Idiopathic CD4 lymphocytopenia
- Type
- Biological anomaly
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Idiopathic CD4 lymphocytopenia is a rare primary immunodeficiency disorder characterized by persistent CD4 T-cell lymphopenia (less than 300 cells/µL on multiple occasions) not associated with any other underlying primary or secondary immune deficiency. Patients typically present opportunistic infections (with cryptococcal, mycobacterial, candidal, varicella zoster virus infections and progressive multifocal leukoencephalopathy being the most prevalent), malignancies (mainly lymphoproliferative disorders), or autoimmune disorders. Some individuals are asymptomatic and incidentally diagnosed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic CD4 lymphocytopenia | |
| fr | Lymphocytopénie CD4 idiopathique | |
| de | CD4-Lymphozytopenie, idiopathische | |
| es | Linfocitopenia CD4 idiopática | |
| it | Linfocitopenia CD4 idiopatica | |
| nl | Idiopathische CD4-lymfocytopenie | |
| pt | Linfocitopenia CD4 idiopática | |
| pl | Idiopatyczna limfocytopenia CD4 | |
| cs | Idiopatická CD4-pozitivní T-lymfocytopenie | |
| uk | Ідіопатична CD4 лімфоцитопенія | |
| zh | 特发性CD4淋巴细胞减少症 | |
| tr | İdiyopatik CD4 lenfositopeni |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 615518 | E | validated | |
| ICD-10 | D72.8 | NTBT | attributed | validated |
| ICD-11 | 4A00.2 | NTBT | index | validated |
| MONDO | 0014226 | E | validated | |
| UMLS | C4706550 | E | validated | |
| GARD | 12375 | E | validated | |
| MedDRA | 10083934 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:480549Non-severe combined immunodeficiency
rare genetic diseases; rare immunological diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/228000
- This record as JSON: /data/d/228000.json