Idiopathic CD4-Positive T-Lymphocytopenia (T Lymphocytopenia, Idiopathic CD4 Positive)

Reproducible depletion of CD4+ lymphocytes below 300 per cubic millimeter 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.

Also Known As:

T Lymphocytopenia, Idiopathic CD4 Positive; T-Lymphocytopenia, Idiopathic CD4-Positive; CD4+ T-Lymphocytopenia; CD4+ T-Lymphocytopenia, Idiopathic; Idiopathic CD4 Positive T-Lymphocytopenia; Idiopathic CD4+ T-Lymphocytopenia; T-Lymphocytopenia, Idiopathic CD4+; CD4 Positive T Lymphocytopenia, Idiopathic; CD4+ T Lymphocytopenia; CD4+ T Lymphocytopenia, Idiopathic; CD4+ T-Lymphocytopenias; Idiopathic CD4+ T Lymphocytopenia; T Lymphocytopenia, Idiopathic CD4+; T-Lymphocytopenia, CD4+; T-Lymphocytopenias, CD4+; CD4-Positive T-Lymphocytopenia, Idiopathic

Networked: 37 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Infections
2.	Opportunistic Infections (Opportunistic Infection)
3.	Progressive Multifocal Leukoencephalopathy
4.	Common Variable Immunodeficiency
5.	Cryptococcal Meningitis

Experts

1.	Henriksson, G: 2 articles (06/2006 - 02/2000)
2.	Manthorpe, R: 2 articles (06/2006 - 02/2000)
3.	Feng, Yi: 1 article (07/2021)
4.	Huang, Jinghua: 1 article (07/2021)
5.	Jiang, He: 1 article (07/2021)
6.	Lan, Guanghua: 1 article (07/2021)
7.	Liang, Hao: 1 article (07/2021)
8.	Shao, Yiming: 1 article (07/2021)
9.	Yuan, Zongxiang: 1 article (07/2021)
10.	Zhou, Xinjuan: 1 article (07/2021)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Idiopathic CD4-Positive T-Lymphocytopenia:

1.	InterferonsIBA 03/01/2009 - "Studies for impaired immunity including human immunodeficiency virus infection, idiopathic CD4+ T lymphocytopenia syndrome, and interferon deficiency were negative. " 11/01/1994 - "CD4+ T-lymphocytopenia in HIV-infected patients receiving interferon therapy for chronic hepatitis C. "
2.	Adrenal Cortex Hormones (Corticosteroids)IBA 12/01/2004 - "Opportunistic infections mainly occur in patients receiving corticosteroids, and with CD4+ T-lymphocytopenia. " 06/01/2013 - "Pulmonary nocardiosis is exceptional in sarcoidosis and mainly occur, in patients receiving corticosteroids, and with CD4+ T-lymphocytopenia. " 12/01/2004 - "In spite of CD4+ T-lymphocytopenia and corticosteroids-induced immune suppression, the risk of opportunistic infection is not usually considered to be increased in sarcoidosis. " 01/01/1995 - "This case is reported to alert physicians to the possible association of bacterial esophagitis with CD4+ T-lymphocytopenia without HIV infection and to discuss the possible etiological role of corticosteroid treatment." 04/01/2010 - "Fatal cytomegalovirus infection with CD4+ T-lymphocytopenia during corticosteroid therapy for bronchial asthma."
3.	AntibodiesIBA 02/01/2000 - "However, no correlation was found between the presence of anti-CD4 antibodies and CD4+ T lymphocytopenia in the Sjögren patients. " 07/01/1996 - "The administration of drugs to human immunodeficiency virus patients with a pronounced CD4+ T lymphocytopenia may cause non-specific binding of the murine antibodies used in flow cytometry. " 07/01/1996 - "Antibodies against retroviral proteins and nuclear antigens in a subset of idiopathic CD4+ T lymphocytopenia patients."
4.	Mefloquine (Lariam)FDA LinkGeneric 09/01/2017 - "Progressive multifocal leukoencephalopathy in a 44-year old male with idiopathic CD4+ T-lymphocytopenia treated with mirtazapine and mefloquine." 12/01/2015 - "Demonstration of survival and outcome of progressive multifocal leukoencephalopathy (PML) in a 56-year-old patient with common variable immunodeficiency, consisting of severe hypogammaglobulinemia and CD4+ T lymphocytopenia, during continuous treatment with mirtazapine (30 mg/day) and mefloquine (250 mg/week) over 23 months. "
5.	Interleukin-2 (IL2)IBA 11/01/2008 - "Erratum for Yilmaz-Demirdag et al., Interleukin-2 treatment for persistent cryptococcal meningitis in a child with idiopathic CD4+ T-lymphocytopenia." 08/01/2000 - "Successful IL-2 therapy for relapsing herpes zoster infection in a patient with idiopathic CD4+ T lymphocytopenia."
6.	Complement System Proteins (Complement)IBA 04/01/2013 - "Significant positive correlations of CD4+ T lymphocytopenia with other risk factors (parotid swelling, vasculitis, rheumatoid factors, low complement, cryoglobulinemia) were detected. " 06/01/2006 - "Significant predictors of lymphoproliferative disease were purpura/skin vasculitis (hazard ratio (HR) = 4.64, 95% CI 1.13 to 16.45), low complement factor C3 (HR = 6.18, 95% CI 1.57 to 24.22), low C4 (HR = 9.49, 95% CI 1.94 to 46.54), CD4+ T lymphocytopenia (HR = 8.14, 95% CI 2.10 to 31.53), and a low CD4+/CD8+ T cell ratio < or = 0.8 (HR = 10.92, 95% CI 2.80 to 41.83). "
7.	Mirtazapine (Rexer)FDA LinkGeneric 09/01/2017 - "Progressive multifocal leukoencephalopathy in a 44-year old male with idiopathic CD4+ T-lymphocytopenia treated with mirtazapine and mefloquine." 12/01/2015 - "Demonstration of survival and outcome of progressive multifocal leukoencephalopathy (PML) in a 56-year-old patient with common variable immunodeficiency, consisting of severe hypogammaglobulinemia and CD4+ T lymphocytopenia, during continuous treatment with mirtazapine (30 mg/day) and mefloquine (250 mg/week) over 23 months. "
8.	NK Cell Lectin-Like Receptor Subfamily KIBA 01/01/2020 - "We observed lymphadenopathy (LAD), cytopenias, liver disease, cavum septum pellucidum (CSP), and increased CD4-CD8-B220-TCRαβ+ T cells (αβDNTs), in addition to the previously described features of an inverted CD4/CD8 ratio, CD4+ T lymphocytopenia, increased B cells, dysgammaglobulinemia, and decreased expression of the natural killer group 2, member D (NKG2D) receptor. "
9.	Nuclear Antigens (Extractable Nuclear Antigens)IBA 07/01/1996 - "Antibodies against retroviral proteins and nuclear antigens in a subset of idiopathic CD4+ T lymphocytopenia patients."
10.	alpha-beta T-Cell Antigen Receptors (T-Cell Receptor alpha-Chain)IBA 04/01/1997 - "Herein we report a patient with Behçet's like syndrome, idiopathic CD4+ T-lymphocytopenia, opportunistic infections, and a large polyclonal population of TCR alpha beta + CD4- CD8- T cells. "

Therapies and Procedures

1.	Therapeutics 10/01/1996 - "We conclude that allogeneic bone marrow transplantation presents a potentially curative therapy for CD4+ T-lymphocytopenia." 09/01/2002 - "There have been several reports of HIV-negative patients with chronic idiopathic CD4+ T lymphocytopenia, the diagnostic criteria for which are: depressed numbers of circulating T lymphocytes (less than 300/ micro l or less than 20%) on more than one occasion; no laboratory evidence of HIV-1 or HIV-2 infection; and the absence of any defined immunodeficiency or therapy associated with depressed levels of CD4+ T lymphocytes. " 01/01/2010 - "We are hereby reporting one such case we encountered at our centre who presented with monoparesis and subsequently developed multiple cranial nerve palsy and deteriorating sensorium unresponsive to therapy which eventually turned out to be a case of "Idiopathic CD4+ T-Lymphocytopenia" with cryptococcal meningitis." 04/01/2006 - "We describe a patient with progressive multifocal leukoencephalopathy with underlying idiopathic CD4+ T lymphocytopenia in whom functional recovery occurred without antiviral therapy." 11/01/1994 - "CD4+ T-lymphocytopenia in HIV-infected patients receiving interferon therapy for chronic hepatitis C. "
2.	Highly Active Antiretroviral Therapy (HAART) 01/01/2003 - "Two months after starting highly active antiretroviral treatment (HAART), an individual with human immunodeficiency virus type 1 (HIV-1) infection and profound CD4+ T lymphocytopenia developed several erythematous plaques on his face, which were due to borderline tuberculoid leprosy with reversal reaction. "
3.	Photopheresis 08/01/1996 - "Photoaccentuated erythroderma associated with CD4+ T lymphocytopenia: successful treatment with 5-methoxypsoralen and UVA, interferon alfa-2b, and extracorporeal photopheresis."
4.	Kidney Transplantation 06/01/2004 - "We describe a case of late onset Candida albicans and HSV-1 esophagitis, and Pneumocystis carinii pneumonia, that occurs 9 years after renal transplantation in a patient with severe CD4+ T-lymphocytopenia and hypogammaglobulinaemia. "
5.	Bone Marrow Transplantation (Transplantation, Bone Marrow) 10/01/1996 - "Allogeneic bone marrow transplantation can restore CD4+ T-lymphocyte count and immune function in idiopathic CD4+ T-lymphocytopenia." 10/01/1996 - "We conclude that allogeneic bone marrow transplantation presents a potentially curative therapy for CD4+ T-lymphocytopenia."