Idiopathic CD4+ lymphocytopenia | |
---|---|
Other names | Immunodeficiency 13 |
Idiopathic CD4+ lymphocytopenia is inherited via autosomal dominant manner [1] | |
Specialty | Immunology |
Idiopathic CD4+ lymphocytopenia (ICL) is a rare medical syndrome in which the body has too few CD4+ T lymphocytes, which are a kind of white blood cell. [2] ICL is sometimes characterized as "HIV-negative AIDS", though, in fact, its clinical presentation differs somewhat from that seen with HIV/AIDS. [3] People with ICL have a weakened immune system and are susceptible to opportunistic infections, although the rate of infections is lower than in people with AIDS. [4]
The cause of ICL, like all idiopathic conditions, is unknown. It does not appear to be caused by a transmissible agent, such as a virus. [5] It is widely believed that there is more than one cause. [6][ non-primary source needed]
The loss of CD4+ T cells appears to be through apoptosis. [4] [7] The accelerated deaths of the T cells is likely driven by crosslinking T cell receptors. [7]
The mandatory criteria for diagnosis of idiopathic CD4+ lymphocytopenia include: [8]
A one-time finding of low CD4+ cells is usually associated with a recent infection and resolves on its own. [7] Alternative explanations for the low CD4 counts include conditions such as blood cancers ( aleukemia), treatment with chemotherapy, immunosuppressive medications, or other medications that suppress or kill T cells, infections, and problems with blood production. [2] [9][ non-primary source needed]
All criteria must be fulfilled for a diagnosis of ICL. In addition, if these findings are present but combined with other significant findings, such as anemia or thrombocytopenia, then other diagnoses must be considered[ citation needed].
Fludarabine-based hematopoietic stem cell transplantation (HSCT) has shown to be a feasible treatment for ICL. [10][ non-primary source needed]
In contrast to the CD4+ cell depletion caused by HIV, in general, patients with idiopathic CD4 lymphocytopenia have a good prognosis. [6] [11] [12] [13][ non-primary source needed] The decline in CD4+ T-cells in patients with ICL is generally slower than that seen in HIV-infected patients. [3] The major risk to people with ICL is unexpected infections, including cryptococcus, atypical mycobacterial and Pneumocystis jiroveci pneumonia ( PCP). The condition may also resolve on its own. [14]
ICL sometimes precedes and may be the first signal of several blood cancers. ICL patients have developed primary effusion lymphoma, [15][ non-primary source needed] [16] primary leptomeningeal lymphoma, [9] diffuse large cell lymphoma, [17][ non-primary source needed] MALT lymphoma, [18] and Burkitt's lymphoma, [19] among others.
ICL may indirectly trigger autoimmune diseases. It has been associated with several cases of autoimmune disease Sjögren syndrome. [4] [20]
Because all of the reported autoimmune diseases and lymphomas involve B cells, one hypothesis proposes that ICL's narrow T cell repertoire predisposes the immune system to B cell disorders. [4]
ICL is a very rare disease. [2] In 1993, a total of 47 confirmed cases were reported in a survey sponsored by the Centers for Disease Control. [21]
Idiopathic CD4+ lymphocytopenia | |
---|---|
Other names | Immunodeficiency 13 |
Idiopathic CD4+ lymphocytopenia is inherited via autosomal dominant manner [1] | |
Specialty | Immunology |
Idiopathic CD4+ lymphocytopenia (ICL) is a rare medical syndrome in which the body has too few CD4+ T lymphocytes, which are a kind of white blood cell. [2] ICL is sometimes characterized as "HIV-negative AIDS", though, in fact, its clinical presentation differs somewhat from that seen with HIV/AIDS. [3] People with ICL have a weakened immune system and are susceptible to opportunistic infections, although the rate of infections is lower than in people with AIDS. [4]
The cause of ICL, like all idiopathic conditions, is unknown. It does not appear to be caused by a transmissible agent, such as a virus. [5] It is widely believed that there is more than one cause. [6][ non-primary source needed]
The loss of CD4+ T cells appears to be through apoptosis. [4] [7] The accelerated deaths of the T cells is likely driven by crosslinking T cell receptors. [7]
The mandatory criteria for diagnosis of idiopathic CD4+ lymphocytopenia include: [8]
A one-time finding of low CD4+ cells is usually associated with a recent infection and resolves on its own. [7] Alternative explanations for the low CD4 counts include conditions such as blood cancers ( aleukemia), treatment with chemotherapy, immunosuppressive medications, or other medications that suppress or kill T cells, infections, and problems with blood production. [2] [9][ non-primary source needed]
All criteria must be fulfilled for a diagnosis of ICL. In addition, if these findings are present but combined with other significant findings, such as anemia or thrombocytopenia, then other diagnoses must be considered[ citation needed].
Fludarabine-based hematopoietic stem cell transplantation (HSCT) has shown to be a feasible treatment for ICL. [10][ non-primary source needed]
In contrast to the CD4+ cell depletion caused by HIV, in general, patients with idiopathic CD4 lymphocytopenia have a good prognosis. [6] [11] [12] [13][ non-primary source needed] The decline in CD4+ T-cells in patients with ICL is generally slower than that seen in HIV-infected patients. [3] The major risk to people with ICL is unexpected infections, including cryptococcus, atypical mycobacterial and Pneumocystis jiroveci pneumonia ( PCP). The condition may also resolve on its own. [14]
ICL sometimes precedes and may be the first signal of several blood cancers. ICL patients have developed primary effusion lymphoma, [15][ non-primary source needed] [16] primary leptomeningeal lymphoma, [9] diffuse large cell lymphoma, [17][ non-primary source needed] MALT lymphoma, [18] and Burkitt's lymphoma, [19] among others.
ICL may indirectly trigger autoimmune diseases. It has been associated with several cases of autoimmune disease Sjögren syndrome. [4] [20]
Because all of the reported autoimmune diseases and lymphomas involve B cells, one hypothesis proposes that ICL's narrow T cell repertoire predisposes the immune system to B cell disorders. [4]
ICL is a very rare disease. [2] In 1993, a total of 47 confirmed cases were reported in a survey sponsored by the Centers for Disease Control. [21]