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Wiskott-Aldrich Syndrome

Jason Cross, PharmD, Sudeep Kaur Aulakh, MD and Nathan Zephier Lamore, Doctor of Pharmacy Reviewed 06/2021
 


BASICS

DESCRIPTION

  • Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive condition characterized by eczema, immune deficiency, and microthrombocytopenia  (1).

  • Clinically it presents as severe ecze...

DIAGNOSIS

  • Should be suspected in a male with microthrombocytopenia and excessive bruising/bleeding

  • Definitive diagnosis: male patient with microthrombocytopenia (platelet <70,000/mm3) and one of the...

TREATMENT

GENERAL MEASURES

Ensure immunizations are up to date (4). 

MEDICATION

Used to provide supportive care 
  • Enhance immune function:

    • Intravenous immunoglobulin (IVIG) infusions for significant antibo...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Avoid contact sports and prevent head injuries.

  • Avoid crowds.

  • No live-virus vaccinations

Patient Monitoring

As needed for therapy; monitor for infections, disease pro...

REFERENCES

1
Hacein-Bey Abina  S, Gaspar  HB, Blondeau  J, et al. Outcomes following gene therapy in patients with severe Wiskott-Aldrich syndrome. JAMA. ...

ADDITIONAL READING

  • Aldrich  RA, Steinberg  AG, Campbell  DC. Pedigree demonstrating a sex-linked recessive condition characterized by draining ears, eczematoid dermatitis an...

SEE ALSO

Immune Thrombocytopenia (ITP); Immunodeficiency Diseases 

CODES

ICD10

D82.0 Wiskott-Aldrich syndrome 

SNOMED

36070007 Wiskott-Aldrich syndrome (disorder) 

CLINICAL PEARLS

  • Rare, X-linked; consider WAS if prolonged bleeding following circumcision and in children with recurrent infections, eczema, and low platelet counts.

  • HSCT may be curative

  • Mnemonic (TIME)...

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