Idiopathic CD4+ lymphocytopenia

Idiopathic CD4+ lymphocytopenia is inherited via autosomal dominant manner.

A rare medical syndrome in which the body has too few CD4+ T lymphocytes.

Patients 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.

Cause is unknown. 

It is not caused by a transmissible agent, such as a virus.

The loss of CD4+ T cells appears to be through apoptosis.

The accelerated deaths of the T cells is likely driven by crosslinking T cell receptors.


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+

Laboratory evidence of lack 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.

Other  explanations for the low CD4 counts include: leukemia,  treatment with chemotherapy, immunosuppressive medications, or other medications that suppress or kill T cells, infections, and problems with blood production.

It is associated with increase acceptability to viral, encapsulated, fungal, and mycobacterial diseases.

It is associated with a reduced response to antigens and an increased risk of cancer.


Hematopoietic stem cell transplantation (HSCT) has shown to be a feasible treatment for ICL.

In contrast to the CD4+ cell depletion caused by HIV, in general, patients with idiopathic CD4 lymphocytopenia have a good prognosis.

The decline in CD4+ T-cells in patients with ICL is generally slower than that seen in HIV-infected patients.

The major risks: unexpected infections, including cryptococcus, atypical mycobacterial and Pneumocystis jiroveci pneumonia (PCP). 

The condition may also resolve on its own.

It sometimes precedes and may be the first signal of several blood cancers. 

ICL patients have developed primary effusion lymphoma, primary leptomeningeal lymphoma, diffuse large cell lymphoma, MALT lymphoma, and Burkitt’s lymphoma, among others.

It may indirectly trigger autoimmune diseases. 

It has been associated with several cases of autoimmune disease Sjögren syndrome.

It seems to predispose the immune system to B cell disorders.

Leave a Reply

Your email address will not be published. Required fields are marked *