Types of PI

Primary Immunodeficiency (PI) is an umbrella term for over 300 genetic defects and disorders of the immune system recognized by the World Health Organization. Together they affect approximately 29,000 Canadians. Primary Immunodeficiency is not considered a rare disease as a category, but each individual form of PI is rare.

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There are 2 names in this directory beginning with the letter W.
WHIM Syndrome
WHIM Syndrome is an autosomal recessive disorder. WHIM stands for Warts, Hypogammaglobulinemia, Infection, Myelokathexis. The condition results in severe warts, recurring infections, and low immunoglobulins and low granulocytes (neutropenia). Myelokathexis is when bone marrow fails to release granulocytes into the body's blood stream. The treatment for the condition involves immunoglobulin replacement and granulocyte growth factor. Credits: Immune Deficiency Foundation

Wiskott-Aldrich Syndrome
Wiskott-Aldrich syndrome (WAS) is a primary immunodeficiency that primarily affects males. The condition causes a poor immune system and abnormal bleeding from a reduced ability to form blood clots. The abnormal bleeding is a result of microthrombocytopenia, which is a decreased number and size of clotting blood cells. Individuals with WAS can usually be identified by three major symptoms: abnormal bleeding, recurrent infections, and eczema of the skin. Individuals with WAS are also at a higher risk of developing cancer (eg. Lymphoma). Symptoms of WAS can be treated with antibiotics and IVIG infusions, while ultimately gene therapy or a bone marrow transplant could cure their condition. Wiskott-Aldrich syndrome is estimated to occur between 1 and 10 cases per million males worldwide. Credits: Immune Deficiency Foundation, Genetics Home Reference
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