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A5 hemolytic (membrane (paroxysmal nocturnal hemoglobinuria (treatment…

- - - - complement: eculizumab
      - thrombosis: warfarin
      - marrow: immunosuppression
    - - flow cytometry: GPI achor
      - chronic intravascular hemolysis, venous thrombosis, bone marrow failure
    - - GPI anchor, complement, chronic intravas hemolysis (acquired mutation in x chromosome)
  - - - blood film: ovalocytes, stomatocytes
  - - - blood film: elliptocytes
  - - - splenctomy (post-splenectomy sepsis)
    - - flow cytometry (ema stain)
      - (- coombs) blood film: spherocytes
      - cbc: h MCHC
    - - vertical interactions (autosomal dominant)
- - - - IgM autoantibody on RBC
        
        intravas hemolysis
        
        diagnosis
        
        cold: red cell agglutination
        
        treatment
        
        x splenctomy (post-splenectomy sepsis)
        
        monoclonal antibody: cd20: rituximab
        
        warm
    - - IgG autoantibody on RBC
        
        extravas hemolysis
        
        treatment
        
        monoclonal antibody: cd20: rituximab
        
        v splenctomy (post-splenectomy sepsis)
        
        diagnosis
        
        blood: spherocytes (+ coombs)
        
        cbc: h MCHC
    - - evans syndrome
- - - - destruction > production + compensation = shortened lifespan
        
        hemoglobinuria, hemosiderinuria: iron-deficiency anemia
        
        red cell fragmentation sydromes
        
        cold AIHA
        
        G6PD
        
        PNH
- - - - hypercellular
    - - reticulocytosis/polychromasia
  - - - moerate splenomegaly
        
        splenectomy (overwhelming post-splenectomy infection OPSI)
    - - fluctuating jaundice pigment stones
        
        cholecystectomy
- - - - oxidant stress, oxidized hb, intravas hemolysis (x-recessive)
    - - blood film: heinz, bite blister
      - biochemistry: n G6PD (reticulocyte h G6PD)
    - - x oxidant stress
      - maintain urine output (Hb: nephrotoxic)
- - - - TTP thrombotic thrombocytopenic purpura
        
        aetiology
        
        ultra-large vWF, platelet aggregation, thrombosis
        
        diagnosis
        
        micrangiopathic hemolytic anemia (hemolytic anemia + schistocytes)
        
        thrombocytopenia
        
        renal impairent
        
        fever
        
        fluctuating neurlogical signs
      - HUS hemolytic uremic syndrome
        
        aetiology
        
        toxins of O157 O104, shigella: thrombogenic
        
        diagnosis
        
        TTP
      - DIC disseminated intravascular coagulation
        
        aetiology
        
        microthrombi, thrombosis: infarct, bleeding
        
        diagnosis
        
        inc clotting time: APTT, PT
        
        thrombocytopenia
        
        dec fibrinogen
        
        inc fibrin degradation products e.g. D-dimer