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Monocyte-macrophage disorders                                                                               Case studies


               ■    Describe the general characteristics of lysosomal storage disorders.                                     nalyze the patient history, clinical signs and symptoms, and labora-

                 Identify and differentiate the defects found in Gaucher’s disease and                                         tory data for the stated nonmalignant disorders of granulocytes and

                   Niemann-Pick disease.                                                                                       monocytes case studies, answer the related critical thinking ques-

                 Compare the etiology and laboratory   ndings in miscellaneous lyso-                                           tions, and conclude the most likely diagnosis.

                   somal storage disorders.
                                                                                                                              NOTE:


                                                                                                                              ■   indicates MLT and MLS core content
                                                                                                                                indicates MLT (optional) and MLS advanced content













               T                               m                                                                            .  I               m      m          m                     m       m

                     m

                            ’                                    x  m           . N    m                                    .  D               m      m          m                     m

                                                                m

                                                      k                        q                       -

                     ,                                     k                                   k          . A              Pathophysiology of Granulopoietic Alterations

                                                                                                                           I       m     ,                                       k

                                               m              .
                                                                                                                           m        . T                                               k

                                                                                                                           m        ,          ,                           .

               GRANULOPOIE  IC AL  ERA  IONS:                                                                                   T   m              ,      m              ,        m


               QUAN  I  A  IVE DISORDERS                                                                                                                                     . T           m
                                                                                                                                 m             m      m     . M      m                              m —

               Q                                         k           m                                   -                 m     m               ,          ,        m                          —             -

                                  (leukocytosis)                      (leukocytopenia)                                                                     ,                                         m

                                  k                                                     (              .  ).                                            .  T                                         m

               I                                     ,                   ,                                                    m      m                                         ,

                                                          ,                     ,                          ,                                                                          (CGP)           m       -

                                 .  A                        m                                m          -                                                     (MGP).

                     . D                                             m                                                          T                                                 (  BGP)             m

                                ,                  ,                ,        m                    ,                        CGP              MGP. C

                                 .                                                                                           q           m. U             m                    ,

                                                                                                                                                  x m           q            z  .


               Leukocytosis                                                                                                     A        m                CGP   z                       m             m     -
                                                                                                                                                                           mm                           k

               L    k                                                                                     -                                                    m  .  T    MGP

                                                       k                                               :                                                         ,

                               ,                  ,                ,  m             ,          m-                              m               ,                                                   . T    MGP

                             . A                                                       k                                             mm                                           ,

               m                                                     m              ,                                                                        .

                                              m         q                            m

                                                     k                                                   -

                                                                                                .                          Neutrophilia

                    N   m     ,                         k                                                                  N                                                                                            .

                                                                                      . P                                  T

                    k                                                                .                                          A

                    N    m                   k                                                            -                ■

                                                                      . T                                                  ■    A            m
                                                                                                                           ■    M                                                  m      ,        ,          m
                .  I               m      m           mm                                                                   ■    P               m                                       ,           ,

                     m        ’                          m      m                                                          ■    S                                                      x         ,            ,

                .  I               m       z                         m       m              -     -                                m

                               m      m                         m                                                          ■    S  m      x    ,         ,             m                    m

                                                                                                                                                            m
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