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module 8 study tool - Coggle Diagram

- - - - decreased MCV, MCH, MCHC
      - secondary leukopenia and thrombocytopenia
      - dark urine
      - elecrtophoresis
        
        will show deficient beta chain production
        
        in adult: 0% A, 90% F, less than 2.5% A2
    - - has moderate microcytic hypochromic anemia and shows target cells, basophilic stippling and nRBC's
  - - - halotype (--/--)
        
        not compatible with life
      - severe anemia, microcytic, hypochromic, elevated reticulocytes, elevated nRBC's
      - dangerous for mom and fetus
    - - 1 unaffected alpha chain, one deleted
        
        (--/alpha, alpha)
      - asymptomatic
      - you can find microcytic, hypochromic anemia, Hgb H inclusions
      - electrophoresis in newborns shoe 5-6% barts
      - can be masked by iron deficiency anemia
    - - only 1/4 of the chain genes are missing
      - asymptomatic
      - can find borderline low MCV, slight microcytosis
      - genetic analysis is the only reliable diagnostic tool
    - - halotype: (--/-alpha)
      - Barts produced at birth with y chain production
      - at 6 months changes to Hgb H
      - chronic hemolysis/ anemia, splenomegaly, 50% chance of skeletal changes, Hgb=8-10 g/dL, elevated retics, nrbcs, inclusions can be seen with brilliant cresyl blue
      - electrophoresis
        
        adult: 40% hgb H
        
        infant 25% bart hgb
- - - - symptoms: splenomegaly
      - electophoresis: a band at s and one at A2
      - morphology: Microcytic, hypochromic, nucleated RBCs, Reticulocytes, target cells, polychromasia, & Sickle cells
    - - morphology: microcytic, hypochromic, polychromasia, target cells
      - symptoms: asymptomatic
      - electrophoresis: strong band in HgbC/A2 position
    - - both chains are abnormal
        
        codes for beta s and c chains
        
        hemoglobin A is absent
      - "mild" sickle cell disease
        
        sickling and Hgb C crystals are there
        
        vaso occlusive crises
        
        splenomegaly
      - normochromic/normocytic anemis
      - solubility test is positive
      - in the electrophoresis Hgb s and c move the same amount
        
        F is normal
    - - morphology: few target cells
      - symptoms: asymptomatic
      - electrophoresis
        
        acidic: moves to A
        
        alkaline: moves to a band at s and one at A2
    - - hemoglobin crystals (bar of gold)
      - mainly known in African people
      - decreased life span of RBC (30-55 days)
      - joint and abdominal pain
      - enlarged spleen
      - the trait is asymptomatic
      - no treatment
      - normochromic, normocytic anemia
      - increased target cells
      - 2-3% reticulocytes
      - negative solubility test
      - Definitive Dx by electrophoresis, HPLC, and nucleic acid testing
      - both chains are abnormal
        
        creates beta s and beta c chains
        
        no hemoglobin a
    - - symptoms
        
        homozygous: mild hemolytic anemia and splenomegaly
        
        heterozygous: asymptomatic
      - electrophoresis
        
        alkaline: moves with C, E, and A2
        
        acidic: moves to A position
      - morphology: target cells
  - - - most common: africa
      - other places: mediterranean, middle east, india, nepal
    - - net negative charge made
      - deoxyhemoglobin s molecules polymerize
        
        creates the sickle cell shape
        
        85-38% oxygen saturation
        
        happens in hypoxic, acidotic, hypertonic microenvironment
      - anemia
    - - symptoms: hemolytic anemia, Vaso occlusion of microvasculature, infections, acute splenic sequetration
      - peripheral smear: poik, aniso, polychromasia
        
        5-20% reticulocytes
        
        variety of cells but diagnosis would be the sickle shaped cells
      - increased: bilirubin, uric acid, lactic dehydrogenase
      - decreased haptoglobin
    - - Adults: screening solubility test
      - newborn
        
        prenatal DNA analysis
        
        Hgb electrophoresis
        
        isoelectric focusing
        
        high performance liquid chromatography
        
        alkaline hemoglobin electrophoresis
    - - prophylactic penicillin
      - avoid dehydration
      - PRBC transfusion
      - pharmacological agents to increase hemoglobin F production
      - hematopoietic stem cell transplant