihydrorhodamine) Flow Cytometry
Treatment
Antimicrobial Prophylaxis
TMP-SMX
Itraconazole
Interferon-Gamma
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3 mins
Leukocyte Adhesion Deficiency
Pathophysiology
Autosomal Recessive
Integrin CD18 Protein
Defect in LFA-1
Attached to ICAM
Signs and Symptoms
Neutrophilia
Bacterial Infections
Non-Pyogenic
Delayed Separation of Umbilical Cord
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2 mins
Hyper IgM Syndrome
Pathophysiology
Deficient CD40 Ligand on Helper T-cells
Defective CD40 Receptor on B-cells
Inability to Class Switch
Signs and Symptoms
Pyogenic Abscess
Decreased IgG, IgA, IgE
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4 mins
Common Variable Immunodeficiency
Pathophysiology
Defect in B Cell Maturation
Decreased Plasma Cells
Decreased Immunoglobulins
Signs & Symptoms
Sinopulmonary Infections
Increased Risk of Autoimmune Disease
Lymphoma
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1 min
Selective IgA Deficiency
Pathophysiology
Defect in Class Switching
Decreased IgA
Signs and Symptoms
Often Asymptomatic
Increase in Atopic & Autoimmune Diseases
Sinopulmonary Infections
Increased Risk Giardia Lamblia Infections
Inflammatory Bowel Disease
Celiac Disease
Considerations
Anaphylaxis From IgA Containing Blood Products
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2 mins
Severe Combined Immunodeficiency (SCID)
Both B and T Cell Deficiency
Recurrent Viral, Bacterial, Fungal, Protozoal Infections
Defective Interleukin (IL) 2 Receptor
X-Linked
Adenosine Deaminase Deficiency
Failure to Synthesize MHC II Antigens
Treat with Bone Marrow Transplant
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2 mins
Hypersensitivity Type I
Previous Antigen Exposure
Immediate Reaction
IgE Cross-linking
Presensitized Mast Cells
Histamine Release
Vasodilation
Anaphylaxis
Allergies
Treatment
Epinephrine
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1 min
Hypersensitivity Type II
Cytotoxic
IgM or IgG antibody-mediated
Membrane Attack Complex (MAC)
Mechanism
Opsonization
Complement Activation
NK Cells
Testing
Direct Coombs test
Indirect Coombs Test
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1 min
Hypersensitivity Type III
Immune Complex
Antigen-antibody (IgG) complex activates complement
Neutrophils release lysozomal enzymes
Examples
Arthus Reaction
Serum Sickness
Systemic Lupus Erythematosus (SLE)
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1 min
Hypersensitivity Type IV
Delayed-Type Hypersensitivity
T Cell Mediated
Antibody Independant
Macrophages Activated
Examples
Transplant Rejection
PPD Skin Test
Contact Dermatitis
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1 min

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