Dermatologic Manifestations of Wiskott-Aldrich Syndrome Follow-up
- Author: Akimichi Morita, MD, PhD; Chief Editor: Dirk M Elston, MD more...
Further Outpatient Care
Obtaining a blood analysis on at least a monthly basis is necessary in Wiskott-Aldrich syndrome patients. Malignancy evaluations should be conducted, especially in elderly Wiskott-Aldrich syndrome patients.
With advancing age, T-cell function is progressively impaired, and patients are increasingly susceptible to infections caused by herpes and other viruses and Pneumocystis carinii.
Autoimmune phenomena may develop. Arthritis, renal disease, dermatomyositis, and autoimmune hemolytic anemia have been reported.
Lymphoreticular malignancy develops in 18-20% of patients, particularly in those with autoimmune manifestations. Non-Hodgkin lymphoma is the most common malignancy.
Intracranial hemorrhage is a constant threat.
Death usually occurs during infancy. The cause of death in Wiskott-Aldrich syndrome is infection in 55-60% of patients, bleeding in 24-27% of patients, and lymphoreticular malignancy in about 5-10% of patients. Ten percent of Wiskott-Aldrich syndrome patients die from lymphoreticular malignancy, usually as adolescents or young adults.
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