Matthew Shared "Heme/Lymph 2" - 113 Picmonics
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Heme/Lymph 2
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
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
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
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
42 secs
Complement Disorders
- Early Complement Deficiency
- Pyogenic Infections
- SLE
- Sinus Infections
- Terminal Complement Deficiency
- Autosomal Recessive
- Neisseria Infections
- C5-C9
- MAC (Membrane Attack Complex)
4 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
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
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
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
Acute Myelogenous Leukemia (AML)
- Myeloblasts Increase on Peripheral Smear
- Median Onset 60 Years
- T 15;17 M3 Subtype
- Auer Rods
- Disseminated Intravascular Coagulation (DIC) is a Common Presentation
- M5 Gum Infiltration
- Responds to Vitamin A
3 mins
Chronic Myelogenous Leukemia (CML)
- Pathophysiology
- BCR-ABL
- 9;22 Translocation
- Philadelphia Chromosome
- Signs & Symptoms
- Tyrosine Kinase
- Low Alkaline Phosphatase
- Splenomegaly
- Considerations
- Blast Crisis
- Age 30 to 90 Years
- Responds to Imatinib
3 mins
Acute Lymphoblastic Leukemia (ALL)
- Ages < 15
- May Present with Bone Marrow Involvement in Children
- Mediastinal Mass in Adolescents
- TdT Positive
- CALLA Positive
- t(12;21) Better Prognosis
- Spread to CNS and Testes
- Most Responsive to Chemotherapy
2 mins
Chronic Lymphocytic Leukemia (CLL)
- Signs and Symptoms
- Age > 60 Years
- B Cell Neoplasm
- Smudge Cells
- Often Asymptomatic
- Cold IgM Autoimmune Hemolytic Anemia
- Warm IgG Autoimmune Hemolytic Anemia
- Richter's Transformation
3 mins
Hairy Cell Leukemia
- Pathophysiology
- Mature B Cell Tumor
- Signs and Symptoms
- Splenomegaly
- Diagnosis
- Flow Cytometry
- Cells Have Filamentous Hair-Like Projections
- Stains TRAP Positive
- Dry Tap on Bone Marrow Aspiration
- Treatment
- Cladribine
- Pentostatin
2 mins
Leukemia Assessment
- Cause
- Immature WBCs
- Assessment
- Recurrent Infections
- Easy Bruising
- Bleeding
- Anemia
- Fatigue
- Weight Loss
- Bone Pain
- Considerations
- May Have Normal or High WBC Count
2 mins
Leukemia Interventions
- Diagnosis
- Bone Marrow Aspiration
- X-Rays
- Interventions
- Chemotherapy
- Bleeding Precautions
- Neutropenic Precautions
- Strict Asepsis
- Bone Marrow Transplant
- Blood Transfusions
- Monitor Temperature Elevations
- Adequate Nutrition
- Imatinib (Gleevec) for Chronic Myelogenous Leukemia (CML)
3 mins
Auer Rods
- Azurophilic granular needles in leukemic blasts
- Disseminated Intravascular Coagulation (DIC)
- Acute Myelogenous Leukemia (AML)
- All-trans Retinoic Acid (Vitamin A)
1 min
Burkitt's Lymphoma
- Adolescents or young adults
- C-myc gene moves next to heavy chain Ig gene
- T 8;14
- Starry sky appearance
- Sheets of lymphocytes with interspersed macrophages
- Jaw lesion in endemic (African) form
- Pelvis or abdomen in non endemic form (American form)
- Associated with EBV
2 mins
Follicular Lymphoma
- Derives from Germinal Center
- Occurs in adults
- T14;18
- BCL 2 overexpression
- BCL 2 inhibits apoptosis
- Indolent
- Generalized lymphadenopathy
- Bone marrow involvement
2 mins
Mantle Cell Lymphoma
- Older Males
- Activation of Cyclin D Gene
- T 11;14
- CD5
- Poor Prognosis
55 secs
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