{"orpha":98832,"name":"Acute myeloid leukemia with minimal differentiation","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["AML M0","Minimally differentiated acute myeloblastic leukemia"],"definition":"A rare subtype of acute myeloid leukemia characterized by clonal proliferation of poorly differentiated myeloid blasts in the bone marrow, blood or other tissues. It usually presents with anemia, thrombocytopenia and other nonspecific symptoms related to ineffective hematopoesis (fatigue, bleeding and bruising, recurrent infections, bone pain) and/or extramedullary site involvement (gingivitis, splenomegaly). Low remission rates are reported.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98832/","orphanet_url":"https://www.orpha.net/en/disease/detail/98832","names":{"fr":{"name":"Leucémie aiguë myéloblastique avec différenciation minimale","synonyms":["AML M0","LAM M0","Leucémie aiguë myéloblastique avec différenciation minime","Leucémie aiguë myéloïde avec différenciation minimale","Leucémie myéloblastique aiguë type peu différenciée"],"definition":"La leucémie aigüe myéloblastique avec différenciation minimale est un sous-type rare de leucémie myéloïde aigüe caractérisé par une prolifération clonale de myéloblastes peu différenciés dans la moelle osseuse, le sang ou d'autres tissus. Elle se présente généralement par une anémie, une thrombocytopénie et par d'autres signes non spécifiques liés à une hématopoïèse inefficace (fatigue, saignements, ecchymoses, infections récurrentes, douleur osseuse) et/ou une atteinte extra-médullaire (gingivite, splénomégalie). Le taux de rémission est bas."},"de":{"name":"Leukämie, akute myeloische, mit minimaler Ausreifung","synonyms":["AML M0"],"definition":"Ein seltener Subtyp der akuten myeloischen Leukämie, der durch klonale Proliferation von schlecht differenzierten myeloischen Blasten im Knochenmark, Blut oder anderen Geweben gekennzeichnet ist. Sie präsentiert sich in der Regel mit Anämie, Thrombozytopenie und anderen unspezifischen Symptomen im Zusammenhang mit ineffektiver Hämatopoese (Müdigkeit, Blutungen und Blutergüsse, rezidivierende Infektionen, Knochenschmerzen) und/oder extramedullärer Beteiligung (Gingivitis, Splenomegalie). Es werden niedrige Remissionsraten berichtet."},"es":{"name":"Leucemia mieloide aguda con mínima diferenciación","synonyms":["AML M0","Leucemia mieloblástica aguda mínimamente diferenciada"],"definition":"Es un subtipo de leucemia mieloide aguda poco frecuente caracterizada por una proliferación clonal de mieloblastos pobremente diferenciados en la médula ósea, sangre u otros tejidos. Por lo general, se presenta con anemia, trombocitopenia y otros síntomas inespecíficos relacionados con la hematopoyesis ineficaz (fatiga, sangrado y hematomas, infecciones recurrentes, dolor de huesos) y/o afectación extramedular (gingivitis, esplenomegalia). Se han descrito bajas tasas de remisión."},"it":{"name":"Leucemia mieloblastica acuta leggermente differenziata","synonyms":["AML M0"],"definition":"La leucemia mieloblastica acuta minimamente differenziata è un sottotipo raro di leucemia mieloide acuta; è caratterizzata dalla proliferazione clonale di mieloblasti poco differenziati nel midollo osseo, nel sangue o in altri tessuti. Di solito esordisce con anemia, trombocitopenia e altri sintomi aspecifici, secondari ad una inadeguata ematopoiesi (affaticamento, emorragie ed ecchimosi, infezioni ricorrenti, dolore osseo), e/o interessamento extramidollare (gengiviti, splenomegalia). Le remissioni sono rare."},"nl":{"name":"Acute myeloïde leukemie met minimale differentiatie","synonyms":["AML M0","Minimaal gedifferentieerde acute myeloblastische leukemie"],"definition":"Een zeldzaam subtype van acute myeloïde leukemie, gekarakteriseerd door klonale proliferatie van weinig gedifferentieerde myeloïde blastcellen in beenmerg, bloed of andere weefsels. Het presenteert zich doorgaans met anemie, trombocytopenie, en andere niet-specifieke symptomen gerelateerd aan ineffectieve hematopoëse (vermoeidheid, bloeding en kneuzing, terugkerende infecties, botpijn) en/of betrokkenheid van extramedullaire regio's (gingivitis, splenomegalie). Er wordt lage mate van remissie gerapporteerd."},"pt":{"name":"Leucemia mieloblástica, aguda, minimamente diferenciada"},"pl":{"name":"Ostra białaczka mieloblastyczna o minimalnym zróżnicowaniu","synonyms":["AML M0","Minimalnie zróżnicowana ostra białaczka mieloblastyczna"]},"cs":{"name":"Akutní myeloblastická leukémie minimálně diferencovaná","synonyms":["AML M0"]},"uk":{"name":"Гостра мієлоїдна лейкемія з мінімальною диференціацією","synonyms":["ГМЛ M0","Мінімально диференційована гостра мієлобластна лейкемія"],"definition":"A rare subtype of acute myeloid leukemia characterized by clonal proliferation of poorly differentiated myeloid blasts in the bone marrow, blood or other tissues. It usually presents with anemia, thrombocytopenia and other nonspecific symptoms related to ineffective hematopoesis (fatigue, bleeding and bruising, recurrent infections, bone pain) and/or extramedullary site involvement (gingivitis, splenomegaly). Low remission rates are reported."},"zh":{"name":"急性髓细胞白血病微分化型","synonyms":["微分化型急性髓系白血病"],"definition":"急性髓系白血病一种罕见的急性髓系白血病亚型，其特征是骨髓、血液或其他组织中的低分化髓母细胞克隆性增殖。它通常表现为贫血、血小板减少和其他与无效造血(疲劳、出血和瘀伤、反复感染、骨痛)和/或髓外部位受累(牙龈炎、脾肿大)等非特异性症状。据报道，该病缓解率很低。"},"tr":{"name":"Minimal farklılaşma ile seyreden akut miyeloid lösemi","synonyms":["AML M0","Minimal diferansiye akut miyeloblastik lösemi"]}},"xrefs":[{"source":"ICD-10","code":"C92.0","qualifier":"NTBT","icd_relation":"inclusion","validated":true},{"source":"ICD-11","code":"2A60.30","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1468530237"},{"source":"ICD-11","code":"XH90G0","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"2099978200"},{"source":"MONDO","code":"0005223","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0522631","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":167714,"name":"Unclassified acute myeloid leukemia","classifications":[194,202,233]}],"children":[],"genes":[{"symbol":"FLT3","name":"fms related receptor tyrosine kinase 3","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":"22338050[PMID]","hgnc":"3765","ensembl":"ENSG00000122025","omim":"136351","loci":["13q12.2"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.01,"area":"Europe","status":"Validated","source":"RARECARE surveillance of rare cancers in Europe[OTHER]"}],"phenotypes":null}