Page 51 - Color Atlas Of Pathophysiology (S Silbernagl Et Al, Thieme 2000)
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A.  Causes of Corpuscular Hemolytic Anemia
           Gene defects              Glucose             Enzyme defects
         4                                                 2
                                          Carrier            ATP
         Sickle-cell                 Hexokinase             deficiency
         anemia            Erythrocyte
            Thalassemia                          Pyruvate    Cell
                            Hb    Glucose–6–P    kinase     swelling
                                            Glycolysis
                            Ankyrin
                                    Gluc–6–PDH        ATP   Hemolysis
                               –   –
                             Cl /HCO 3                 +               Anemias
                                                      K
        Deficiency or                Pentose–P cycle         Na +
        functional disorder  Spectrin
        of spectrin
                                    2 GSH    GSSG
        1                    Decay-                         Defect     Hemolytic
                             accelerating
          Osmotic            factor (DAF)
         resistance                 R 1 –SS–R 2  R 1 –SH,R 2 –SH  3
                       DAF defect
                    5                 Oxidative stress                 Plate 3.7
          Hereditary                         +
         spherocytosis  Complement
                       activation
                                     Horse beans, primaquine,
                                       sulfonamides, etc.   Favism
                       Paroxysmal
                      nocturnal hemo-
                      globinuria (PNH)
        B.  Causes and Consequences of Acut Intravascular Hemolysis
           Corpuscular causes        Extracorpuscular causes
               (e.g. PNH)     immunological  toxic    mechanical
                             (e.g. transfusion  (e.g. snake poison)  (e.g. artificial
                               reaction)              heart valve)


                          Acute intravascular hemolysis
                                                          CO
                Erythrocyte fragments  Free Hb
                                                          Hb deficiency
               Thrombosis,             Haptoglobin
                embolism               overload
                                                         Fe deficiency
                                          Renal filtration
                                            of Hb
                                                      Hemoglobinuria
                Ischemia
                                       Acute renale failure
                                                                      41

       Silbernagl/Lang, Color Atlas of Pathophysiology © 2000 Thieme
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