Idiopathic CD4 positive T-lymphocytopenia
Prevalence estimates on Rare Medical Network websites are calculated based on data available from numerous sources, including US and European government statistics, the NIH, Orphanet, and published epidemiologic studies. Rare disease population data is recognized to be highly variable, and based on a wide variety of source data and methodologies, so the prevalence data on this site should be assumed to be estimated and cannot be considered to be absolutely correct.
Age of Onset
Autosomal dominant ?A pathogenic variant in only one gene copy in each cell is sufficient to cause an autosomal dominant disease
Autosomal recessive ?Pathogenic variants in both copies of each gene of the chromosome are needed to cause an autosomal recessive disease and observe the mutant phenotype
X-linked dominant ?X-linked dominant inheritance, sometimes referred to as X-linked dominance, is a mode of genetic inheritance by which a dominant gene is carried on the X chromosome.
X-linked recessive ?Pathogenic variants in both copies of a gene on the X chromosome cause an X-linked recessive disorder
Mitochondrial or multigenic ?Mitochondrial genetic disorders can be caused by changes (mutations) in either the mitochondrial DNA or nuclear DNA that lead to dysfunction of the mitochondria and inadequate production of energy.
Multigenic or multifactor ?Inheritance involving many factors, of which at least one is genetic but none is of overwhelming importance, as in the causation of a disease by multiple genetic and environmental factors.
Other Names (AKA)
IMMUNODEFICIENCY 13; IMD13; ICL;
Congenital and Genetic Diseases; Immune System Diseases
Idiopathic CD4 positive T-lymphocytopenia (ICL) is a rare disorder of the
Signs and Symptoms of ICL vary. Some people have no symptoms, however most have illnesses suggestive of a lowered immune system, including infections (varicella-zoster virus, human papilloma virus), autoimmune disorders (autoimmune hemolytic anemia, lupus), and certain types of
- Bacterial: Mycobacterium, Salmonella, Nocardia
- Fungal: Candidiasis, Cryptococcus, Histoplasmosis
- Viral: Cytomegalovirus, varicella zoster virus, human papillomaviruses
- Parasitic: Toxoplasmosis, leishmaniasis
Autoimmune disorders including Sjögren syndrome, sarcoidosis, psoriasis, autoimmune hemolytic anemia, and lupus and malignancies (
This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.
|Medical Terms||Other Names||
|Percent of people who have these symptoms is not available through HPO|
|Bronchiolitis obliterans organizing pneumonia||0011945|
Decreased immune function
Decreased blood lymphocyte number
Low lymphocyte number
[ more ]
|Recurrent otitis media||
Recurrent middle ear infection
The following diseases may also cause decreased CD4+ T cell counts and could be considered as an alternative diagnosis: acute or chronic retroviral infections (HIV, human T-lymphotropic virus type 1), sarcoidosis, common variable immunodeficiency,
Two different methods to increase CD4+ T cell counts have been used on a few affected individuals with varying levels of success: Interleukin-2 (IL2) and
These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.
- The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
- Online Mendelian Inheritance in Man (OMIM) is a catalog of human genes and genetic disorders. Each entry has a summary of related medical articles. It is meant for health care professionals and researchers. OMIM is maintained by Johns Hopkins University School of Medicine.
- Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
- Karin Nielsen-Saines. Idiopathic CD4+ lymphocytopenia. UpToDate. July 31, 2015; https://www.uptodate.com/contents/idiopathic-cd4-lymphocytopenia?source=search_result&search=Idiopathic+CD4%2B+lymphocytopenia&selectedTitle=1~19.
- T-Cells. Arizona State University ask a biologist. https://askabiologist.asu.edu/t-cell. Accessed 8/24/2016.
- Magdalena M. Gorska, Rafeul Alam. A mutation in the human Uncoordinated 119 gene impairs TCR signaling and is associated with CD4 lymphopenia. blood. 2012; 119:1399-1406. https://www.bloodjournal.org/content/119/6/1399.long?sso-checked=true#ref-1.
- Dina S. Ahmad, Mohammad Esmadi, William C. Steinmann. Idiopathic CD4 Lymphocytopenia: Spectrum of opportunistic infections, malignancies, and autoimmune diseases. Avicenna J Med. April-June 2013; 3(2):37-47. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3734630/.
- Luo L, Li T. Idiopathic CD4 lymphocytopenia and opportunistic infection--an update. FEMS Immunol Med Microbiol. Dec 2008; 54(3):283-289. https://femsim.oxfordjournals.org/content/54/3/283.long.
- Gholamin, Mehrana; Bazi, Alib; Abbaszadegan, Mohammad Rezac. Idiopathic lymphocytopenia. Current Opinion in Hematology. January 2015; 22(1):46-52. https://www.ncbi.nlm.nih.gov/pubmed/25463685.
- OncoLink Team. Interleukin-2 (Proleukin®, IL-2, Aldesleukin). OncoLink. January 4, 2016; https://www.oncolink.org/cancer-treatment/chemotherapy/oncolink-rx/interleukin-2-proleukin-r-il-2-aldesleukin.
- Bone marrow transplant. MedlinePlus. 2/12/2016; https://medlineplus.gov/ency/article/003009.htm.