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Wiskott-Aldrich Syndrome

Mustafa Alavi, MD and Anthony M. Cheng, MD Reviewed 06/2019
 


BASICS

DESCRIPTION

  • Wiskott-Aldrich syndrome (WAS) is a rare X-linked recessive condition characterized by eczema, recurrent infections, autoimmunity, and malignancies.

  • It is seen almost exclusively in m...

DIAGNOSIS

  • Typical presentation is a boy with early-onset thrombocytopenia, small platelet size, atopic dermatitis, and frequent infections.

  • Triad of mucosal bleeding/petechiae, eczema, and recurrent ot...

TREATMENT

GENERAL MEASURES

  • Treatment approaches are variable for this pleomorphic, rare disease (1).

  • HSCT, from bone marrow or cord blood, achieved a 75% 7-year disease-free survival in a retrospective ...

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
Conley ME, Saragoussi D, Notarangelo L, et al. An international study examining therapeutic options used in treatment of Wiskott-Aldrich syndrome. Clin Immunol.  2003;109(3):272–277. ...

ADDITIONAL READING

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

SEE ALSO

Immune Thrombocytopenia (ITP); Immunodeficiency Diseases 

CODES

ICD10

D82.0 Wiskott-Aldrich syndrome 

ICD9

279.12 Wiskott-aldrich syndrome 

SNOMED

36070007 Wiskott-Aldrich syndrome (disorder) 

CLINICAL PEARLS

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

  • Without HSCT, death usually occurs between th...

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