{"orpha":2420,"name":"Primary pulmonary lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare neoplastic disease defined as a clonal lymphoid proliferation affecting one or both lungs (parenchyma and/or bronchi) in a patient with no detectable extrapulmonary involvement at diagnosis or during the subsequent 3 months. PPL comprises low grade/indolent B cell PPL forms, the most frequent form represented by the marginal B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) and other non-MALT low grade lymphomas; and more rarely high-grade B-cell PPL (including diffuse large B cell lymphoma) and lymphomatoid granulomatosis (LYG).","note":null,"url":"https://raremap.alftech.space/d/ORPHA-2420/","orphanet_url":"https://www.orpha.net/en/disease/detail/2420","names":{"fr":{"name":"Lymphome pulmonaire primitif","definition":"Maladie néoplasique rare définie comme une prolifération lymphoïde clonale affectant un ou les deux poumons (parenchyme et/ou bronches) chez un patient sans atteinte extrapulmonaire détectable au moment du diagnostic ou au cours des trois mois suivants. La maladie comprend les formes à cellules B de bas grade/indolentes, la forme la plus fréquente représentée par le lymphome marginal à cellules B du tissu lymphoïde associé à la muqueuse (lymphome MALT) et d'autres lymphomes de bas grade non-MALT ; et plus rarement l'entité à cellules B de haut grade (y compris le lymphome diffus à grandes cellules B) et la granulomatose lymphomatoïde (LYG)."},"de":{"name":"Lungenlymphom, primäres","definition":"Eine seltene Neoplasie, definiert als klonale Proliferation von lymphatischem Gewebe in einer oder beiden Lungen (Parenchym und/oder Bronchien) bei einem Patienten ohne nachweisbare extrapulmonale Beteiligung zum Zeitpunkt der Diagnose oder in den folgenden drei Monaten. Die PPL umfasst niedriggradige/indolente B-Zell-PPL-Formen, wobei die häufigste Form das marginale B-Zell-Lymphom des Mukosa-assoziiertes Lymphgewebes (MALT-Lymphom) und andere niedriggradige Non-MALT-Lymphome sind; seltener sind hochgradige B-Zell-PPL (einschließlich des diffusen großzelligen B-Zell-Lymphoms) und lymphomatoide Granulomatose (LYG)."},"es":{"name":"Linfoma pulmonar primario","definition":"Es una enfermedad neoplásica poco frecuente, definida como una proliferación clonal linfoide que afecta a uno o ambos pulmones (parénquima y/o bronquios) en un paciente sin afectación extrapulmonar detectable en el momento del diagnóstico o durante los siguientes 3 meses. El LPP comprende formas de LPP de células B de bajo grado de malignidad e indolentes, siendo la forma más frecuente la representada por el linfoma de células B de la zona marginal de tejido linfoide asociado a mucosas (linfoma MALT) y otros linfomas no-MALT de bajo grado; y, excepcionalmente, el LPP de células B de alto grado de malignidad (que incluye el linfoma difuso de células B grandes) y la granulomatosis linfomatoide (GL)."},"it":{"name":"Linfoma polmonare primitivo","definition":"Il linfoma polmonare primitivo (PPL) è una neoplasia rara caratterizzata dalla proliferazione linfoide clonale che interessa uno o entrambi i polmoni (parenchima e/o bronchi), in assenza di un evidente interessamento extrapolmonare al momento della diagnosi o nei tre mesi successivi. Il PPL comprende alcune forme a cellule B a basso grado di malignità/silenti, le più comuni delle quali sono il linfoma a cellule B della zona marginale del tessuto linfoide associato alle mucose (linfoma MALT) e altri linfomi non-MALT a basso grado di malignità; e, più raramente, i PPL a cellule B ad alto grado di malignità (compreso il linfoma diffuso a grandi cellule B) e la granulomatosi linfomatoide (LYG)."},"nl":{"name":"Primair pulmonaal lymfoom","definition":"Een zeldzame neoplastische aandoening, gedefinieerd als een klonale lymfoïde proliferatie die één of beide longen treft (parenchym en/of bronchiën) bij een patiënt zonder detecteerbare extrapulmonale betrokkenheid op het moment van de diagnose of gedurende de daaropvolgende 3 maanden. PPL omvat vormen van laaggradig/indolent B-cel PPL, met als meest frequente vorm marginale zone B-cellymfoom van mucosageassocieerd lymfoïd weefsel (MALT-lymfoom), andere niet-MALT laaggradige lymfomen, en in zeldzamere gevallen hooggradig B-cel PPL (waaronder diffuus grootcellig B-cellymfoom) en lymfomatoïde granulomatose (LYG)."},"pt":{"name":"Linfoma pulmonar primário"},"pl":{"name":"Pierwotny chłoniak płuc"},"cs":{"name":"Primární plicní lymfom"},"uk":{"name":"Первинна лімфома легень","definition":"A rare neoplastic disease defined as a clonal lymphoid proliferation affecting one or both lungs (parenchyma and/or bronchi) in a patient with no detectable extrapulmonary involvement at diagnosis or during the subsequent 3 months. PPL comprises low grade/indolent B cell PPL forms, the most frequent form represented by the marginal B-cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma) and other non-MALT low grade lymphomas; and more rarely high-grade B-cell PPL (including diffuse large B cell lymphoma) and lymphomatoid granulomatosis (LYG)."},"zh":{"name":"原发性肺淋巴瘤"},"tr":{"name":"Primer pulmoner lenfoma"}},"xrefs":[{"source":"ICD-10","code":"C85.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2C25.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1042489672"},{"source":"MONDO","code":"0016570","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C4273669","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10037418","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":279911,"name":"Primary organ-specific lymphoma","classifications":[194,202,233]},{"orpha":101945,"name":"Rare bronchopulmonary and pleural cavity tumors","classifications":[184,202]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}