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 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]
Diagnosis
The mandatory criteria for diagnosis of idiopathic CD4+ lymphocytopenia include:[8]
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
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].
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 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]
^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.
^ abBusse PJ, Cunningham-Rundles C (March 2002). "Primary leptomeningeal lymphoma in a patient with concomitant CD4+ lymphocytopenia". Ann. Allergy Asthma Immunol. 88 (3): 339–42. doi:10.1016/S1081-1206(10)62019-4. PMID11926631.
^Hamidieh, A. A.; Pourpak, Z.; Hamdi, A.; Nabavi, M.; Ghavamzadeh, A. (2013). "Successful fludarabine-based hematopoietic stem cell transplantation in a pediatric patient with idiopathic CD4+ lymphocytopenia". Pediatric Transplantation. 17 (4): E109–11. doi:10.1111/petr.12086. PMID23581828. S2CID206265698.
^Richetta A, Amoruso GF, Ascoli V, et al. (2007). "PEL, Kaposi's sarcoma HHV8+ and idiopathic T-lymphocitopenia CD4+". Clin Ter. 158 (2): 151–5. PMID17566517.
^Campbell JK, Prince HM, Juneja SK, Seymour JF, Slavin M (April 2001). "Diffuse large cell lymphoma and t(8;22) (q24;q11) in a patient with idiopathic CD4+ T-lymphopenia". Leuk. Lymphoma. 41 (3–4): 421–3. doi:10.3109/10428190109057998. PMID11378556. S2CID23366810.
^Longo F, Hébuterne X, Michiels JF, Maniere A, Caroli-Bosc FX, Rampal P (January 1999). "[Multifocal MALT lymphoma and acute cytomegalovirus gastritis revealing CD4 lymphopenia without HIV infection]". Gastroenterol. Clin. Biol. (in French). 23 (1): 132–6. PMID10219614.
^Shimano S, Murata N, Tsuchiya J (July 1997). "[Idiopathic CD4+ T-lymphocytopenia terminating in Burkitt's lymphoma]". Rinsho Ketsueki (in Japanese). 38 (7): 599–603. PMID9267164.
