Section 16. Immune Disorders

Chapter 184. Immunodeficiency Disorders

Topics: Introduction | X-Linked Agammaglobulinemia | Selective Antibody Deficiency | Common Variable Immunodeficiency | Transient Hypogammaglobulinemia of Infancy | Chronic Mucocutaneous Candidiasis | DiGeorge Anomaly | Ataxia-Telangiectasia | Severe Combined Immunodeficiency Disease | Wiskott-Aldrich Syndrome | Hyperimmunoglobulinemia E Syndrome | Chronic Granulomatous Disease | Immunodeficiency due to Spleen Disorders

Wiskott-Aldrich Syndrome

Wiskott-Aldrich syndrome is a hereditary immunodeficiency disorder characterized by abnormal antibodies and T lymphocytes, a low platelet count, and eczema.

Wiskott-Aldrich syndrome affects only boys. The number of platelets is low. Consequently, bleeding problems, usually bloody diarrhea, may be the first symptom. Eczema also develops at an early age. Susceptibility to infections, particularly of the respiratory tract, is increased because the antibody levels are low and T lymphocytes malfunction. The risk of developing cancers such as lymphoma and leukemia is increased.

Stem cell transplantation is necessary to preserve life. Without it, most boys with this disorder die by age 15. Surgical removal of the spleen may relieve the bleeding problems. Antibiotics are given continuously to prevent infections, and immune globulin may help.