{"orpha":86870,"name":"Blastic plasmacytoid dendritic cell neoplasm","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["BPDCN"],"definition":"A rare and highly aggressive myeloid malignancy characterized by predominant cutaneous involvement with concomitant or subsequent bone marrow, lymph node and visceral involvement, as well as central nervous system (CNS) disease. It derives from the precursors of plasmacytoid dendritic cells (pDC).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-86870/","orphanet_url":"https://www.orpha.net/en/disease/detail/86870","names":{"fr":{"name":"Tumeur à cellules plasmacytoïdes dendritiques blastiques","synonyms":["TCPDB"],"definition":"Maladie myéloïde rare et très agressive caractérisée par une atteinte cutanée inaugurale, associée à une atteinte concomitante ou survenant plus tard, de la moelle osseuse, des ganglions lymphatiques et des viscères, ainsi qu'à une maladie du système nerveux central (SNC). Le précurseur de la cellule dendritique plasmacytoïde (CDp) en est à l'origine."},"de":{"name":"Blastische plasmazytoide dendritische Zell-Neoplasie","synonyms":["BPDCN","NK-Zell-Lymphom, lymphoblastoide Variante","NK-Zell-Lymphom, monomorphisches"],"definition":"Eine seltene und hochaggressive myeloische Neoplasie, die durch eine vorherrschende Beteiligung der Haut mit gleichzeitiger oder nachfolgender Beteiligung des Knochenmarks, der Lymphknoten und der viszeralen Organe sowie durch eine Erkrankung des zentralen Nervensystems (ZNS) gekennzeichnet ist. Sie geht von den Vorläufern der plasmazytoiden dendritischen Zellen (pDC) aus."},"es":{"name":"Neoplasia blástica de células dendríticas plasmocitoides","synonyms":["BPDCN"],"definition":"Es una neoplasia maligna mieloide poco frecuente y muy agresiva caracterizada por una afección cutánea predominante con afectación concomitante o posterior de la médula ósea, los ganglios linfáticos y las vísceras, así como enfermedad del sistema nervioso central (SNC). Deriva de los precursores de las células dendríticas plasmocitoides (CDp)."},"it":{"name":"Neoplasia a cellule dendritiche plasmacitoidi blastiche","synonyms":["BPDCN"],"definition":"Si tratta di una neoplasia ematologica rara, che origina dai precursori delle cellule dendritiche plasmacitoidi, che spesso interessa la cute, il midollo osseo e i linfonodi, con frequente disseminazione leucemica. Dal punto di vista clinico, la malattia di solito esordisce con lesioni cutanee asintomatiche solitarie o multiple (noduli violacei isolati, papule isolate simili ad ecchimosi, o noduli/macule/papule violacee disseminate). Alcuni pazienti presentano una leucemia. La biopsia della cute evidenzia un infiltrato monomorfo diffuso di blasti di medie dimensioni che assomigliano ai linfoblasti o ai mieloblasti, con un massiccio coinvolgimento del derma. Il decorso clinico è aggressivo e l'età ha un impatto negativo sulla prognosi."},"nl":{"name":"Blastaire plasmacytoïde dendritische cel-neoplasma","synonyms":["BPDCN"],"definition":"Een zeldzame en zeer agressieve myeloïde maligniteit, gekenmerkt door overwegend betrokkenheid van huid met concomitante of daaropvolgende betrokkenheid van beenmerg, lymfeklieren en viscera, alsook aandoening van centraal zenuwstelsel (CZS). Het ontstaat uit precursoren van plasmacytoïde dendritische cellen (pDC)."},"pt":{"name":"Neoplasia de células dendríticas plasmocitóides blásticas","synonyms":["BPDCN"],"definition":"Doença maligna mieloide rara e altamente agressiva, caracterizada por envolvimento predominantemente cutâneo com envolvimento concomitante ou subsequente da medula óssea, gânglios linfáticos e visceral, bem como doença do sistema nervoso central (SNC). Deriva dos precursores das células dendríticas plasmocitoides (CDp)."},"pl":{"name":"Nowotwór hematodermiczny CD4+/CD56+ (blastyczny chłoniak skóry)","synonyms":["BPDCN","Chłoniak z blastycznych komórek NK","Jednopostaciowy chłoniak z komórek NK","Limfoblastoidalny wariant chłoniaka z komórek NK","Nowotwór z blastycznych plazmacytoidalnych komórek dendrytycznych","Nowotwór z blastycznych plazmocytoidnych komórek dendrytycznych"]},"cs":{"name":"CD4+/CD56+ hematodermická neoplázie","synonyms":["BPDCN","Blastický NK-buněčný lymfom","Blastická neoplázie z plazmocytoidních dendritických buněk","Lymfoblastoidní varianta NK-buněčného lymfomu","Monomorfní NK-buněčný lymfom"]},"uk":{"name":"Бластна плазмоцитоїдна дендритна клітинна пухлина","synonyms":["BPDCN"],"definition":"A rare and highly aggressive myeloid malignancy characterized by predominant cutaneous involvement with concomitant or subsequent bone marrow, lymph node and visceral involvement, as well as central nervous system (CNS) disease. It derives from the precursors of plasmacytoid dendritic cells (pDC)."},"zh":{"name":"CD4+/CD56+血液皮肤肿瘤","synonyms":["母细胞性浆细胞样树状突细胞肿瘤"]},"tr":{"name":"CD4 + / CD56 + hematodermik neoplazm","synonyms":["BPDCN","Blastik NK hücreli lenfoma","Monomorfik NK hücreli lenfoma","NK hücreli lenfomanın lenfoblastoid varyantı","Blastik plazmasitoid dendritik hücreli neoplazm"]}},"xrefs":[{"source":"ICD-10","code":"C86.4","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"2A60.5","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"783045723"},{"source":"MONDO","code":"0019467","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"19467","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1301363","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"10556","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":171918,"name":"T-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":12.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2015[OTHER]"},{"type":"Annual incidence","qualification":"Value and class","class":"<1 / 1 000 000","value":0.03,"area":"United States","status":"Validated","source":"36946176[PMID]"}],"phenotypes":null}