Emily Shared "Immuno Block 1 Disorders" - 18 Picmonics

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Immuno Block 1 Disorders

Transfusion Reactions
Acute Hemolytic
Febrile, Non-Hemolytic
Mild Allergic
Anaphylactic
Circulatory Overload
Sepsis Reaction
Transfusion-Related Acute Lung Injury (TRALI)
Massive Blood Transfusion
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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
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
Amyloidosis
Primary
Multiple Myeloma
Secondary
Tuberculosis (TB)
Rheumatoid Arthritis
Congo Red Stain
Apple Green Birefringence
Nephrotic
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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
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
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
Human Immunodeficiency Virus (HIV) Stages
Primary Infection
Within 3 Weeks
Flu-like Symptoms
Asymptomatic Stage
Years
Symptomatic Stage
CD4+ < 500
AIDS
Opportunistic Infections
CD4+ < 200
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2 mins
Hyper IgE Syndrome
Pathophysiology
Job's Syndrome
Abnormal Chemotaxis
Decreased IFN Gamma
Signs and Symptoms
Leonine Facies
Cold Non-Inflamed Staph Aureus Abscesses
Retained Primary Teeth
Eczema
Red Hair
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1 min
IL-12 Receptor Deficiency
Pathophysiology
IL-12 Secreted by Macrophages
Decreased Th1 Response
Decreased IFN Gamma
Signs and Symptoms
Mycobacterial Infections
Salmonella
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1 min
Chronic Mucocutaneous Candidiasis
Mechanism and Characteristics
T Cell Dysfunction
Candida Albicans
Signs and Symptoms
Infections of Skin
Infections of Mucous Membrane
Susceptible to HPV Infections like Condyloma Acuminatum
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42 secs
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
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
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
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
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
Paroxysmal Nocturnal Hemoglobinuria (PNH)
Pathophysiology
Defective PIG-A Gene
Deficiency of DAF (Decay Accelerating Factor CD55)
Symptoms
Dark Urine in the Morning
Intravascular Hemolysis
Thrombosis
Diagnosis
Decreased CD55 and CD59
Treatment
RBC Transfusion
Eculizumab
Bone Marrow Transplant is Curative
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3 mins

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