{"orpha":443159,"name":"Lymphoplasmacytic lymphoma without IgM production","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Lymphoplasmacytic lymphoma without Immunoglobulin M production"],"definition":"A rare B-cell non-Hodgkin lymphoma characterized by the presence of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells, and either non-secreting or secreting IgG or IgA paraproteins. The disease usually involves the bone marrow, sometimes also the spleen or lymph nodes. Patients typically present with symptoms related to anemia. Hyperviscosity, autoimmune phenomena, and B symptoms may also be observed. Mortality is higher as compared to Waldenström macroglobulinemia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-443159/","orphanet_url":"https://www.orpha.net/en/disease/detail/443159","names":{"fr":{"name":"Lymphome lymphoplasmocytaire sans sécrétion d'IgM","synonyms":["Lymphome lymphoplasmocytaire sans sécrétion d'immunoglobuline M"],"definition":"Lymphome non hodgkinien rare à cellules B, caractérisé par la présence de petits lymphocytes B, de lymphocytes plasmacytoïdes et de plasmocytes, ainsi que de paraprotéines IgG ou IgA non sécrétantes ou sécrétantes. La maladie touche généralement la moelle osseuse, parfois aussi la rate ou les ganglions lymphatiques. Les patients présentent généralement des symptômes liés à l'anémie. Une hyperviscosité, des mécanismes auto-immuns et des symptômes B peuvent également être observés. La mortalité est plus élevée par rapport à la macroglobulinémie de Waldenström."},"de":{"name":"Lymphoplasmozytisches Lymphom ohne IgM-Produktion","definition":"Ein seltenes B-Zell-Non-Hodgkin-Lymphom, das durch das Vorhandensein kleiner B-Lymphozyten, plasmazytoider Lymphozyten und Plasmazellen gekennzeichnet ist und entweder nicht sezernierende oder sezernierende IgG- oder IgA-Paraproteine aufweist. Die Krankheit befällt in der Regel das Knochenmark, manchmal auch die Milz oder die Lymphknoten. Die Patienten zeigen typische Symptome einer Anämie. Hyperviskosität, Autoimmunphänomene und B-Symptome können ebenfalls beobachtet werden. Die Mortalität ist höher als bei der Waldenström-Makroglobulinämie."},"es":{"name":"Linfoma linfoplasmacítico sin producción de IgM","definition":"Es un linfoma no Hodgkin de células B poco frecuente caracterizado por la presencia de pequeños linfocitos B, linfocitos plasmocitoides y células plasmáticas, que pueden secretar o no paraproteínas IgG o IgA. La enfermedad suele afectar a la médula ósea y, en ocasiones, también al bazo o a los ganglios linfáticos. Por lo general, los pacientes se presentan con síntomas asociados a anemia. También se puede observar hiperviscosidad, fenómenos autoinmunes y síntomas B. La mortalidad es mayor que en la macroglobulinemia de Waldenström."},"it":{"name":"Linfoma linfoplasmocitico senza produzione di IgM","definition":"Si tratta di un linfoma raro non-Hodgkin a cellule B, caratterizzato dalla presenza di piccoli linfociti B, linfociti plasmacitoidi, plasmacellule e paraproteine IgG o IgA non secernenti o secernenti. La malattia di solito interessa il midollo osseo e, occasionalmente, anche la milza e i linfonodi. In genere i pazienti presentano i sintomi correlati all'anemia. La malattia può associarsi ad iperviscosità, sintomi autoimmuni e sintomi B. La mortalità è più elevata rispetto alla macroglobulinemia di Waldenström."},"nl":{"name":"Lymfoplasmacytair lymfoom zonder IgM-productie","definition":"Een zeldzaam non-Hodgkinlymfoom van B-cellen, gekarakteriseerd door de aanwezigheid van kleine B-lymfocyten, plasmacytoïde lymfocyten, en plasmacellen, die al dan niet IgG- of IgA-paraproteïnen uitscheiden. De ziekte tast doorgaans beenmerg aan, en soms ook milt of lymfeklieren. Patiënten presenteren zich doorgaans met symptomen gerelateerd aan anemie. Hyperviscositeit, auto-immune fenomenen, en B-symptomen worden mogelijk ook waargenomen. De mortaliteit ligt hoger dan bij macroglobulinemie van Waldenström."},"pt":{"name":"Linfoma linfoplasmocítico sem produção de IgM"},"pl":{"name":"Chłoniak limfoplazmatyczny bez produkcji IgM","synonyms":["Lymphoplasmacytic lymphoma without Immunoglobulin M production"]},"cs":{"name":"Lymfoplazmocytární lymfom bez produkce IgM","synonyms":["Lymfoplazmocytární lymfom bez produkce imunoglobulinu M"]},"uk":{"name":"Лімфоплазмоцитарна лімфома без вироблення IgM","synonyms":["Лімфоплазмоцитарна лімфома без вироблення імуноглобуліну М"],"definition":"A rare B-cell non-Hodgkin lymphoma characterized by the presence of small B-lymphocytes, plasmacytoid lymphocytes, and plasma cells, and either non-secreting or secreting IgG or IgA paraproteins. The disease usually involves the bone marrow, sometimes also the spleen or lymph nodes. Patients typically present with symptoms related to anemia. Hyperviscosity, autoimmune phenomena, and B symptoms may also be observed. Mortality is higher as compared to Waldenström macroglobulinemia."},"zh":{"name":"不产IgM的淋巴浆细胞性淋巴瘤","synonyms":["不产IgM的淋巴浆细胞性淋巴瘤"]},"tr":{"name":"IgM üretimi olmayan lenfoplazmasitik lenfoma","synonyms":["İmmünoglobulin M üretimi olmayan lenfoplazmasitik lenfoma"]}},"xrefs":[{"source":"ICD-10","code":"C83.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018621","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680061","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300842,"name":"Indolent B-cell non-Hodgkin lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":[],"prevalence":[],"phenotypes":null}