Hereditary WBC Disorders

Hereditary WBC Disorders

1. May-Hegglin Anomaly

   • Associated Conditions
     • Abnormal bleeding may be present in some individuals.
     • thrombocytopenia
     • giant platelets
   • WBC's affected
     • neutrophils
   • appearance
     • Döhle-like bodies
       • Rod shaped inclusions at the edge of the cytoplasm. They are like Döhle bodies but larger.
   • prevalence
     • unknown

2. Alder-Reilly Anomaly

   • Associated Conditions
     • dwarfism
     • gargoylism
   • WBC's affected
     • All cell lines
       • hallmark feature
   • Appearance
     • coarse dark granulation
   • prevalence
     • rare

3. Pelger-Huet Anomaly

   • prevalence
     • common
   • associated conditions
     • leukemias
     • myleoproliferative disorders
     • severe infections
   • WBC's affected
     • segmented neutrophils
   • appearance
     • hyposegmentation of nucleus
       • heterozygotes
         • peanut-shaped
         • dumbbell-shaped
         • pince-nez-shaped
       • homozygotes
         • spherical non-lobed

4. Chediak-Higashi Syndrome

   • prevalence
     • rare
   • associated conditions
     • neutropenia
     • albinism
     • photophobia
     • recurrent staphylococcus infections
   • WBC's affected
     • neutrophils (mostly)
     • lymphocytes
     • monocytes
   • appearance
     • giant purple-gray granules

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