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http://content.nejm.org/cgi/content/abstract/328/6/393
Volume 328:393-398 February 11, 1993 Number 6
Idiopathic CD4+ T-Lymphocytopenia -- Four Patients with Opportunistic Infections and No Evidence of HIV Infection
Robert A. Duncan, C. Fordham von Reyn, George M. Alliegro, Zahra Toossi, Alan M. Sugar, and Stuart M. Levitz
http://www.ncf-net.org/forum/HopewithICL-Fall05.htm
Over a decade ago, Dr. Paul Cheney, Dr. Anthony Komaroff and other CFIDS/ME physicians had identified a condition known as ICL in some of their patients. ICL is the acronym for "Idiopathic CD4 Lymphocytopenia" and it denotes a dramatic loss of a specific T-cell lymphocyte known as a CD4 (T-helper) cell. The extensive reduction in CD4 cells is a hallmark characteristic of AIDS, a disease characterized by a progressive loss of function of the immune system.
Scientists at Enzon Pharmaceuticals have been studying the mechanisms involved in CD4 depletion and have developed a potential drug treatment for CD4 T-cell lymphopenia. Since ICL is typically defined as a CD4 cell count below 200 cells/microliter, a condition that unfortunately is present in some CFIDS/ME patients, this treatment may offer a means to restore some of the immune dysfunction that has occurred as a result of the disease process. More importantly, it may provide a way to reduce opportunistic infections that often accompany this illness since CD4 cells are critical to appropriate immunologic response.
...................Scientists have discovered that some patients with ICL have a deficiency in an important enzyme known as adenosine deaminase.
A deficiency of adenosine deaminase (ADA) results in severe combined immunodeficiency (SCID) in humans. ADA is an enzyme in the purine degradation pathway that deaminates adenosine and deoxyadenosine to yield inosine and deoxyinosine respectively. The enzyme has a wide tissue distribution with the highest activity occurring in lymphocytes. ADA deficiency inhibits the normal catabolism of purines and results in the accumulation of metabolic substrates that are especially toxic to lymphocytes. ADA deficiency results in T-cell apoptosis (cell death) as well as an alteration to T-cell differentiation and therefore an alteration in the production of mature T-cells.
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http://clinicaltrials.gov/ct2/show/NCT00839436
Interleukin-7 (CYT107) Treatment of Idiopathic CD4 Lymphocytopenia: Expansion of CD4 T Cells (ICICLE)
This study is currently recruiting participants.
Verified by National Institutes of Health Clinical Center (CC), January 2009
First Received: February 6, 2009 Last Updated: September 10, 2009
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Linfocitopenia idiopatica delle cellule CD4 |