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
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
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
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
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
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
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
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
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
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
2 mins
Graves' Disease Characteristics
- Women 20-40 years old
- Pathophysiology
- Anti-TSH Receptor Antibodies
- Symptoms
- Ophthalmopathy
- Exophthalmos
- Hyperthyroidism
- Goiter
- Pretibial Myxedema
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
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
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
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
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
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
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
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
3 mins
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