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CHAPTER 13  ■  Bone Marrow Failure Syndromes                                253





                   ■           I—                                                      ,                                       Constitutional Bone Marrow  Failure
                                 m                      ,                                                   .                  Syndromes

                        M                                                     ×     /L              .


                   ■           II—m            m                                                                               ■   F           ’          m
                                                           m                                                                             m                        m   .

                                                      m                                                    .                        F           ’        m                 . A                                m

                        M                                                   ×     /L            ×     /L.                                                              , F           ’        m               m


                   ■            III—                              m      k                  .                                           q          .

                   ■   D                                                                m                                      ■   T
                                m      z                ,                                                      ,                    F           ’        m                 m                                           -

                        D       -  k             ,     m                .                                                           m     m           k                        x                                 ,
                                                                                                                                     x  m     , m             C (MMC)                x             (DEB).

                        A                                                                          ,   m      -                     T                                     F           ’        m        m m
                   k                      m                         q                                                                                      m    m           k                 m   ,              -


                                      m                             m   . O                                                                                         .
                   m                              m                                    m   , CAM                               ■   C                       m                                                         m


                            m       k                                                         . A
                                   m                                ,                                 -                                               ,                      m    k              ,                 HLA


                   k                                                          .

                                                                                                                                           m     .


                                                                                                                               ■   F  m                          m                        F           ’        m
                     NOTE: This is a good time to complete the end of chapter                                                                                                                     m         .

                     Review Questions
                                                                                                                               ■   D   k            C                                                 m             -
                                                                                                                                                                    m   .




                   CHAP  ER HIGHLIGH  S                                                                                        Bone Marrow  Failures Involving a Single-


                                                                                                                               Cell Lineage
                   Bone Marrow  Failure Syndromes

                                                                                                                               Red Blood Cells
                   ■   B       m                              m                          z

                                 q                                  m                                                          ■   P                                                      m                            -

                                              ,                ,       /                     m                                                                       . Imm                                         -

                                                 .                                                                                                                                                 m
                                                                                                                                              .
                   ■   B       m                                                                        q        .                 D   m      -B     k             m

                   ■   C                                                    m                 m                                ■

                                                         ,                       .                                                                                             z                                   m  -
                                                                                                                                                 ,          m                   ,                 ,                    ,


                   Aplastic Anemia                                                                                                                                           k
                                                                                                                                    m                              m   .

                   ■   A                 m                                                , k                                  ■   T   m          mm

                                                                    ,                             z                                                    q                                                                 -

                                                                                            . T                   -                         (  EC).

                        m                                                                                                      ■   F                                                                        m    (CDA)

                                                ,      ,               ,               m    B .                                                                    .

                   ■   T                                                          m                                            ■   Im                                                                             m     -

                                                                    mm                ,                       -                                       m         RNA     m           .

                                                                m                           mm       m      -                  ■         m                                                m                  m

                           m (      m              )                           m                          .                                                  q             m                          . M

                   ■   C            mm                         m                                                                     ER   m                k                m                   .

                                                         .                                                                     ■   I       m     ,       m                             DNA    q

                   ■   I    m                                q                              m   ,

                        m                                  m  mm                     m            ,                                                         x    m                            m     m   .

                                -                                        .                                                     ■         m                                                     ,              m

                   ■   A                 m                       z                           m                                                                                                       m                  -

                                                                                                                                    m                                  ,          ,                   m.

                             ,                        ,                   .                                                    ■   M                                                                    m

                   ■   I                            (                   ,      k          ,              m    -                        m                                                           m

                               )                    ,                                                                .                                   .
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