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Hematopoietic stem cell transplantation:

-   The only known cure as of now. (May not be appropriate for all patients (graft-versus-host))

-   Removal of diseased cell. replacement of healthy cells from healthy donor

-   A bone marrow, peripheral blood or umbilical cord blood transplant from a healthy individual that matches the diseased.

-  The abnormal stem cells will develop into normal immune cells & platelets.

Gene therapy:

-  Similar to stem cell treatment

-   Transplantation of genetically modified stem cells

-   Using a virus as the carrier, the normal copy of the WAS gene is introduced into cells extracted from the sick child's bone marrow. These bone marrow cells are subsequently put back into the patient after they have picked up the virus. The body's normal WAS protein is then produced by bone marrow-derived blood cells as a result of this.

-   However, the virus used to introduce a copy of DNA into the patient's chromosomes may cause abnormal production of a protein that might cause cancer. (only a small percentage of patients developed cancer as a result.)

-  Low to almost zero chances of graft-versus-host disease.

Other types of therapy:

-   Is not a cure but helps patients who sufferer from Wiskott-Aldrich syndrome (WAS)

1.   Immunoglobulin infusions to prevent infections, this therapy can be given either intravenously or subcutaneously.

2.  Antibiotic prophylaxis, or low doses of antibiotics (taken every day or a few days every week) are used to prevent infections that the immune cells of a patient who has WAS will have difficulty avoiding.

3.  Platelet transfusions may be used to prevent or treat bleeding episodes.

4.  Systemic steroids or high-dose immunoglobulin replacement therapy may be used to treat autoimmune complications.

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Explain which branch of immunity is affected 

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Explain which branch of immunity is affected 

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It affects Innate and Adaptive Immune System.

Innate Immune system: It is a non-specific, phagocytic cell deficiency which results in defective intracellular killing of pathogens.

Trigger: Phagocytic cell is missing Wiskott-Aldrich Syndrome protein (WASp). WASp is a protein that is required for immune cell functions like cytoskeletal reorganization, immune synapse formation and intracellular signalling.

Response: Neutrophils are not able to bind to the endothelial cells of the blood capillaries. Macrophages are not able to form phagocytic cups (invagination process by cell membrane formed by actin dependent process)

Process: Neutrophils are not able to extravasate out of the blood capillaries which prevents them from migrating to the site of infection. Macrophages are not able to phagocytose the pathogens since the lack of the WAS protein affects cytoskeletal reorganization, preventing the cell from changing its shape.

Outcome: Increased risk of infection as neutrophils are not able to migrate to the site of infection and the Macrophage are not able to phagocytose the pathogens due to the inability to form phagocytic cups.

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Adaptive immune system. It is specific, that defects in B cell function and T cell function affect humoral and cellular immunity.

Trigger: The Wiskott–Aldrich syndrome protein deficiency leads to impairment of cellular immunity: CD4^+ and CD8^+ T cell and affects the ability of B cell to produce antibodies.

Response: CD4^+ and CD8^+ T cells, CD4^+ T cell fail to produce Th1 cytokines upon TCR activation. Without the release of cytokine from Th1, macrophages cannot become more aggressive, increase the amount of lysosome (digestive enzyme) in the cell, and enhances its killing mechanism. Also, Th1 was supposed to help control antibody class switching to convert some to the memory cells. Th2 cytokine is minimally affected. This means that they are able to produce some antibody IgE to meditate allergens.  Defects in CD8^+ T cell, it cannot kill the infected target cell

Process: T cells lose the ability to stimulate B cells in response to capsular polysaccharides present in some bacteria. CD4^+ T cell is unable to help B cell to class-switched and produced the antibody required.  CD8^+ T cell cannot remove the pathogen effectively.

Outcome: Without treatment, most patients do not live beyond adolescence. Signs of Wiskott-Aldrich Syndrome include eczema and reduced ability to form blood clots. This is due to unable to effectively remove pathogens and produce sufficient IgE to mediate allergens. The syndrome is a recurrent infection, and due to a lack of memory B cells, they will have to fight the pathogen through the primary immune response and not the secondary immune response.

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-Easy bruising and bleeding
-Eczema
-Recurrent infections
-Atopic Dermatities ( dry red patches)

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-Caused by a genetic mutation in WAS gene
-WASP produced by WAS gene
-It reduced the ability to form blood clots due to a low amount of platelets
-This disease is a primary immunodeficiency as it is inherited in an X-linked manner
-Primarily affects males
-With females being the carrier
-Affects the dendritic cells which affect the activation of T cells and B cells

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Rare genetic immunodeficiency that keeps a child's immune system from functioning properly. It also makes it difficult for a child's bone marrow to produce platelets, making a child prone to bleeding. It occurs mostly in males