D82: Immunodeficiency associated with other major defects

D82.0: Wiskott-Aldrich syndrome

The blood cells include the red blood cells, the white blood cells and the platelets. White blood cells are part of the body’s immune system. They fight pathogens. Platelets are important for blood clotting.

With this condition, there are usually not enough platelets. If you don’t have enough platelets, then you may have bleeding. These may only be light bleeds into the skin or mucous membrane, for example. You may get frequent nose bleeds. You may also bleed more easily for a longer time if you hurt yourself.

With this condition, too, the white blood cells do not usually work properly. For example, they then produce fewer antibodies than usual. Some of the immune system’s antibodies can make pathogens and foreign substances visible to the immune system. Other antibodies attack pathogens or foreign substances directly. If you produce fewer antibodies than usual, you may be more susceptible to infection. With this condition you may also get a skin rash.

With this condition, parts of the immune system may also target the body's own tissue or cells. This can cause further illness. The condition may also favor certain types of cancer, such as leukemia.

The disease is hereditary. Other family members can also be affected. It is usually only men who fall ill. Women can pass the condition on to their children without being ill themselves.