{"orpha":314684,"name":"Primary bone lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"Primary bone lymphoma is a rare lymphoid hemopathy defined as single or multiple tumors in the bone, not associated with infringement or violation of other extranodal malignant lymph nodes outside the area. It usually presents with bone pain, nerve compression, a palpable mass or fracture, while systemic features (fever, night sweats, fatigue, loss of appetite, weight loss) are not common.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-314684/","orphanet_url":"https://www.orpha.net/en/disease/detail/314684","names":{"fr":{"name":"Lymphome primitif des os","synonyms":["Lymphome osseux primitif"],"definition":"Hémopathie lymphoïde rare définie par des tumeurs uniques ou multiples situées dans l'os, non associées à une atteinte ou à une infiltration d'autres ganglions lymphatiques malins extranodaux en dehors de la zone. Elle se manifeste généralement par des douleurs osseuses, une compression nerveuse, une masse palpable ou une fracture, tandis que les caractéristiques systémiques (fièvre, sueurs nocturnes, fatigue, perte d'appétit, perte de poids) sont peu fréquentes."},"de":{"name":"Knochenlymphom, primäres","definition":"Das primäre Knochenlymphom ist eine seltene lymphoide Hämopathie, die als einzelner oder multipler Tumor im Knochen definiert ist, der nicht mit einer Verletzung oder Verletzung anderer extranodaler maligner Lymphknoten außerhalb des Gebiets verbunden ist. Es präsentiert sich in der Regel mit Knochenschmerzen, Nervenkompression, einer palpablen Masse oder Fraktur, während systemische Merkmale (Fieber, Nachtschweiß, Müdigkeit, Appetitlosigkeit, Gewichtsverlust) nicht häufig sind."},"es":{"name":"Linfoma óseo primario","definition":"Es una hemopatía linfoide poco frecuente que se define como la presencia de tumores óseos únicos o múltiples, no asociados a infiltración de otros ganglios linfáticos malignos o extranodales fuera del área. Por lo general, se presenta con dolor óseo, compresión nerviosa, masa palpable o fractura, mientras que los hallazgos sistémicos (fiebre, sudoración nocturna, fatiga, pérdida de apetito, pérdida de peso) son infrecuentes."},"it":{"name":"Linfoma primitivo dell'osso","definition":"Il linfoma primitivo dell'osso è un'emopatia linfoide rara, caratterizzata da uno o più tumori ossei, non associati con l'interessamento di altri linfonodi extra-nodali al di fuori dell'area. Di solito esordisce con dolore osseo, compressione dei nervi e presenza di una massa palpabile o di una frattura. Sono rari i segni clinici sistemici (febbre, sudorazione notturna, affaticamento, perdita dell'appetito, perdita di peso)."},"nl":{"name":"Primair botlymfoom","definition":"Een zeldzame lymfoïde hemopathie, gedefinieerd als één of meerdere tumoren in bot, niet geassocieerd met infiltratie van extranodale delen of lymfeklieren buiten dit gebied. Het presenteert zich meestal met botpijn, compressie van zenuwen, en tastbare massa of fractuur, terwijl systemische kenmerken (koorts, nachtzweten, vermoeidheid, verlies van eetlust, gewichtsverlies) niet gangbaar zijn."},"pt":{"name":"Linfoma ósseo primário"},"pl":{"name":"Pierwotny chłoniak kości"},"cs":{"name":"Primární kostní lymfom"},"uk":{"name":"Первинна кісткова лімфома","definition":"Primary bone lymphoma is a rare lymphoid hemopathy defined as single or multiple tumors in the bone, not associated with infringement or violation of other extranodal malignant lymph nodes outside the area. It usually presents with bone pain, nerve compression, a palpable mass or fracture, while systemic features (fever, night sweats, fatigue, loss of appetite, weight loss) are not common."},"zh":{"name":"原发性骨淋巴瘤","definition":"原发性骨淋巴瘤是一种罕见的淋巴性血液病，其定义为骨内单发或多发肿瘤，不侵犯和破坏其它部位的结外恶性淋巴结。它通常表现为骨痛、神经压迫、可触及的肿块或骨折，而全身症状（发热、盗汗、疲劳、食欲不振、体重减轻）并不常见。"},"tr":{"name":"Primer kemik lenfoması"}},"xrefs":[{"source":"ICD-10","code":"C85.7","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0017814","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1332582","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]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}