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WBC Disorders-3

  • First described by Miller, Oski and Harris in 1971.
  • It is a syndrome caused by loss of chemotactic function of              neutrophils
  • Marrow contains normal number of matured neutrophils, but patients have severe neutropenia because the cells are unable to migrate  from the  marrow to peripheral blood.
  • Infections – as neutrophils fails to migrate at the site of                   inflammation

 

  • Oculocutaneous albinism
  • Photophobia
  • Nystagmus
  • Recurrent infections (usually   involving upper respiratory     tract  & skin)
  • Neurological problems
  • GI disturbances
  • Generalized lymphadenopathy
  • Hepatosplenomegaly
  • Oral ulcerations
  • Glossitis
  • Severe gingivitis
  • Sometimes associated with        lymphoma
  • Often fatal in early life as a       result of a lymphoma-like            terminal  phase, hemorrhage,    or infection

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