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Jake Shared "Immuno 10/30" - 19 Picmonics

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Immuno 10/30

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
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
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
Thymic Aplasia (DiGeorge Syndrome)
Pathophysiology
DiGeorge Syndrome
22q11 Deletion
Failure to Develop Third and Fourth Pharyngeal Pouches
Signs and Symptoms
Undeveloped Thymus
T Cell Deficiency
Recurrent Viral and Fungal Infections
Undeveloped Parathyroids
Hypocalcemia
Tetany
Aortic Defects
Congenital Heart Defects
Diagnosis
Absent Thymic Shadow on CXR
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2 mins
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
Multiple Sclerosis Features and Mechanisms
Features
More Common in Women
Presents in 20's and 30's
Northern Europeans
Mechanism
Unknown mechanism, genetic factor
HLA-DRB1
Autoimmune Demyelination of CNS
White Matter of Brain and Spinal Cord
T Cell Mediated Inflammation
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2 mins
Hyper IgE Syndrome
Job Syndrome (Autosomal Dominant Hyper IgE Syndrome)
Pathophysiology
Autosomal Dominant
STAT3 Mutation
Increased IgE
Abnormal Chemotaxis
Signs and Symptoms
Leonine Facies
Cold (Non-Inflamed) Staph Aureus Abscesses
Retained Primary Teeth
Eczema
Management
Prophylactic Antibiotics
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2 mins
Bruton's Agammaglobulinemia
Pathophysiology
X-linked Recessive
Defective Bruton Tyrosine Kinase
Blocks B Cell Maturation
Signs & Symptoms
Decreased Immunoglobulins of All Classes
Bacterial Infections After Six Months
Maternal IgG Protects Before Six Months
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2 mins
Ataxia Telangiectasia
Pathophysiology
Autosomal Recessive
ATM Gene
Defect in DNA repair
Signs & Symptoms
Cerebellar defects
Spider Angiomas
Sensitive to Radiation
Defective Tumor Suppressor Gene
Increased risk of Cancer, Leukemias, and Lymphomas
Increased AFP levels
IgA deficiency
Repeated sinopulmonary infections
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2 mins
Graves' Disease Characteristics
Women 20-40 years old
Pathophysiology
Anti-TSH Receptor Antibodies
Symptoms
Ophthalmopathy
Exophthalmos
Hyperthyroidism
Goiter
Pretibial Myxedema
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1 min
Hashimoto's Thyroiditis
Symptoms
Hypothyroidism with Bouts of Hyperthyroidism
Enlarged, Nontender Thyroid
Mechanism
Autoimmune
Anti-Thyroid Peroxidase (TPO)
Anti-Thyroglobulin
Lab Findings
Hurthle Cells
Lymphoid Follicles
Associations
HLA-DR5 and HLA-DR3
Increased Risk Of Non-Hodgkin Lymphoma
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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
Transplant Rejection
Hyperacute
Occurs within Minutes
Antibody Mediated Due to Preformed Antidonor Antibodies
Acute
Occurs within Weeks
Cytotoxic T Lymphocytes Cause Cell-mediated or Antibody-mediated Response
Chronic
Occurs Months to Years Later
Antibody and T Cell-mediated Vascular Damage
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2 mins
Chediak-Higashi Syndrome
Pathophysiology
Autosomal Recessive
Defective Lysosomal Trafficking Regulator Gene (LYST)
Defect In Microtubular Function
Decrease In Phagocytosis
Signs And Symptoms
Recurrent Pyogenic Infections
Staph Aureus And Streptococci
Partial Albinism
Peripheral Neuropathy
Pancytopenia
Diagnosis
Giant Granules In Granulocytes And Platelets
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2 mins
Wiskott Aldrich
Pathophysiology
X-linked Recessive
WASp Protein Mutation
Antigen Presentation Defect
Progressive Deletion of B and T Cells
Signs and Symptoms
Thrombocytopenia
Eczema
Infections
Decreased IgM
Increased IgE, IgA
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2 mins
Adenosine Deaminase Deficiency
Adenosine to Inosine
Excess ATP
Inhibition of Ribonucleotide Reductase
Prevents DNA Synthesis
Decreases B Cells and T Cells
Major cause of SCID
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1 min
Cold Agglutinin Autoimmune Hemolytic Anemia (C-AIHA)
Pathophysiology
IgM Binds to RBCs at Cold Temperatures (28-31C)
Extravascular Hemolysis
Waldenstrom's Macroglobulinemia
Mycoplasma Pneumoniae
Infectious Mononucleosis
Lymphomas and CLL
Symptoms
Acrocyanosis with Cold Exposure
Blue Fingers and Toes
Diagnosis
Positive Coombs Test
Treatment
Avoid Cold
Rituximab
+/- Fludarabine
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4 mins
Warm Agglutinin Autoimmune Hemolytic Anemia (W-AIHA)
Pathophysiology
Most Common Autoimmune Hemolytic Anemia
IgG Coats RBCs at Warm Temperatures
Fc Portion of IgG Recognized By Macrophages
Extravascular Hemolysis
Medications
Alpha Methyldopa
Chronic Lymphocytic Leukemia (CLL)
Systemic Lupus Erythematosus (SLE)
Diagnosis
Positive Coombs Test
Treatment
Corticosteroids
Splenectomy
Intravenous Immunoglobulin (IVIG)
Rituximab
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3 mins

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