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328            PART 4  ■  Erythrocyte Disorders





               β             m                                                . H   E
                   m                                                    m                      β

                                m            .

                    C                                             . I                       m

                   m     m       (A/E)     m              m    (E/E)                         -

               m    (E/β).

                    I               m  z                  ,                              m      m

               m                      m                                                                 .

                                                                                         m      .  I

                                                                 H   E       m        m

                                      m   ,                         (                           ,

                H)                                                             z               m       -

                               . I         U         S       , m                 H   E                                     FIGURE 17.13  H     z           . C                           . (R                  m

                              m                                                                          -                 A             SC. Anderson’s Atlas o  Hematology, P                  , PA:

                                                   m               -   k                      (  .  .,                     W         K         H        /L               W      m  & W  k    , C
                                                                                                                                ,             m        .)
               S              A       ).





               Hemoglobin H Disease                                                                                           m     m                                   .  P                        q


               H   H                   m                                     m                m   .                         m       m              m                                       x

               T                m         q                         m                                                                      m     m              x        m      m

                              α-                    (      α-            m   ).                                              %         %.  T        m     m                          m       m       -

                    T        m                               m                                                                   m                                 x                    . I

                                 S              A    ,       M                               ,                                                m                         x             m

                                M         E    . B                                      x      mm -                                               m               x           . Ex                         H   M

                            m S              A                                            ,                                                                                     -  -                            -

                                     H   H                       U         S                                                          (G  PD)                 , m

                                 .                                                                                         m       m              m           q     k                                          m

                    H    H  m                                                  k            H                                                                         . I       -

                  m                                   . T        m                             m                           m       m                               ,    x                              m

                       m                                                  H   H            . A                                                               .

                             x                                     m                . T

                      m                                                     m   ,  x                                       Unstable Hemoglobins

               H   H            ,                                               RDW                                        U                 m                          m

                                       MCV                        . A                                                       m                                                          k

                m        x         m                       ,                   ,      k              ,                                      m

                                   m                                                            . T                                     m             m           . M                      m

                                                                                %            %       m                                                  m        m                      .

                                        m             . S     m                            m                                    I               m                        m        m

                            .
                                                                                                                                                                                      H     z           . A

                                                                                                                                   ,                                     m           ,  m  m               m
               Methemoglobinemia                                                                                                    ,            m                  . H  m                                -


                                                                                                                                                                        m                m
               M       m              m

               m       m                                                                 .  C                                                       x    z                                     . H     z      -

               m       m              m                       q             x                     ,                             (F   .     .    )                               α-     β-                   m    -

               H   M             ,        NADH-m       m                             (                                          .         m              m              ,             H   B            H   H,

                                         )              .                                                                                             m    .

                    H   M                              m . I                      m                 -

                                            m                                           m                                  HEREDI  ARY PERSIS  ENCE OF FE  AL

                                                              z                                  m.                        HEMOGLOBIN

               NADH-                               z  m                                     m

                  ,                                                                              k                         R                  H   F (            m            )                                   -

                                 .                                                                                         m   . T                   x                    %         %                 m       -

                    P                                       m       m              m            -                              . T            m

                                    m       m                                            %                                    H   F.
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