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 Table of Contents  
Year : 2018  |  Volume : 30  |  Issue : 4  |  Page : 412-416

HIV negative aids-idiopathic CD4 lyphocytopenia

1 Department of Oral Medicine, Diagnosis and Radiology, Baba Jaswant Singh Dental College and Hospital, Ludhiana, Punjab, India
2 Faculty of Dental Sciences, SGT University, Gurgaon, Haryana, India
3 Department of Periodontics, SKSS Dental College, Ludhiana, Punjab, India

Date of Submission16-Jun-2018
Date of Acceptance20-Aug-2018
Date of Web Publication17-Jan-2019

Correspondence Address:
Dr. Ashima Bali Behl
Department of Oral Medicine, Diagnosis and Radiology, Baba Jaswant Singh Dental College and Hospital, Ludhiana, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaomr.jiaomr_103_18

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Idiopathic CD4+ T-cell lymphocytopenia (ICL) is a rare disorder of immune system with heterogeneous clinical manifestations and immunologic profile. This is a rare condition, which was first defined in 1992 by the Centers for Disease Control and Prevention. It is marked by a CD4 count that is <300 cells/mm3 without human immunodeficiency virus (HIV) infection. Its course differs from that of acquired immunodeficiency syndrome (AIDS), although patients with this disorder may develop opportunistic infections. Hence, the clinicians should be aware of this rare immunologic disorder and that a decrease in the CD4 count is not a hallmark for HIV infection, but could be due to other idiopathic causes as well.

Keywords: CD4 – T-cell lymphocytopenia, HIV negative CD4+ lymphocytopenia, idiopathic CD4+ lymphocytopenia

How to cite this article:
Behl AB, Vohra P, Bali V. HIV negative aids-idiopathic CD4 lyphocytopenia. J Indian Acad Oral Med Radiol 2018;30:412-6

How to cite this URL:
Behl AB, Vohra P, Bali V. HIV negative aids-idiopathic CD4 lyphocytopenia. J Indian Acad Oral Med Radiol [serial online] 2018 [cited 2021 Jun 13];30:412-6. Available from: https://www.jiaomr.in/text.asp?2018/30/4/412/250239

   Introduction Top

Autoimmune manifestations have long been perceived as paradoxical in patients with primary immune deficiencies (PIDs). More than 100 cases of PID have been identified so far. Human immunodeficiency virus (HIV) infection is known to be typically characterized by a profound depression in CD4' lymphocyte numbers in peripheral blood.[l],[2],[3],[4],[5],[6] It is now clear that infection with HIV I & II can result in depletion of CD4 T – helper lymphocytes and development of AIDS.

In the past 10–12 years, several groups have been described as HIV negative patients with CD4 lymphocyte depletion in conjunction with opportunistic infections or Kaposi sarcoma termed as idiopathic CD4 T-lymphocytopenia (ICL).

According to the Centers for Disease Control and Prevention (CDC) in 1992,[1],[2] ICL is a rare syndrome characterized by the depletion in the CD4 T-cells without evidence of HIV infection. The initial identification was based upon several cases of severe opportunistic infections and CD4 T lymphocytopenia in the absence of HIV infection. The clinical, immunologic, and epidemiologic features of this syndrome differ substantially from those of HIV infection. The suggested criteria for diagnosis are:

  1. Absolute CD4 T lymphocyte count <300/ml or <20% of total T cells on more than one occasion
  2. No evidence of HIV infection
  3. Absence of any defined immune deficiency disease or therapy associated with depressed levels of CD4+ T lymphocytes.

Episodic oral erythematous candidiasis and persistent angular cheilitis are an indication of opportunistic infection which may be expected in a reduced state of immunocompetence. The rate at which such CD4 lymphocytopenia occurs varies substantially between individuals.[7],[8],[9] Since risk of acquired immunodeficiency syndrome (AIDS) is small until the CD4 lymphocyte count is low,[9],[10],[11] this variation accounts in large part for the extensive differences between individuals in the time taken for AIDS to develop after infection with HIV.[7],[11] In addition to inter-individual variation in the rate of CD4+ lymphocyte depletion, patients also vary in the CD4+ lymphocyte count at which AIDS develops.[12],[13],[14],[15],[16]


Idiopathic CD4 lymphocytopenia is a rare immunodeficiency of unknown cause that clinically closely resembles a HIV infection, but the absence of this and related viruses by means of all serologic and molecular methods of detection is a crucial element of the case definition.[4] Patients have persistently low CD4 T-cell counts (<300 cells/mm3) and experience opportunistic infections, autoimmune disease, and hematologic malignancies.

The etiology of ICL is not clear.[17],[18] In some cases, low CD4 T lymphocyte counts may reflect transient responses to infections or other conditions or even normal clinical status in asymptomatic patients. In other patients, low CD8 T lymphocyte counts and low immunoglobulin levels (especially IgG) cause suspicion of possible generalized immunodeficiency disorders. ICL may be found in some patients with diseases associated with immunosuppression (Kaposi's sarcoma)[19],[20] and lymphoma.[21],[22] There is no evidence for a transmissible agent or an environmental cause of the disease.[6],[23] The loss of naive T-cells, decreased CD127 (IL-7 receptor) expression, together with increased serum IL-7 have been postulated to be the cause.[24] Sexual and household contacts are not affected and persons who donated blood to the affected patients showed normal CD4 T lymphocyte counts. There is no geographic clustering of affected patients.

The genetic etiology in patients with ICL have been described in different studies:[25]

  • One study described two novel missense mutations in RAG1 gene related to mild clinical ICL phenotype. RAG1 & RAG2 mutations have been responsible for majority of patients and that the RAG mutations can be responsible for the clinical presentations from almost normal to severe immunodeficiencies[26]
  • In the other study, mutations in the magnesium transporter gene, MAGT1, to an X-linked primary immunodeficiency with impaired thymic production of CD4 T cells and circulating T cells were reported
  • Study in 2012 described a heterozygous dominant negative missense mutations of the signaling adaptor protein Uncoordinated 119 (Unc119) in an ICL patient. These mutations disrupts the UNC119 lymphocyte specific kinase (Lck) interaction.[27],[28]


ICL is a condition of unknown etiology and is considered as a heterogeneous syndrome possibly encompassing different disorders sharing a common feature of reduced circulating CD4 T-cell counts.[25] Heterogeneous immunologic profiles have been reported so far in ICL patients. Mechanistic studies of T-cell function in ICL remain scarce. Decreased T-cell responses as well as increased T-cell activation have been reported.[29],[30] The failure of CD4 T-cell homeostasis in ICL could be attributed to decreased production, increased destruction, tissue sequestration, or any combination of these.[25] CD8 T-cell counts remain in the normal range or are often decreased in ICL.[24] Functional investigations have revealed an increased propensity of ICL T cells to undergo apoptosis, a process partially dependent on FAS expression.[17],[19] Markers for activation and turnover are increased in CD4 T cells but not in CD8 T cells, pointing at a specific alteration of the CD4 T-cell compartment.[24] Another factor that may contribute to the CD4 T-cell defect is a decreased clonogenic capacity of the bone marrow (BM) in ICL patients.[31] A frequent alteration observed in ICL consists of increased levels of IL-7 in peripheral blood, consistent with the triggering of a homeostatic response to restore normal CD4 T-cell counts.[18],[32] qw The literature indicates that neither human retroviruses (HIV-1, HIV-2, HTLV-I, or HTLV-II) nor other transmissible agents play a role in the pathogenesis of ICL.[1],[2],[6] Patients with ICL are registered worldwide.[6]

The underlying pathophysiology of this unusual disorder is yet to be ascertained conclusively. Infectious etiology has been ruled out. Some researchers have considered ICL to be an extreme of the spectrum of common variable immunodeficiency.[31] The recent views on potential mechanism for CD4 depletion include FAS/FAS ligand over expression leading to apoptosis[18] defective cytokine (tumor necrosis factor and interferon-gamma) production, regenerative failure of hematopoietic stem/progenitor cells,[32] impaired thymic T-cell maturation,[33] and autoantibody against CD4 cells.[34] The molecular basis of ICL is still unknown. It has been hypothesized that expression or function of CXCR4 could be altered in ICL CD4_ T cells.

Studies have reported the occurrence of CD4 – lymphocytopenia in children also.[3],[4],[5] Only few pediatric cases with this type have been reported from India. The definition of idiopathic CD4+ T-lymphocytopenia among children as given by the CDC includes the following criteria:[6]

  1. A CD4+ T-cell count of <1000 cells/mm3 in children 0–23 months of age and of <300 cells/mm3 in children 2–12 years of age, or a CD4+ T-lymphocyte count that is <20% of total lymphocytes, on at least two separate measurements
  2. No serologic evidence of infection on HIV testing (even if the child's mother is HIV seropositive)
  3. Absence of any defined immunodeficiency or therapy associated with T-cell depletion.

Clinical features

ICL was initially considered to be an extremely rare syndrome.[6],[35] There are numerous reports of ICL associated with different diseases and clinical conditions.[36],[37],[38] Opportunistic infections, mostly seen in HIV patients, are the most common among them.[23],[39],[40],[41],[42],[43] Patients have persistently low CD4 T-cell counts (<300 cells/mms3) and experience opportunistic infections, autoimmune disease, and hematologic malignancies. Clinically ICL may present with a spectrum ranging from asymptomatic to serious infections [Table 1]. Opportunistic infections like candida, cytomegalovirus, and Mycobacterium tuberculosis have been described in adult patients with ICL.[6] Reports have suggested that around 50% of these patients demonstrate at least one skin manifestation ranging from infections to atopic dermatitis to basal cell carcinoma.[44]
Table 1: Conditions seen along with ICL patients

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The repertoire of lymphocyte abnormalities in ICL is distinct from that found in HIV. In ICL generally there is lymphopenia, as unlike HIV infection, the CD8+ counts do not rise.[6] As compared to HIV infected, these patients have decreased counts of CD8+ cells and more lymphopenia.[23],[45]

ICL has been associated with various cutaneous disorders including psoriasis,[46] cutaneous T-cell lymphoma,[47] atopic dermatitis,[48] skin carcinomas including squamous cell carcinoma in situ and basal cell carcinoma,[45] chronic pruritic papules,[49] and cutaneous infections such as papillomavirus, herpes zoster, molluscum contagiosum, and tinea corporis.[44],[50]

Manifestations of proven or suspected autoimmune mechanism associated with common variable immunodeficiency.

Aside from the low CD4 lymphocyte counts, immunologic findings in these patients are distinct from abnormalities found in HIV infection.[23] In general, immunologic evaluation might only reveal reduction in the number or activity of NK cells (CD32, CD161, or CD56), with the preservation of T cells and B cells. A very small percentage of patients with ICL have hypergammaglobulinemia that is typically seen in HIV-infected patients. Almost all of them have normal or slightly low levels of immunoglobulins.[23],[51] As compared to HIV-infected, these patients have lower counts of CD8 cells and more lymphopenia. CD4 CT-cells counts are relatively stable, without progression over time.[23],[45] In these patients, care should be taken to exclude other immunodeficiencies known to be associated with defects of NK cell number or function, such as severe combined immunodeficiency (SCID),[52] Chédiak–Higashi syndrome, X-linked lymphoproliferative disorder, CD40 ligand deficiency, Wiskott–Aldrich syndrome, X-linked agammaglobulinemia, and nuclear factor (NF) kB essential modulator (NEMO) deficiency.[53]


The optimal treatment for this disorder has not been determined. Until the date, the treatment of ICL is mainly symptomatic, with emphasis on prevention and treatment of opportunistic infections. Some novel approaches like allogenic BM transplantation,[54] IL-2 administration[55] has been tried in adult patients and may prove beneficial in the future. The natural history of ICL is variable. Though most reports suggest that these patients have a stable course unlike HIV/AIDS, but mortality remains at high probability due to the recurrent serious opportunistic infections.

According to the report by Wilhelm in 2001, several different phases of an immunologic disorder have responded to IL-2 therapy.

  • In the first phase, ICL was asymptomatic and discovered only by accident
  • The second phase was characterized by monoclonal immunoglobulin increases and associated renal failure manifested by increased levels of creatinine, plus anemia, wasting, and failure to respond to conventional therapies for multiple myeloma
  • A third phase involved IL-2 therapy with alleviation of the major disease manifestations. CD4 T cells increased and the monoclonal gammopathies and creatinine levels decreased.

   Conclusion Top

Autoimmune manifestations are frequent and often multiple in patients with PID. The diagnosis of PID should be made early in disease evolution since it may influence the therapeutic strategy. ICL although a rare entity, should be included in the differential diagnosis of unexplained opportunistic infections and suspected T-cell disorder.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

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