Idiopathic CD4 positive T-lymphocytopenia

What causes idiopathic CD4 positive T-lymphocytopenia (ICL)?

The cause of ICL is not well understood. Contributing factors may be related to CD4+ T cell development and function, various deficiencies of the immune system, and genetic factors.

Last updated on 05-01-20

How is idiopathic CD4 positive T-lymphocytopenia (ICL) diagnosed?

There are no universal diagnostic criteria for ICL; however, it should be suspected in an individual with laboratory findings including CD4+ T cell counts below 300 cells/mm3 or less than 20 percent of total lymphocytes in the absence of other immunodeficiencies, including HIV.

The following diseases may also cause decreased CD4+ T cell counts and could be considered as an alternative diagnosis: acute or chronic retroviral infections (HIV, human T-lymphotropic virus type 1), sarcoidosis, common variable immunodeficiency, congenital immunodeficiencies, and immunosuppressive states induced by chemotherapy, acute respiratory distress syndrome, or autoimmune disorders.

Last updated on 05-01-20

What is the long-term outlook for idiopathic CD4 positive T-lymphocytopenia (ICL)?

The long-term outlook (prognosis) for individuals with ICL varies depending on the extent of immunosuppression and the associated symptoms. Rarely, ICL can be a temporary finding that reverses over time. In most cases the level of CD4+ T cells stabilizes, rather than continuing to decrease.

Last updated on 05-01-20

How might idiopathic CD4 positive T-lymphocytopenia (ICL) be treated?

Treatment of ICL mainly focuses on managing associated symptoms. For individuals without symptoms, regular measurement of CD4+ T cell counts and antibiotic use may be suggested. There are no guidelines regarding how frequently monitoring should occur.

Two different methods to increase CD4+ T cell counts have been used on a few affected individuals with varying levels of success: Interleukin-2 (IL2) and bone marrow transplantation. IL2 is a type of protein naturally occurring in the immune system that works to increase the production and function of the immune system. A bone marrow transplant is a procedure to replace damaged or destroyed bone marrow (soft, fatty tissue inside your bones that produces blood cells) with healthy bone marrow stem cells. Stem cells are immature cells in the bone marrow that give rise to all of your different blood cells.

Last updated on 05-01-20

Walker UA & Warnatz K. Idiopathic CD4 lymphocytopenia Curr Opin Rheumatol. July, 2006; 18(4). 389-95.. Reference Link Gholamin M, Bazi A & Abbaszadegan MR. Idiopathic lymphocytopenia Curr Opin Hematol. January, 2015; 22(1). 46-52. Reference Link Said S & cols. Idiopathic CD4+ lymphocytopenia in Hispanic male: case report and literature review Int Med Case Rep J. July 30, 2014; 7. 117-20. Reference Link

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