Wiskott-Aldrich syndrome (WAS) or eczema-thrombocytopenia-immunodeficiency syndrome

A rare X-linked recessive disease.

  • symptoms appear during first year of life.
  • abnormalities of humoral immunity begin within the first 6 months of life
  • leading to the inability to produce Antibodies to polysaccharide antigens causing susceptibility to encapsulated microbes
  • decreased serum IgM and increased serum IgA and IgE levels present
  • T-cell dysfunction is initially mild, yet progressively deteriorates.

Manifestation:

  • eczema
  • thrombocytopenia (bruising )
  • immune deficiency (recurrent infections)
  • bloody diarrhea (secondary to the thrombocytopenia).
  • predisposition to the development of certain cancers (Hodgkin lymphoma and leukemia)

Treatment:

  • eczema symptoms management
  • infection control
  • management of bleeding episodes with transfusions as necessary

Martin, Glenn and Porth, Carol, Mattson. 2009. Pathophysiology Concepts of Altered Health States. 8th ed. Lippincott Williams and Wilkins. Philadelphia

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