Dermatologic Manifestations of Wiskott-Aldrich Syndrome Treatment & Management

Updated: Aug 13, 2019
  • Author: Akimichi Morita, MD, PhD; Chief Editor: Dirk M Elston, MD  more...
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Treatment

Medical Care

Transfusions of platelets and plasma decrease the risk of fatal hemorrhage in Wiskott-Aldrich syndrome patients. Agents such as eltrombopag that mimic thrombopoietin are promising. [16, 17]

Appropriate antibiotics should be used to treat bacterial infections. The antibiotics should be chosen based on the bacteria obtained and detected from the infected site.

Intravenous infusions of immune globulin decrease the risks of infection.

Infusion of transfer factor results in decreased frequency of infection and improvement of eczema. [18]

Bone marrow transplantation with HLA-identical marrow should be considered if patients have recurrent problems. Full engraftment results in normal platelet numbers and functions, immunologic status, and clearance of the eczema. [19]

Topical steroids may improve the eczema.

Gene therapy is promising for Wiskott-Aldrich syndrome. [20, 21, 22, 23, 24, 25, 26]

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Surgical Care

Splenectomy should be considered for Wiskott-Aldrich syndrome patients with thrombocytopenia who have no HLA-matched bone marrow transplantation donor. [19, 27, 28]

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Consultations

Possible consultations include a pediatrician and/or oncologist.

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Diet

In patients with food allergies, diet control must be considered.

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Long-Term Monitoring

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.

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