Page 345 - Clinical Hematology_ Theory _ Procedures ( PDFDrive )
P. 345

CHAPTER 17  ■  Hemoglobinopathies and Thalassemias                                   329





                                                                                                                               ■   C  mm                                    H    SC
                     NOTE: This is a good time to complete end of chapter                                                           β-            m    (S β-            m   ).

                     Review Questions.
                                                                                                                               ■   I           U          S       ,             m           %              A
                                                                                                                                    Am                                                    .


                                                                                                                               ■   A
                   CHAP  ER HIGHLIGH  S                                                                                             m                                                      k      . A           m

                                                                                                                                                      ,     k          k

                   Hemoglobin Defects                                                                                                                                                                    .


                                                                                                                               ■   D                                                                k                     -
                   ■   A       m         m              m                             m  m        ,                                                      m                                    . T

                               m                  .                                                                                               SCD                        q                H   S

                   ■   P                     m                  m              m  z                   -                                              .

                            z                           .

                   ■   T       k             m                          m             C, E,     D,

                                          SC, SE,     SD            .                                                          Thalassemia

                   ■   I                          m             S,                       m                                         T               m              m            m             m         mm

                                      m            m              m                                                            ■                                     m     . T                 m

                          x                                                                                                                           m

                                      m                         .           m                     -                                          .

                                                                                          k                                        T                                            m          m

                             x                    m             S       m     z                 m                              ■                                           m                                               -

                                                                                m                     m   -                            ,      f                           mRNA,

                                           m                               m                                                                  x            ,                                                ,

                              x                .                                                                                                                                  .

                   ■   A                                            m              S  m                                            C                        m                m                  : m  j

                                                                                      m                                        ■    m      . T

                                                                                                                                       m                      .

                                .                                                                                                  β T          m                        m         mm              -

                   ■   I            m        m             C,            m            m                                        ■               . M                                     β-

                         m                                      x                                                                   k                                                              β-          ,

                                                              m                         .                                                                                x                m

                   ■   H  m                          m                     m          m            -                                γ-                     ,                                         ,

                              . A       x m             %              m                              m-                                                      .

                                                                                                             z                     α               m                                                          z

                           m                     .                                                                             ■                                                α            .  α                m

                   ■   T       mm             m                        m                                                                                                                                       -

                                                                                                                                                                      m                   m

                           m            .                                                                                                   .

                   ■   I             m                         ,

                                                                                m      . A

                                                                m  z                                                           Other Hemoglobinopathies

                                                    m                                                   -

                        z         ,     m                                               m                   -                  ■   H   C                                             m

                              . A                                               z                      m                            H   S (    k          )            .

                            m     m              .                                                                             ■   H    E                                                            q
                                                                                                                                    S              A    . I      m                T           ,           q



                   Sickle Cell Disease (Sickle Cell Anemia)                                                                                  H   E              m         %.
                                                                                                                               ■   M       m              m

                   ■   SCD                    z                                      H   S,       m   ,                             m       m                                                            .
                                                                      m                                                        ■   U                m                        m

                               k                                                          m                                          m                                                         k                  -

                                               .                                                                                                           m


                   ■   SCD (H   SS),       m         mm         m         m            -                                                                    m             m           .
                                 ,           x                                                  k                              ■   H                                      H   F

                            m                    . O           k                                      m                                            z                         m                                      m  -

                                                                   k            .                                                                                             .
   340   341   342   343   344   345   346   347   348   349   350