Evans syndrome
Synonyms: Immune pancytopenia · Autoimmune hemolytic anemia and autoimmune thrombocytopenia
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult, Childhood
- Status
- active
A rare chronic hematologic disorder characterized by the simultaneous or sequential association of autoimmune hemolytic anemia (AIHA; a disorder in which auto-antibodies are directed against red blood cells causing anemia of varying degrees of severity) with immune thrombocytopenic purpura (ITP; a coagulation disorder in which auto-antibodies are directed against platelets causing hemorrhagic episodes) and occasionally autoimmune neutropenia, in the absence of a known underlying etiology.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Evans syndrome | Immune pancytopenia · Autoimmune hemolytic anemia and autoimmune thrombocytopenia |
| fr | Syndrome d'Evans | Anémie hémolytique et thrombocytopénie autoimmunes · Pancytopénie auto-immune |
| de | Evans-Syndrom | Autoimmune hämolytische Anämie und Immunthrombozytopenie · Immune Panzytopenie |
| es | Síndrome de Evans | Anemia hemolítica autoinmune y trombocitopenia autoinmune · Pancitopenia inmune |
| it | Sindrome di Evans | Anemia emolitica autoimmune e trombocitopenia autoimmune · Pancitopenia immune |
| nl | Syndroom van Evans | Auto-immune hemolytische anemie en auto-immune trombocytopenie · Immuun pancytopenie |
| pt | Síndrome Evans | |
| pl | Zespół Evansa | Autoimmunologiczna niedokrwistość hemolityczna i autoimmunologiczna trombocytopenia · Pancytopenia immunologiczna |
| cs | Evansův syndrom | Autoimunní hemolytická anémie a autoimunní trombocytopenie · Imunitní pancytopenie |
| uk | Синдром Еванса | Аутоімунна гемолітична анемія та аутоімунна тромбоцитопенія · Імунна панцитопенія |
| zh | Evans综合征 | 自身免疫性溶血性贫血伴自身免疫性血小板减少 |
| tr | Evans sendromu | Bağışıklık pansitopeni · Otoimmün hemolitik anemi ve otoimmün trombositopeni |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D69.3 | NTBT | inclusion | validated |
| ICD-11 | 3A20.5 | E | specific | validated |
| MONDO | 0016030 | E | validated | |
| MeSH | C536380 | E | validated | |
| UMLS | C0272126 | E | validated | |
| GARD | 6389 | E | validated | |
| MedDRA | 10053873 | 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 (2):
- ORPHA:98375Autoimmune hemolytic anemia
rare hematological diseases - ORPHA:71203Autoimmune thrombocytopenia
rare hematological diseases
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 | 1-9 / 1 000 000 | 0.1 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
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
13 annotations · validated 2016-12-15| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0001890 | Autoimmune hemolytic anemia | Diagnostic criterion |
| HP:0001973 | Autoimmune thrombocytopenia | |
| Very frequent (99-80%) | ||
| HP:0001904 | Neutropenia in presence of anti-neutropil antibodies | |
| HP:0000967 | Petechiae | |
| Frequent (79-30%) | ||
| HP:0000978 | Bruising susceptibility | |
| HP:0002094 | Dyspnea | |
| HP:0000421 | Epistaxis | |
| HP:0012378 | Fatigue | |
| HP:0000952 | Jaundice | |
| HP:0001254 | Lethargy | |
| HP:0001324 | Muscle weakness | |
| HP:0000980 | Pallor | |
| Occasional (29-5%) | ||
| HP:0001279 | Syncope | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 26190425[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1959
- This record as JSON: /data/d/1959.json