Idiopathic CD4 positive T-lymphocytopenia

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Disease Overview

Idiopathic CD4 positive T-lymphocytopenia (ICL) is a rare disorder of the immune system. People with ICL have low levels of a type of white blood cell, called a CD4+ T cell. These low levels can not be explained by other causes of immunodeficiency, including HIV infection.[12089] T cells have many jobs in our immune system, such as attacking bacteria and viruses.[12090] CD4 is a protein found on the surface of many different cells within your immune system. It lets the different cells of your immune system work with each other. When CD4+ T cells are decreased, your body becomes more prone to infection.[12089]

Signs and Symptoms of ICL vary. Some people have no symptoms, however most have illnesses suggestive of a lowered immune system, including infections (varicella-zoster virus, human papilloma virus), autoimmune disorders (autoimmune hemolytic anemia, lupus), and certain types of cancer (non-Hodgkin lymphoma).[12089] A few people with ICL are found to carry specific gene mutations; however, for most cases of ICL the underlying cause is not known.[12089][12091] Currently, there is no cure for ICL, but treatments are available to help manage individual symptoms.[12089]


Synonyms

  • IMMUNODEFICIENCY 13
  • IMD13
  • ICL
  • IDIOPATHIC CD4 LYMPHOPENIA
  • Idiopathic CD4 lymphocytopenia

For more information, visit GARD.

National Organization for Rare Disorders