Primary acquired pure red cell aplasia
Synonyms: Primary acquired PRCA
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare acquired aplastic anemia characterized by a severe normocytic anemia with normal peripheral leukocyte and platelet counts, reticulocytopenia, high serum ferritin and transferrin saturation levels and isolated, almost complete absence of erythroblasts in the bone marrow with normal granulopoesis and megakaryopoesis. It presents with signs of severe anemia (fatigue, lethargy, pallor, intolerance of physical exercise and exertional dyspnea) in the absence of hemorrhagic symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary acquired pure red cell aplasia | Primary acquired PRCA |
| fr | Aplasie pure des globules rouges | Aplasie primaire acquise pure des globules rouges |
| de | Primäre erworbene reine Aplasie der roten Blutkörperchen | Erworbene reine Erythrozyten-Aplasie · Isolierte aplastische Anämie |
| es | Aplasia de glóbulos rojos pura primaria adquirida | PRCA primaria adquirida |
| it | Aplasia eritrocitaria pura primitiva acquisita | PRCA primitiva acquisita |
| nl | Primaire verworven zuivere erytrocytaire aplasie | Primaire verworven PRCA · Primaire verworven zuivere aplasie van rode bloedcel |
| pt | Aplasia pura primária adquirida dos glóbulos vermelhos | |
| pl | Pierwotna izolowana nabyta aplazja układu czerwonokrwinkowego dorosłych | = en |
| cs | Čistá aplázie červené řady u dospělých | = en |
| uk | Первинна набута чиста еритроцитарна аплазія | Первинна набута PRCA |
| zh | 成人单纯红细胞再生障碍 | = en |
| tr | Yetişkin saf kırmızı hücre aplazisi | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | D60.0 | NTBT | attributed | validated |
| ICD-11 | 3A61 | E | specific | validated |
| UMLS | C4707560 | E | validated | |
| GARD | 10898 | 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:98421Primary acquired red cell aplasia
rare hematological diseases; rare transplant-related disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/98872
- This record as JSON: /data/d/98872.json