Wiskott-Aldrich Syndrome Follow-up

  • Author: Donald A Dibbern Jr, MD; Chief Editor: Michael A Kaliner, MD   more...
 
Updated: Sep 28, 2010
 

Further Inpatient Care

  • Patients with severe infections, bleeding, or malignancies may require hospitalization for intravenous antibiotics, for monitoring and/or transfusions, or for oncologic care, respectively.
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Further Outpatient Care

  • Patients must receive close pediatric or medical follow-up, specialized allergy and immunology care, and, often, hematology or oncology care.
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Inpatient & Outpatient Medications

  • Patients with WAS often need antibiotics for recurrent infections, either in an inpatient or outpatient setting. Patients who had a splenectomy usually require daily prophylactic antibiotics.[25, 45]
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Transfer

  • Patients may need evaluation, and sometimes transfer, to a referral center with expertise in pediatric immunodeficiencies.
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Deterrence/Prevention

  • Genetic testing and prenatal diagnosis are options that may contribute to decreased occurrence of this condition.
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Complications

  • Complications from infection, bleeding, and malignancy characterize WAS.
  • Autoimmune and rheumatologic conditions may also occur.[46] One study found these conditions in 40% of patients, and often multiple conditions coexisted in the same patient. Patients with autoimmune disease were significantly more likely to develop malignancy.[2] Another review of 55 patients with WAS from a single hospital in France, over 20 years, found autoimmune or inflammatory conditions in 72%, most commonly autoimmune hemolytic anemia (see Donath-Landsteiner Hemolytic Anemia and Cold Agglutinin Disease), among multiple other conditions.[47]
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Prognosis

  • Long-term prognosis had been poor; prior to use of stem cell transplantation, few patients survived beyond their teens and most succumbed to complications of bleeding, infection, or malignancy.[48] Median survival in a cohort of patients born after 1964 was 6.5 years, although survival rates have continued to increase over time.[22]
  • With aggressive care, prognosis may substantially improve. One study projects median survival of 25 years for patients who undergo splenectomy, and even longer for patients who undergo successful bone marrow transplant.[25] Success rates of all categories of stem cell transplantation (HLA-identical, matched/related, matched/unrelated, umbilical cord blood) have also continued to climb over time.[27, 26]
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Patient Education

  • Educate patients about the function of their platelets and their immune system and about signs and symptoms that require prompt medical attention, including those seen with infections, bleeding, and malignancy.
  • Advise patients to appropriately restrict activities, depending on the severity of thrombocytopenia (eg, protective headgear may be indicated).
  • Teach patients excellent general skin care and moisturization to manage eczema.
  • Refer women known to be carriers for WAS for genetic counseling, and advise them that prenatal diagnosis is available.
  • For excellent patient education resources, visit eMedicine's Skin, Hair, and Nails Center. Also, see eMedicine's patient education article Eczema.
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Contributor Information and Disclosures
Author

Donald A Dibbern Jr, MD  Consulting Staff (Allergist), Providence St Vincent Medical Center

Donald A Dibbern Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, and Oregon Medical Association

Disclosure: Nothing to disclose.

Coauthor(s)

John M Routes, MD  Professor of Pediatrics, Medicine, Microbiology and Molecular Genetics, Chief, Section of Allergy and Clinical Immunology, Department of Pediatrics, Medical College of Wisconsin

John M Routes, MD, is a member of the following medical societies: Alpha Omega Alpha, American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Microbiology, American Society for Virology, Clinical Immunology Society, and Federation of American Societies for Experimental Biology

Disclosure: Nothing to disclose.

Specialty Editor Board

Charles H Kirkpatrick, MD  Professor of Medicine and Immunology, University of Colorado School of Medicine; Director of Adult Immune Deficiency Program, Department of Medicine, University of Colorado Health Sciences Center; Consulting Staff, Department of Medicine, National Jewish Medical and Research Center

Charles H Kirkpatrick, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Physicians, American Federation for Clinical Research, American Society for Clinical Investigation, and Clinical Immunology Society

Disclosure: CSL Behring Consulting fee Consulting

Francisco Talavera, PharmD, PhD  Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Michael R Simon, MD, MA  Clinical Professor Emeritus, Departments of Internal Medicine and Pediatrics, Wayne State University School of Medicine; Adjunct Staff, Division of Allergy and Immunology, Department of Internal Medicine, William Beaumont Hospital

Michael R Simon, MD, MA is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American College of Allergy, Asthma and Immunology, American College of Physicians, American Federation for Medical Research, Michigan Allergy and Asthma Society, Michigan State Medical Society, Royal College of Physicians and Surgeons of Canada, and Society for Experimental Biology and Medicine

Disclosure: Secretory IgA, Inc. Ownership interest Management position

Timothy D Rice, MD  Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, St Louis University School of Medicine

Timothy D Rice, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Physicians

Disclosure: Nothing to disclose.

Chief Editor

Michael A Kaliner, MD  Clinical Professor of Medicine, George Washington University School of Medicine; Chief, Section of Allergy and Immunology, Washington Hospital Center; Medical Director, Institute for Asthma and Allergy

Michael A Kaliner, MD is a member of the following medical societies: American Academy of Allergy Asthma and Immunology, American Association of Immunologists, American College of Allergy, Asthma and Immunology, American Society for Clinical Investigation, American Thoracic Society, and Association of American Physicians

Disclosure: Alcon Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering/Merck Consulting fee Consulting; Teva Consulting fee Consulting; Meda Honoraria Speaking and teaching; Ista Consulting

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Eczematous lesions in Wiskott-Aldrich syndrome. The lesion is essentially indistinguishable from that of atopic dermatitis except for the presence of purpura and petechiae.
 
 
 
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