{"orpha":98839,"name":"Intravascular large B-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Angioendotheliomatosis proliferans systemisata","Angiotropic large cell lymphoma","Intravascular lymphomatosis","Malignant angioendotheliomatosis","Tappeiner-Pfleger disease"],"definition":"Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-98839/","orphanet_url":"https://www.orpha.net/en/disease/detail/98839","names":{"fr":{"name":"Lymphome B à grandes cellules intravasculaires","synonyms":["Angioendothéliomatose maligne","Lymphomatose intravasculaire","Lymphome angiotrope à grandes cellules","Maladie de Tappeiner-Pfleger"],"definition":"Forme très rare de lymphome B diffus à grandes cellules, caractérisée par une croissance sélective de cellules lymphomateuses dans la lumière des petits vaisseaux sanguins (en particulier les capillaires), se manifestant dans la plupart des cas par un large éventail de signes cliniques (puisque tous les tissus peuvent être touchés). Les patients des pays occidentaux présentent plus souvent des symptômes neurologiques et cutanés, tandis que ceux des pays asiatiques présentent plus souvent une hépatosplénomégalie et une thrombocytopénie. Une lymphadénopathie n'est pas associée. L'évolution clinique de la maladie est agressive et le pronostic défavorable."},"de":{"name":"B-Zell-Lymphom, intravaskuläres großzelliges","synonyms":["Angioendotheliomatose, maligne","Angioendotheliomatosis proliferans systematisata","Angiotropes großzelliges B-Zell-Lymphom","Lymphomatose, intravaskuläre","Tappeiner-Pfleger-Krankheit"]},"es":{"name":"Linfoma B intravascular de células grandes","synonyms":["Angioendoteliomatosis maligna","Angioendoteliomatosis sistémica proliferante","Enfermedad de Tappeiner-Pfleger","Linfoma angiotrópico de células grandes","Linfomatosis intravascular"],"definition":"Es una forma muy poco frecuente de linfoma B difuso de células grandes caracterizada por el crecimiento selectivo de células de linfoma dentro de la luz de los vasos sanguíneos pequeños (especialmente los capilares) que la mayoría de las veces se presenta con una amplia gama de hallazgos clínicos (puesto que puede afectar a cualquier tejido). Los pacientes de países occidentales manifiestan con mayor frecuencia síntomas neurológicos y cutáneos, mientras que los afectados de países asiáticos muestran hepatoesplenomegalia y trombocitopenia con mayor frecuencia. Este linfoma se caracteriza por la ausencia de linfadenopatías, un curso clínico agresivo y un mal pronóstico."},"it":{"name":"Linfoma intravascolare a grandi cellule B","synonyms":["Angioendoteliomatosi maligna","Angioendoteliomatosi proliferante sistemica","Linfoma angiotropico a grandi cellule","Linfomatosi intravascolare","Malattia di Tappeiner-Pfleger"],"definition":"Il linfoma intravascolare a grandi cellule B (IVLBCL) è una forma molto rara di linfoma diffuso a grandi cellule B (si veda questo termine); è caratterizzato dalla crescita selettiva delle cellule del linfoma all'interno del lume dei piccoli vasi sanguigni (soprattutto i capillari) e nella maggior parte dei casi esordisce con un'ampia gamma di segni clinici, in quanto interessa potenzialmente tutti i tessuti. I pazienti dei Paesi occidentali presentano più spesso sintomi neurologici e cutanei, mentre quelli dei Paesi asiatici presentano più spesso epatosplenomegalia e trombocitopenia. L'IVLBCL non si associa a linfoadenopatia. Il decorso clinico della malattia è aggressivo e la prognosi è sfavorevole."},"nl":{"name":"Intravasculair grootcellig B-cellymfoom","synonyms":["Angio-endotheliomatosis proliferans systemisata","Angiotroop grootcellig lymfoom","Intravasculaire lymfomatose","Kwaadaardige angio-endotheliomatose","Maligne angio-endotheliomatose","Ziekte van Tappeiner-Pfleger"],"definition":"Intravasculair grootcellig B-cellymfoom (IVLBCL) is een zeer zeldzame vorm van diffuus grootcellig B-cellymfoom (zie deze term) die gekarakteriseerd wordt door de selectieve groei van lymfoomcellen in de lumina van kleine bloedvaten (voornamelijk de capillairen), en die meestal tot uiting komt met een brede waaier van klinische manifestaties (aangezien mogelijk eender welk weefsel kan betrokken zijn), waarbij patiënten uit Westerse landen frequenter neurologische en cutane symptomen vertonen, terwijl patiënten uit Aziatische landen frequenter hepatosplenomegalie en trombocytopenie vertonen. IVLBCL wordt gekenmerkt door afwezigheid van lymfadenopathie, een agressief klinisch verloop en een ongunstige prognose."},"pt":{"name":"Linfoma, intravascular, de grandes células B"},"pl":{"name":"Chłoniak wewnątrznaczyniowy z dużych komórek B","synonyms":["Angiotropowy chłoniak wielkokomórkowy","Choroba Tappeinera i Pflegera","Limfomatoza wewnątrznaczyniowa","Złośliwa angioendoteliomatoza"]},"cs":{"name":"Intravaskulární velkobuněčný B-lymfom","synonyms":["Angioendotheliomatosis proliferans systemisata","Intravaskulární lymfomatóza","Maligní angioendoteliomatóza","Tappeinerova-Pflegerova nemoc","Angiotropní velkobuněčný lymfom","Tappeiner-Pflegerova nemoc"]},"uk":{"name":"Внутрішньосудинна велика В-клітинна лімфома","synonyms":["Ангіоендотеліоматоз проліферативний системний (Angioendotheliomatosis proliferans systemisata)","Ангіотропна великоклітинна лімфома","Внутрішньосудинний лімфоматоз","Злоякісний ангіоендотеліоматоз","Хвороба Таппейнера-Пфлегера"],"definition":"Intravascular large B-cell lymphoma (IVLBCL) is a very rare form of diffuse large B-cell lymphoma (see this term) characterized by the selective growth of lymphoma cells within the lumina of small blood vessels (especially the capillaries) that most often presents with a wide range of clinical manifestations (as potentially any tissue can be involved), with patients from Western countries more frequently manifesting with neurological and cutaneous symptoms while patients from Asian countries more frequently displaying hepatosplenomegaly and thrombocytopenia. IVLBCL is characterized by an absence of lymphadenopathy, an aggressive clinical course and a poor prognosis."},"zh":{"name":"血管内大B细胞淋巴瘤","synonyms":["系统性增生性血管内皮瘤病"],"definition":"血管内大B细胞淋巴瘤(IVLBCL)是一种非常罕见的弥漫性大B细胞淋巴瘤(见该词条)，其特征是淋巴瘤细胞在小血管(尤其是毛细血管)的管腔内选择性生长，临床表现多样(因为可能累及任何组织)，西方国家的患者更常出现神经和皮肤症状，而亚洲国家的患者更常见地表现为肝脾肿大和血小板减少。IVLBCL无淋巴结病变，临床病程侵袭性强，预后差。"},"tr":{"name":"İntravasküler büyük B hücreli lenfoma","synonyms":["Tappeiner-Pfleger hastalığı","İntravasküler lenfomatoz","Anjiyotropik büyük hücreli lenfoma","Habis anjioendotelyomatoz","Anjiyoendotelyomatozis proliferans sistemisata"]}},"xrefs":[{"source":"ICD-10","code":"C83.3","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A81.1","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"952730197"},{"source":"MONDO","code":"0020324","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0334660","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10069643","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":544,"name":"Diffuse large B-cell lymphoma","classifications":[194,202,233]}],"children":[],"genes":[{"symbol":"BCL2","name":"BCL2 apoptosis regulator","gene_type":"gene with protein product","association":"Biomarker tested in","status":"Assessed","source":"19040469[PMID]","hgnc":"990","ensembl":"ENSG00000171791","omim":"151430","loci":["18q21.33"]},{"symbol":"BCL6","name":"BCL6 transcription repressor","gene_type":"gene with protein product","association":"Disease-causing somatic mutation(s) in","status":"Assessed","source":null,"hgnc":"1001","ensembl":"ENSG00000113916","omim":"109565","loci":["3q27.3"]}],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}