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306            PART 4  ■  Erythrocyte Disorders




                    P                                                               m

                         m            -                  -                                                                      BOX  16.5

                                                                      m                      .

                                  .           B  x      .           ,                    ,

                                                         m                m                           -                       Differential Testing in Hemolytic Anemias

               m                          m                                                                                   A                                              ,

                            .


                                                                                                                              B       m           x  m

                                                                                                                              C
                  NOTE: This is a good time to complete Review Questions

                  related to the preceding content.                                                                           G  PD
                                                                                                                              M  j                                 (                         )

                                                                                                                              L


               COMPLEMEN  -MEDIA  ED DISEASE



               T                     m


                     x   m                          m                    (PNH),                                               m     m    .  W                                  m    m
                  m                m               m    (  HUS)                          m  -                                       ,                        m                                   m       ,


                             m               m    (HUS),                                                                                m      ,                        m         . C                          -
               (CAD),                          CD                     . C                                                           m             PNH    m


                    m     m     -m                                                                  .                            m     m     .
                                                            x m                .                                                PNH    x                                 m                 m   ,

                                                                                                                           m                   ,              m       . I                        m

               Paroxysmal Nocturnal Hemoglobinuria                                                                                                               z                 m         (       x   m   )

                                                                                                                                   -                  (               )                                   (    m  -
               Etiology                                                                                                                   ). S  m                       PNH                m


               PNH             q             m                       m                         m                              m                                                               m

                       x                     m                     m                                    -                                  m                .

                                            GPI,                    m

               m                        z                                                                                  Epidem iology

               m  m                     .                                                                                            -                                                                  m

                    PNH             ,     q        ,                                                                           m   . A       x m           %         %                                     -

                       m                         x                            m          m     -                           m                 m                        k  m   .

                               m                             q            m       m                                             T   m                                                                      (         ,

               X-       m     m           , PIGA. T                           PIGA         ,                                                  ). M                                                             .

                    m         x                     m                        ,       q                                     S                          -    m    m                         .

                                      GPI                               . T

                                                      ,     m     m                                    ,                   Pathophysiology

               CD              CD    .  T                                 m  m                                             M                                           m     PIGA                                -

                                                                                                                                                                                                    m







                                                                                                                                                         Clinical Manifestations of
                                             Typical Pro  le of Quantitative                                                    TABLE        16.5        Complement-Mediated Diseases

                    TABLE        16.4        LABORATORY Findings in

                                             Hemolytic Anemias                                                                 PNH                                              Hemolysis, Thrombosis



                   Test                                                         Result                                         HUS (typical)                                    Hemolysis, thrombocytopenia,

                                                                                                                                                                                renal failure

                   Hemoglobin, hematocrit, RBC                                  Decreased                                      HUS (atypical)                                   Hemolysis (extravascu-
                   count
                                                                                                                                                                                lar), thrombocytopenia,
                   Serum haptoglobin                                            Decreased                                                                                         thrombosis, renal failure


                   Red blood cell survival ( Cr)                                Decreased                                      CAD                                              Hemolysis (extravascular and
                                                         51

                   Lactic dehydrogenase (LDH)                                   Increased                                                                                       intravascular)

                     isoenzymes (LD and LD)                                                                                    TTP                                              Hemolysis, thrombocytopenia,
                                            1
                                                         2
                   Bilirubin (total)                                            Usually increased                                                                               thrombosis


                   Antiglobulin test                                            Positive or negative                           Congenital CD59 de  ciency                       Hemolysis
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