{"orpha":300324,"name":"Persistent polyclonal B-cell lymphocytosis","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["PPBL","Persistent polyclonal B-cell lymphocytosis with binucleated lymphocytes"],"definition":"Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare, generally benign, lymphoproliferative hematological disease characterized by: chronic, stable, persistent, polyclonal lymphocytosis of memory B-cell origin, the presence of binucleated lymphocytes in the peripheral blood, and a polyclonal increase in serum immunoglobulin M (IgM). Patients are most frequently asymptomatic or may present with mild splenomegaly.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300324/","orphanet_url":"https://www.orpha.net/en/disease/detail/300324","names":{"fr":{"name":"Lymphocytose B polyclonale persistante","synonyms":["Hyperlymphocytose B polyclonale persistante","LPLB","Lymphocytose B polyclonale persistante à lymphocytes binucléés","PPBL"],"definition":"La lymphocytose B polyclonale persistante (LPLB) est une maladie hématologique lymphoproliférative rare, généralement bénigne, caractérisée par une lymphocytose polyclonale chronique à lymphocytes B mémoires, persistante et stable, par la présence de lymphocytes binucléés dans le sang périphérique, et par une augmentation polyclonale des immunoglobulines M (IgM) sériques. Les patients sont le plus souvent asymptomatiques, ou peuvent présenter une légère splénomégalie."},"de":{"name":"B-Zell Lymphozytose, persistente polyklonale","synonyms":["PPBL","Persistente polyklonale B-Zell Lymphozytose mit binukleären Lymphozyten"],"definition":"Die persistierende polyklonale B-Zell-Lymphozytose (PPBL) ist eine seltene, im Allgemeinen gutartige, lymphoproliferative hämatologische Erkrankung, die gekennzeichnet ist durch eine chronische, stabile, persistierende, polyklonale Lymphozytose der B-Gedächtniszellen, das Vorhandensein von zweikernigen Lymphozyten im peripheren Blut und einen polyklonalen Anstieg des Serum-Immunglobulin M (IgM). Die Patienten sind meist asymptomatisch oder können sich mit einer leichten Splenomegalie präsentieren."},"es":{"name":"Linfocitosis policlonal de células B persistente","synonyms":["LBPP","Linfocitosis policlonal de células B persistente con linfocitos binucleados","PPBL"],"definition":"La linfocitosis policlonal persistente de células B (PPBL) es una enfermedad hematológica linfoproliferativa poco frecuente y, por lo general, benigna. Está caracterizada por linfocitosis policlonal crónica, estable y persistente originada a partir de células B memoria, presencia de linfocitos binucleados y un aumento policlonal de inmunoglobulina M sérica (IgM). Los afectados son, frecuentemente, asintomáticos o pueden presentar ligera esplenomegalia."},"it":{"name":"Linfocitosi B policlonale persistente","synonyms":["Linfocitosi B policlonale persistente con linfociti binucleati","PPBL"],"definition":"La linfocitosi B policlonale persistente (PPBL) è una malattia ematologica linfoproliferativa rara, di solito benigna, caratterizzata da linfocitosi policlonale cronica, stabile, persistente dei linfociti B della memoria, presenza di linfociti binucleati nel sangue periferico e aumento policlonale dell'immunoglobulina M (IgM) nel siero. Nella maggior parte dei casi, i pazienti sono asintomatici o presentano una lieve splenomegalia."},"nl":{"name":"Persisterende polyklonale B-cellymfocytose","synonyms":["PPBL","Persisterende polyklonale B-cellymfocytose met binucleaire lymfocyten"],"definition":"Persistente polyklonale B-cellymfocytose (PPBL) is een zeldzame, doorgaans goedaardige, lymfoproliferatieve hematologische ziekte die gekarakteriseerd wordt door: chronische, stabiele, persistente, polyklonale lymfocytose met een oorsprong in B-geheugencellen, de aanwezigheid van binucleaire lymfocyten in het perifere bloed, en een polyklonale toename van immunoglobuline M (IgM) in het serum. Patiënten zijn meestal asymptomatisch of kunnen milde splenomegalie vertonen."},"pt":{"name":"Linfocitose persistente de células B policlonais"},"pl":{"name":"Przetrwała poliklonalna limfocytoza B-komórkowa","synonyms":["PPBL","Przetrwała poliklonalna limfocytoza B-komórkowa z limfocytami z podwójnym jądrem"]},"cs":{"name":"Perzistující polyklonální B-buněčná lymfocytóza","synonyms":["PPBL","Perzistentní polyklonální B-buněčná lymfocytóza s dvoujadernými lymfocyty"]},"uk":{"name":"Персистуючий поліклональний В-клітинний лімфоцитоз","synonyms":["Персистуючий поліклональний В-клітинний лімфоцитоз з двоядерними лімфоцитами","PPBL"],"definition":"Persistent polyclonal B-cell lymphocytosis (PPBL) is a rare, generally benign, lymphoproliferative hematological disease characterized by: chronic, stable, persistent, polyclonal lymphocytosis of memory B-cell origin, the presence of binucleated lymphocytes in the peripheral blood, and a polyclonal increase in serum immunoglobulin M (IgM). Patients are most frequently asymptomatic or may present with mild splenomegaly."},"zh":{"name":"持续性多克隆B淋巴细胞增多症","synonyms":["持续性多克隆B淋巴细胞增多症伴双核淋巴细胞"],"definition":"持续性多克隆B细胞增多症（PPBL）是一种罕见的良性淋巴细胞增生性血液病，其特征是：慢性稳定性持续多克隆记忆B细胞增多，外周血中存双核淋巴细胞，血清免疫球蛋白M（IgM）多克隆增加。患者常无症状或出现轻度脾肿大。"},"tr":{"name":"Kalıcı poliklonal B-hücreli lenfositoz","synonyms":["PPBL","İki çekirdekli lenfositler ile seyreden kalıcı poliklonal B hücreli lenfositoz"]}},"xrefs":[{"source":"OMIM","code":"606445","qualifier":"E","icd_relation":null,"validated":true},{"source":"ICD-10","code":"D72.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0011672","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"C564707","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1847973","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":171898,"name":"Lymphoid hemopathy","classifications":[194,202,233]}],"children":[],"genes":[{"symbol":"CARD11","name":"caspase recruitment domain family member 11","gene_type":"gene with protein product","association":"Major susceptibility factor in","status":"Assessed","source":"25352053[PMID]","hgnc":"16393","ensembl":"ENSG00000198286","omim":"607210","loci":["7p22.2"]}],"genes_via_subtypes":[],"inheritance":["Multigenic/multifactorial"],"onset":["No data available"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":154.0,"area":"Worldwide","status":"Validated","source":"18668130[PMID]_21396665[PMID]_2785931[PMID]_7934138[PMID]_11552996[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":null}