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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Wiskott Aldrich syndrome


Other Names for this Disease
  • Aldrich syndrome
  • Eczema thrombocytopenia immunodeficiency syndrome
  • Eczema-thrombocytopenia-immunodeficiency syndrome
  • IMD 2
  • Immunodeficiency 2
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Inheritance

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How is Wiskott Aldrich syndrome inherited?

Wiskott Aldrich syndrome (WAS) is inherited in an X-linked recessive manner. A condition is X-linked if the responsible gene is located on the X chromosome. The X chromosome is one of the two sex chromosomes (the other sex chromosome is the Y chromosome). Females have two X chromosomes in each cell and males have an X chromosome and a Y chromosome in each cell.

Although females have two X chromosomes, one of the X chromosomes in each cell is "turned off" and all of the genes on that chromosome are inactivated. Females who have a mutation in a gene on one of their X chromosomes are called carriers of the related condition. Carrier females usually do not have symptoms of the condition because usually the X chromosome with the mutated gene is turned off. Therefore, they have another X chromosome with a working copy of the gene. Sometimes, the X chromosome with the working copy of the gene is turned off, which may cause symptoms of the condition. However, females with symptoms are usually much more mildly affected than males. A male has only one X chromosome, so if he inherits a mutation on the X chromosome, he will have signs and symptoms (be affected).

Males with an X-linked recessive condition always pass the mutated gene to all of their daughters, who will be carriers. A male cannot pass an X-linked gene to his sons because males always pass their Y chromosome to male offspring.[1]

Female carriers of an X-linked recessive condition have a 25% chance with each pregnancy to have a carrier daughter like themselves, a 25% chance to have a non-carrier daughter, a 25% chance to have an affected son, and a 25% chance to have an unaffected son.[1] This also means that each daughter of a carrier mother has a 50% chance of being a carrier, and each son has a 50% chance of having the condition.
Last updated: 4/10/2015

References
  1. WAS Related Disorders. NORD. February 15, 2008; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/76/viewAbstract.


Other Names for this Disease
  • Aldrich syndrome
  • Eczema thrombocytopenia immunodeficiency syndrome
  • Eczema-thrombocytopenia-immunodeficiency syndrome
  • IMD 2
  • Immunodeficiency 2
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.