Idiopathic CD4+ lymphocytopenia
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.[1] ICL is sometimes characterized as "HIV-negative AIDS" by AIDS denialists, though in fact its clinical presentation differs from that seen with HIV/AIDS.[2] 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.[3]
Cause[edit | edit source]
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.[4] It is widely believed that there is more than one cause.[5][non-primary source needed]
Pathophysiology[edit | edit source]
The loss of CD4+ T cells appears to be through apoptosis.[3][6] The accelerated deaths of the T cells is likely driven by crosslinking T cell receptors.[6]
Diagnosis[edit | edit source]
The mandatory criteria for diagnosis of idiopathic CD4+ lymphocytopenia include:[7]
- Low numbers of CD4+ cells, on two or more measurements over at least six weeks:
- CD4 cell count less than 300 cells per microliter, or
- Less than 20% of T lymphocytes are CD4+
- Lack of laboratory evidence of HIV infection
- Absence of any alternative explanation for the CD4 lymphocytopenia
A one-time finding of low CD4+ cells is usually associated with a recent infection and resolves on its own.[6] 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.[1][8][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.
Treatment[edit | edit source]
Fludarabine-based hematopoietic stem cell transplantation (HSCT) has shown to be a feasible treatment for ICL.[9][non-primary source needed]
Prognosis[edit | edit source]
In contrast to the CD4+ cell depletion caused by HIV, in general, patients with idiopathic CD4 lymphocytopenia have a good prognosis.[5][10][11][12][non-primary source needed] The decline in CD4+ T-cells in patients with ICL is generally slower than that seen in HIV-infected patients.[2] 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.[13]
ICL sometimes precedes and may be the first signal of several blood cancers. ICL patients have developed primary effusion lymphoma,[14][non-primary source needed][15] primary leptomeningeal lymphoma,[8] diffuse large cell lymphoma,[16][non-primary source needed] MALT lymphoma,[17] and Burkitt's lymphoma,[18] among others.
ICL may indirectly trigger autoimmune diseases. It has been associated with several cases of autoimmune disease Sjogren's syndrome.[3][19]
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.[3]
Epidemiology[edit | edit source]
ICL is a very rare disease.[1] In 1993, a total of 47 confirmed cases were reported in a survey sponsored by the Centers for Disease Control.[20]
References[edit | edit source]
- ↑ 1.0 1.1 1.2
- ↑ 2.0 2.1
- ↑ 3.0 3.1 3.2 3.3
- ↑ Online Medical Dictionary entry on T-lymphocytopenia Archived 2011-07-19 at the Wayback Machine
- ↑ 5.0 5.1
- ↑ 6.0 6.1 6.2
- ↑ UpToDate article on "Techniques and interpretation of measurement of the CD4 cell count in HIV-infected patients", by John G. Bartlett. Accessed 30 Oct 2006.
- ↑ 8.0 8.1
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External links[edit | edit source]
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Contributors: Prab R. Tumpati, MD
