Idiopathic CD4+ lymphocytopenia

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Idiopathic CD4+ lymphocytopenia
Synonyms Immunodeficiency 13
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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" by AIDS denialists, though in fact its clinical presentation differs 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]

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.[5] It is widely believed that there is more than one cause.[6]

[non-primary source needed]

Pathophysiology[edit | edit source]

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[edit | edit source]

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+
  • 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.[7] 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.[2][9]

[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.[10]

[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.[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 sometimes precedes and may be the first signal of several blood cancers. ICL patients have developed primary effusion lymphoma,[15]

[non-primary source needed][16] primary leptomeningeal lymphoma,[9] diffuse large cell lymphoma,[17]

[non-primary source needed] MALT lymphoma,[18] and Burkitt's lymphoma,[19] among others.

ICL may indirectly trigger autoimmune diseases. It has been associated with several cases of autoimmune disease Sjogren's 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]

Epidemiology[edit | edit source]

ICL is a very rare disease.[2] In 1993, a total of 47 confirmed cases were reported in a survey sponsored by the Centers for Disease Control.[21]

References[edit | edit source]

  1. ↑ OMIM Entry - # 615518 - IMMUNODEFICIENCY 13; IMD13 Full text, omim.org,
  2. ↑ 2.0 2.1 2.2 , Idiopathic CD4 lymphocytopenia, Curr Opin Rheumatol, Vol. 18(Issue: 4), pp. 389–95, DOI: 10.1097/01.bor.0000231908.57913.2f, PMID: 16763460, Full text,
  3. ↑ 3.0 3.1 , Idiopathic CD4 lymphocytopenia and opportunistic infection--an update, FEMS Immunol. Med. Microbiol., Vol. 54(Issue: 3), pp. 283–9, DOI: 10.1111/j.1574-695X.2008.00490.x, PMID: 19049641,
  4. ↑ 4.0 4.1 4.2 4.3 , Idiopathic CD4+ lymphocytopenia and Sjogren syndrome, Arch. Ophthalmol., Vol. 123(Issue: 7), pp. 1012, DOI: 10.1001/archopht.123.7.1012-a, PMID: 16009850, Full text,
  5. ↑ Online Medical Dictionary entry on T-lymphocytopenia Archived 2011-07-19 at the Wayback Machine
  6. ↑ 6.0 6.1 , Idiopathic CD4+ T-lymphocytopenia--four patients with opportunistic infections and no evidence of HIV infection, N. Engl. J. Med., Vol. 328(Issue: 6), pp. 393–8, DOI: 10.1056/NEJM199302113280604, PMID: 8093636,
  7. ↑ 7.0 7.1 7.2 , Apoptotic depletion of CD4+ T cells in idiopathic CD4+ T lymphocytopenia, J. Clin. Invest., Vol. 97(Issue: 3), pp. 672–80, DOI: 10.1172/JCI118464, PMID: 8609222, PMC: 507103,
  8. ↑ 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.
  9. ↑ 9.0 9.1 , Primary leptomeningeal lymphoma in a patient with concomitant CD4+ lymphocytopenia, Ann. Allergy Asthma Immunol., Vol. 88(Issue: 3), pp. 339–42, DOI: 10.1016/S1081-1206(10)62019-4, PMID: 11926631,
  10. ↑ , Successful fludarabine-based hematopoietic stem cell transplantation in a pediatric patient with idiopathic CD4+ lymphocytopenia, Pediatric Transplantation, 2013, Vol. 17(Issue: 4), pp. E109–11, DOI: 10.1111/petr.12086, PMID: 23581828,
  11. ↑ , Acquired immunodeficiency without evidence of infection with human immunodeficiency virus types 1 and 2., Lancet, 1992, Vol. 340(Issue: 8814), pp. 273–4, DOI: 10.1016/0140-6736(92)92359-N, PMID: 1353194,
  12. ↑ , Idiopathic CD4+ T-lymphocytopenia--immunodeficiency without evidence of HIV infection., N Engl J Med, 1993, Vol. 328(Issue: 6), pp. 380–5, DOI: 10.1056/NEJM199302113280602, PMID: 8093634,
  13. ↑ , Idiopathic CD4+ T-lymphocytopenia--an analysis of five patients with unexplained opportunistic infections., N Engl J Med, 1993, Vol. 328(Issue: 6), pp. 386–92, DOI: 10.1056/NEJM199302113280603, PMID: 8093635,
  14. ↑ , Idiopathic CD4+ lymphocytopenia: natural history and prognostic factors, Blood, Vol. 112(Issue: 2), pp. 287–294, DOI: 10.1182/blood-2007-12-127878, PMID: 18456875, PMC: 2442741,
  15. ↑ , Human herpes virus 8-negative primary effusion lymphoma with BCL6 rearrangement in a patient with idiopathic CD4 positive T-lymphocytopenia, Haematologica, Vol. 93(Issue: 1), pp. e21–3, DOI: 10.3324/haematol.12085, PMID: 18166773,
  16. ↑ , PEL, Kaposi's sarcoma HHV8+ and idiopathic T-lymphocitopenia CD4+, Clin Ter, 2007, Vol. 158(Issue: 2), pp. 151–5, PMID: 17566517,
  17. ↑ , Diffuse large cell lymphoma and t(8;22) (q24;q11) in a patient with idiopathic CD4+ T-lymphopenia, Leuk. Lymphoma, Vol. 41(Issue: 3–4), pp. 421–3, DOI: 10.3109/10428190109057998, PMID: 11378556,
  18. ↑ , [Multifocal MALT lymphoma and acute cytomegalovirus gastritis revealing CD4 lymphopenia without HIV infection], Gastroenterol. Clin. Biol., Vol. 23(Issue: 1), pp. 132–6, PMID: 10219614,
  19. ↑ , [Idiopathic CD4+ T-lymphocytopenia terminating in Burkitt's lymphoma], Rinsho Ketsueki, Vol. 38(Issue: 7), pp. 599–603, PMID: 9267164,
  20. ↑ , CD4+ T-lymphocytopenia--a frequent finding in anti-SSA antibody seropositive patients with primary Sjögren's syndrome, J. Rheumatol., Vol. 31(Issue: 4), pp. 726–8, PMID: 15088298, Full text,
  21. ↑ , 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, 1993, Vol. 328(Issue: 6), pp. 373–9, DOI: 10.1056/NEJM199302113280601, PMID: 8093633,

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Contributors: Prab R. Tumpati, MD