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hematological disorders (treatment approaches (splenectomy, apheresis:…

- - - - diagnostics
      - causes
        
        inherited
        
        abnormal hemoglobin
        
        RBC membrane abnormalities
        
        enzyme deficiences
      - r/t RBC destruction
        
        acute autoimmune hemolytic anemia
        
        sickle cell
        
        thalassemia
        
        acquired
        
        antibody related
        
        transfusion reaction
        
        autoimmune hemolytic enemia
        
        corticosteroid treatment
        
        cold agglutinin disease
        
        not antibody related
        
        paroxysmal nocturnal hemoglobinuria
        liver disease
        uremia
        trauma
        mechanical heart valve
        infection
        bacteria/parasite
        disseminated intravascular coagulation
        toxins
        hypersplenism
      - treat & manage
        
        correct cause
        
        transfuse packed RBC
        
        anemia type specific treatments
    - - causes
        
        iron, B12, folate deficiency, B12 def. in vegan
        
        decreased erythropoietin production
        
        cancer
      - aplastic anemia: bone marrow doesn't produce enough blood cells
        
        causes
        
        genetic vs. acquired
        
        often secondary to chronic diseases or med use
        
        most common: autoimmune/ idiopathic
        
        severe
        
        bone marrow transplant
        
        moderate
        
        steriods, epogen, blood transfusions
      - megaloblastic/ vitamin deficient anemias
        
        treatments
        
        causes
        
        alchohol
        small intenstine disorders
        
        aka: pernicious anemia
        
        schilling test: helps determine cause of B12 deficiency
        
        folate: green leafy veg & liver
      - iron deficiency
        
        signs & symptoms
        
        fatigue
        
        palpitations
        
        dyspnea on exertion
        
        PICA
        
        pallor
        
        tachycardia
        
        brittle/ridged nails
        
        tender/pale/smooth tongue
      - anemias of chronic and renal disease
    - - H&H
      - retuculocyte count
        
        increase indicated altered erythropoiesis
      - RBC indices
        
        MCV/RDW #
        
        iron studies
      - B12
      - Folate
      - bone marrow aspiration
      - S&S
- - - - missing/defective von Willenbrand factor (clotting protein)
        
        VWF should bind factor VII to form platelet plug
    - - excessive bruising
        excessive bleeding
        frequent nosebleeds
        heavy periods
    - - 1: most common
    - - w/ clotting factor tests
    - - DDAVP (synthetic vasopressin) to stimulate VWF release
  - - - treat via blood letting
  - - - recessive genetic trait causes production of hemoglobin S
        
        two SS genes = sickle cell anemia
        
        hemoglobin SC, hemoglobin sB thalassemia
  - - - alpha protein production deficiency
        
        Africa, middle east, India, SE Asia, S, china, occasionally Mediterranean
    - - body doesn't make enough beta protein
        
        common in individuals from mediterranean, arabian, african, southeast asian, southern Chinese
- - - - r/t excessive erythropoietin production r/t reduced O2, cyanosis, non pathologic conditions, neoplasms
    - - none if mild
      - treat underlying cause
      - therapeutic phlebotomy