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Wiskott-Aldrich syndrome is a rare genetic immunodeficiency that can be inherited or caused by mutations that results in a reduced ability to form blood clots. Wiskott-Aldrich syndrome is caused by mutations in the WAS gene which codes for the WASp protein. This gene is on the X chromosome and therefore the Wiskott-Aldrich syndrome is an X-linked disease and causes boys to be more affected as they have only one X chromosome. This protein is responsible for relaying signals from the surface of the blood cell to the cell's actin cytoskeleton. Therefore, in people who have this syndrome, their immune cells lack the WASp protein and therefore have a decreased ability in migrating, fight invades, and form functional platelets.
This shows that it is a primary immunodeficiency as it is inherited or genetic and manifests from an early age.

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(ii) Explain which branch of immunity is affected. (e.g., innate, adaptive, etc)

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(ii) Explain which branch of immunity is affected. (e.g., innate, adaptive, etc)

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Wiskott-Aldrich Syndrome affects both innate and adaptive immunity.

For innate immunity, CD8+ t cells are overly expressed due to the enhanced cross-presentation caused by the genetic mutation of the WAS gene. This will lead to a decreased number of Langerhans cells migrating to the skin from the lymph nodes, leading to skin inflammation. The skin inflammation is also contributed by the increased number of cytokines being released by the macrophages.

For adaptive immunity, the memory b cell activation is affected in patients with this syndrome. Due to the decreased number of activated memory b cells, recurrent infections may occur. This is due to the reduced transcription of the B-cell receptor co-receptor CD-19. This impedes the signalling between the memory B cells, leading to recurrent infections

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Symptoms may begin at any point in the person's life. It may be different from person to person and can range from mild to severe depending on how long the Wiskott-Aldrich syndrome persisted and when was it treated.
Some examples would be:
1) Abnormal in the shapes of cells -> an oddity in platelet form, ultrastructure, or intracellular organelles.
2) Low level in the number of red blood cells or haemoglobin -> a reduction in erythrocytes volume or haemoglobin concentration.
3) Eczema -> a range of skin conditions and can cause dryness and rashes of redness of the skin, crusting, flaking and bleeding.

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Currently, there is only one known treatment for Wiskott-Aldrich syndrome (WAS), which is hematopoietic stem cell transplant. Where bone marrow, peripheral blood or umbilical cord blood from a healthy and suitable tissue matched donor will be transplanted and replaced into the bloodstream of individuals with this syndrome. This will prompt blood cells coming from the bone marrow and allow stem cells to develop into normal immune cells and platelets.

A study shows that Wiskott-Aldrich syndrome can be treated using lentiviral gene therapy that reduces bleedings and infection episodes in children with a severe immune disorder of this syndrome. According to the research, most patients treated with a lentiviral gene therapy had a significant reduction in the number and severity of infections, where they were able to stop taking antibiotics needed to protect them from regular infections. In addition to that, this treatment could extend the life expectancy of children with WAS by decades and vastly improve their quality of life. However, this research is still ongoing but shows promise in treating WAS.