{"orpha":98871,"name":"Transient erythroblastopenia of childhood","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Transient acquired pure red cell aplasia"],"definition":"A rare, benign, red cell aplasia of young children or infants characterized by a normocytic normochromic anaemia with severe reticulocytopenia in otherwise normocellular bone marrow, and a complete spontaneous recovery within 1-2 months after diagnosis. Neutropenia and thrombocytosis may be associated findings at diagnosis, and a history of a preceding viral illness is frequent. No organomegaly is observed.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98871/","orphanet_url":"https://www.orpha.net/en/disease/detail/98871","names":{"fr":{"name":"Erythroblastopénie transitoire de l'enfance","synonyms":["Aplasie transitoire acquise de la lignée rouge"],"definition":"Aplasie rare et bénigne de la lignée rouge chez les jeunes enfants ou les nourrissons, caractérisée par une anémie normochrome normocytaire avec une réticulocytopénie sévère dans une moelle osseuse par ailleurs normocellulaire, et une rémission spontanée complète en un à deux mois après le diagnostic. La neutropénie et la thrombocytose peuvent être des signes associés au moment du diagnostic, et des antécédents de maladie virale sont fréquents. Aucune organomégalie n'est observée."},"de":{"name":"Erythroblastopenie, transiente, des Kindesalters","synonyms":["Erythroblastopenie, erworbene transiente"],"definition":"Eine seltene, gutartige Erythrozytenaplasie bei Kleinkindern oder Säuglingen, die durch eine normozytäre normochrome Anämie mit schwerer Retikulozytopenie bei ansonsten normozellulärem Knochenmark gekennzeichnet ist. Die Genesung erfolgt spontan und tritt innerhalb von 1-2 Monaten nach der Diagnose ein. Neutropenie und Thrombozytose können als Begleitbefunde bei der Diagnose auftreten, und häufig liegt eine vorangegangene Viruserkrankung vor. Es wird keine Organomegalie beobachtet."},"es":{"name":"Eritroblastopenia transitoria de la infancia","synonyms":["Aplasia de glóbulos rojos pura adquirida transitoria"],"definition":"Es una aplasia eritrocitaria benigna poco frecuente que afecta a niños pequeños o lactantes caracterizada por anemia normocítica normocrómica con grave reticulocitopenia en una médula ósea por lo demás normocelular y una remisión espontánea completa en los dos primeros meses después del diagnóstico. La neutropenia y la trombocitosis pueden ser hallazgos asociados al diagnóstico, siendo frecuente un antecedente previo de enfermedad viral. No se observan organomegalias."},"it":{"name":"Eritroblastopenia transitoria dell'infanzia","synonyms":["Aplasia pura acquisita transitoria degli eritrociti"],"definition":"È un'aplasia benigna rara degli eritrociti, che colpisce i neonati e i bambini piccoli ed esordisce con un'anemia normocromica normocitica associata ad un'importante reticolocitopenia, nel contesto di un midollo osseo per il resto normocellulare. La guarigione è spontanea e avviene nell'arco di 1-2 mesi dalla diagnosi. Al momento della diagnosi i pazienti possono presentare neutropenia e trombocitosi, spesso con un'anamnesi positiva per precedenti malattie virali. Non si associa ad organomegalia."},"nl":{"name":"Transiënte erytroblastopenie in de kindertijd","synonyms":["Transiënte verworven zuiver erytrocytaire aplasie"],"definition":"Een zeldzame, goedaardige aplasie van rode bloedcellen bij jonge kinderen of zuigelingen, gekarakteriseerd door een normocytaire normochrome anemie met ernstige reticulocytopenie in voor het overige normocellulair beenmerg, en een volledig spontaan herstel binnen 1-2 maanden na de diagnose. Neutropenie en trombocytose zijn mogelijke geassocieerde bevindingen bij de diagnose, en vaak is er een voorgeschiedenis van een eerdere virale ziekte. Er wordt geen organomegalie waargenomen."},"pt":{"name":"Eritroblastopenia da infância, transitória"},"pl":{"name":"Przejściowa erytroblastopenia dziecięca","synonyms":["Przejściowa nabyta aplazja czysto czerwonokrwinkowa"]},"cs":{"name":"Tranzientní erytroblastopenie dětského věku","synonyms":["Tranzientní získaná čistá aplázie červené řady"]},"uk":{"name":"Транзиторна еритробластопенія дитячого віку","synonyms":["Транзиторна набута чиста еритроцитарна аплазія"],"definition":"A rare, benign, red cell aplasia of young children or infants characterized by a normocytic normochromic anaemia with severe reticulocytopenia in otherwise normocellular bone marrow, and a complete spontaneous recovery within 1-2 months after diagnosis. Neutropenia and thrombocytosis may be associated findings at diagnosis, and a history of a preceding viral illness is frequent. No organomegaly is observed."},"zh":{"name":"儿童期短暂性幼红细胞减少症","synonyms":["暂时获得性纯红细胞再生障碍"],"definition":"该病是一种少见的良性红细胞性再生障碍性，多发生于幼童或婴儿，其特征是骨髓中有一种正常细胞的正红细胞贫血，并伴有严重的网织红细胞减少症，确诊后1-2个月内可完全自然恢复。可见中性粒细胞减少症和血小板增多症，既往常有病毒感染史，未观察到器质性肿大。"},"tr":{"name":"Çocuklukta geçici eritroblastopeni","synonyms":["Geçici edinsel saf kırmızı hücre aplazisi"]}},"xrefs":[{"source":"OMIM","code":"227050","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"D60.1","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"3A61.0","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1834257206"},{"source":"MONDO","code":"0009197","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C536980","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0238478","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"7793","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":98421,"name":"Primary acquired red cell aplasia","classifications":[194,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["Childhood","Infancy"],"prevalence":[],"phenotypes":null}