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Idiopathic CD4+ lymphocytopenia (ICL) is a depletion of CD4+ lymphocytes below 300 per cubic millimeter, or less than 20% of total lymphocytes, in the absence of HIV infection or other known causes of immunodeficiency. This is a rare, heterogeneous syndrome and does not appear to be caused by a transmissible agent.[1] People with ICL are susceptible to opportunistic infections, namely, cryptococcus, atypical mycobacterial and Pneumocystis jiroveci pneumonia (PCP).

Criteria for diagnosis of idiopathic CD4 lymphocytopenia include:[2]

  • CD4 cell count less than 300/mm3 or a CD4 percent less than 20 of total lymphocytes on two or more measurements
  • Lack of laboratory evidence of HIV infection
  • Absence of an alternative explanation for the CD4 lymphocytopenia

ICL is a rare disease; in 1993, a total of 47 confirmed cases were reported in a survey sponsored by the Centers for Disease Control.[3] In contrast to the CD4 cell depletion caused by HIV, patients with idiopathic CD4 lymphocytopenia generally have a good prognosis.[4][5][6][7]

See also


  1. Online Medical Dictionary entry on T-lymphocytopenia
  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.
  3. Smith D, Neal J, Holmberg S (1993). "Unexplained opportunistic infections and CD4+ T-lymphocytopenia without HIV infection. An investigation of cases in the United States. The Centers for Disease Control Idiopathic CD4+ T-lymphocytopenia Task Force". N Engl J Med. 328 (6): 373–9. PMID 8093633.
  4. Laurence J, Siegal F, Schattner E, Gelman I, Morse S (1992). "Acquired immunodeficiency without evidence of infection with human immunodeficiency virus types 1 and 2". Lancet. 340 (8814): 273–4. PMID 1353194.
  5. Ho D, Cao Y, Zhu T, Farthing C, Wang N, Gu G, Schooley R, Daar E (1993). "Idiopathic CD4+ T-lymphocytopenia--immunodeficiency without evidence of HIV infection". N Engl J Med. 328 (6): 380–5. PMID 8093634.
  6. Spira T, Jones B, Nicholson J, Lal R, Rowe T, Mawle A, Lauter C, Shulman J, Monson R (1993). "Idiopathic CD4+ T-lymphocytopenia--an analysis of five patients with unexplained opportunistic infections". N Engl J Med. 328 (6): 386–92. PMID 8093635.
  7. Duncan R, von Reyn C, Alliegro G, Toossi Z, Sugar A, Levitz S (1993). "Idiopathic CD4+ T-lymphocytopenia--four patients with opportunistic infections and no evidence of HIV infection". N Engl J Med. 328 (6): 393–8. PMID 8093636.