Page 361 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )
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CHAPTER 18  ■  Disorders of Granulocytes and Monocytes                                   345




                   T                                                                                                           N   m      -P   k                                                           ;

                              . D                                   z  m   ,                       ,                                     ’                                                   .

                                ,                                                                             -                     T                                                                   z  m

                                           ,                                m                                                      m                                                      m                       -

                                                                   . T                                   -                               ,             m        . S           m                 m

                                                           m                          m         :           -                            m                    (F   .      .    ).  T

                    m                 (GAG ) (m                                  [MPS])                                                          , P   k’        ,      m                                  G           ’

                                .                                                                                                  ;             ,                   m                        m                  -

                        M                                                                                 m                           . B       m          x m                         m

                   m                                   ,                A      -R                  m   -                         z       m                 µm       z                      m                     -

                      . D                                                       m    m                                                                                     m.

                                G           ’ , N   m      -P   k,        S    -                       -

                      . I   MPS,         m            m       m                                  -                             Miscellaneous Storage Diseases

                   m                                       .
                                                                                                                               Tay-Sachs Disease


                   Gaucher’s Disease                                                                                           Tay-Sachs disease                                                            -
                                                                                                                                                                                                                            .

                   Gaucher’s disease,                                ,                                -                        I                       m         mm         m                              -

                                                      m            m. G           ’                 m                                                   m                                     m        . C

                       q                                              ,                                                                                                 m           -S

                       m                            . I                       m    ,                   m                                                                      . O           m            -S            -

                                                  m         ;                    ,                   m                                                    . S                 m     m                               -

                       m          .                                                                                                   ,                     ,                                              m

                        T                                                         β-                              ,                                                            m.

                             z  m               m                                m                        -                              -S                                                                             .

                             ,                     m     . A                               z  m         -                      T                m                                                      m          m-

                           ,                       m             (m                )                                           m                         A   k      z  (                               E             )

                   (F   .     .  ). G           ’                                                                              J                      . T    m

                           . T                G           ’                    ,                                                          m          mm                     F         -C                  m-

                                                                                          k                   m.               m               Q          ,       O    O       Am         mm

                   T                                                 m        ,           ,                                    P                   ,              C  j                           L             .

                                      m                                            m. T               -                             M                          HEXA                             -S                    .

                                                              k                                                 -              T   HEXA                                               m  k

                   m                                                m        .                                                    z  m                  -   x     m           A,

                                                                                                                                                                                 . T        z  m

                   Niemann-Pick Disease                                                                                                 m                        k                                                 GM



                   Niemann-Pick disease      m          G           ’
                                                                  m                       m            m.












































                                                                                                                               FIGURE  18.10  H                                 m   m

                   FIGURE 18.9  G           ’             . B       m                               m    -                              m-                                           N   m      -P   k             .

                                             m                              m            . (R                  m               (R                  m C        P  . Color Atlas  and    ext  o  Pulmonary
                   M  C             KD. Clinical Laboratory Medicine,            , P                  ,                        Pathology, P                  , PA: L               W      m  & W  k    ,         ,

                   PA: L               W      m  & W  k    ,         ,             m        .)                                            m        .)
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