eMedicine Specialties > Allergy and Immunology > Immunodeficiencies

Wiskott-Aldrich Syndrome: Follow-up

Author: Donald A Dibbern Jr, MD, Consulting Staff (Allergist), Providence St Vincent Medical Center
Coauthor(s): John M Routes, MD, Professor of Pediatrics, Medical College of Wisconsin; Chief, Section of Allergy and Clinical Immunology, Department of Pediatrics, Children's Hospital of Wisconsin
Contributor Information and Disclosures

Updated: Jun 5, 2009

Follow-up

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.

Further Outpatient Care

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

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

Transfer

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

Deterrence/Prevention

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

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

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

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.

Miscellaneous

Medicolegal Pitfalls

  • Failure to measure quantitative levels of other immunoglobulin classes (ie, IgA, IgG, IgE) for accurate diagnosis and to help rule out other serious comorbid humoral immunodeficiency diseases (eg, common variable immunodeficiency) when discovering low or absent IgM levels
  • Failure to fully evaluate cellular and humoral immunodeficiencies, platelet counts, and blood smears
  • Failure to promptly and aggressively treat infections
  • Failure to offer daily prophylactic antibiotics to patients after splenectomy (Data suggest higher death rates in these patients.)25,45
  • Failure to inform patients and families about bleeding risks and appropriate activity restrictions that will help patients avoid this severe complication
  • Failure to monitor for malignancy (Early detection may help treatment and survival.)
  • Failure to consider appropriate bone marrow transplant options
  • Failure to refer women known to be carriers for WAS for genetic counseling and failure to advise them that prenatal diagnosis is available

Special Concerns

  • Detect carrier status by using a variety of genetic methods, including X-chromosome inactivation analysis49 and polymerase chain reaction and linkage analysis.4
  • Pregnant women who are carriers for WAS can have prenatal diagnosis performed via amniocentesis or chorionic villous sampling.33
  • Patients who had a splenectomy usually require daily prophylactic antibiotics because they carry a substantial risk of sepsis.25,45
  • Use of live virus vaccines poses a risk of infection in patients with immune deficiency such as WAS.
 


More on Wiskott-Aldrich Syndrome

Overview: Wiskott-Aldrich Syndrome
Differential Diagnoses & Workup: Wiskott-Aldrich Syndrome
Treatment & Medication: Wiskott-Aldrich Syndrome
Follow-up: Wiskott-Aldrich Syndrome
Multimedia: Wiskott-Aldrich Syndrome
References
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References

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Further Reading

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Keywords

hypoimmunoglobulinemia M, hypogammaglobulinemia M, dysgammaglobulinemia M, dysimmunoglobulinemia M, dysgammaglobulinemia type V, gamma-M deficiency, selective IgM deficiency, selective immunoglobulin M deficiency, WAS, thrombocytopenia, eczema, autoimmune disease, hematologic malignancy

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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, Medical College of Wisconsin; Chief, Section of Allergy and Clinical Immunology, Department of Pediatrics, Children's Hospital 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.

Medical Editor

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: Lev Pharmaceuticals Consulting fee Consulting

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

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 Board membership

CME Editor

Timothy D Rice, MD, Associate Professor, Departments of Internal Medicine and Pediatrics and Adolescent Medicine, Saint 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: Abbott Consulting fee Consulting; Alcon Consulting fee Consulting; Glaxo Consulting fee Consulting; Greer Consulting fee Consulting; Sanofi Consulting fee Consulting; Schering Consulting fee Consulting; Teva  Consulting; Meda Honoraria Speaking and teaching

 
 
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