{"orpha":300869,"name":"Splenic diffuse red pulp small B-cell lymphoma","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["SDRPL","Splenic diffuse red pulp lymphoma"],"definition":"Splenic diffuse red pulp small B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal proliferation of small, monomorphous, basophilic B-lymphocytes, with villous cytoplasm, in the splenic red pulp, bone marrow and peripheral blood. It typically presents in the late clinical stages with splenomegaly and moderate lymphocytosis. Cytopenias are rare and likely associated with hypersplenism.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-300869/","orphanet_url":"https://www.orpha.net/en/disease/detail/300869","names":{"fr":{"name":"Lymphome splénique diffus de la pulpe rouge à petites cellules B","synonyms":["SDRPL"],"definition":"Le lymphome splénique diffus de la pulpe rouge à petites cellules B est un lymphome non hodgkinien rare et indolent, à cellules B. Il se caractérise par la prolifération anormale de lymphocytes B basophiles, monomorphes, de petite taille et à cytoplasme villeux, au sein de la pulpe rouge splénique, la moelle osseuse et du sang périphérique. Il se manifeste habituellement au cours des stades cliniques tardifs par une splénomégalie et une lymphocytose modérée. La cytopénie est rare et pourrait être associée à un hypersplénisme."},"de":{"name":"B-Zell-Lymphom der roten Pulpa, diffuses splenisches","synonyms":["SDRPL","Splenisches diffuses kleinzelliges B-Zell-Lymphom der roten Pulpa"],"definition":"Das diffuse kleine B-Zell-Lymphom der Milz ist ein seltenes, indolentes B-Zell-Non-Hodgkin-Lymphom, das durch eine abnorme Proliferation kleiner, monomorpher, basophiler B-Lymphozyten mit villösem Zytoplasma in der roten Pulpa der Milz, im Knochenmark und im peripheren Blut gekennzeichnet ist. Sie präsentiert sich typischerweise in den späten klinischen Stadien mit Splenomegalie und moderater Lymphozytose. Zytopenien sind selten und sind vermutlich mit Hypersplenismus assoziiert."},"es":{"name":"Linfoma esplénico de células B con infiltración difusa de la pulpa roja","synonyms":["Linfoma esplénico difuso de la pulpa roja","SDRPL"],"definition":"El linfoma esplénico de células B pequeñas con infiltración difusa de la pulpa roja es un linfoma no-Hodgkin de células B, poco frecuente e indolente, caracterizado por la proliferación anormal de linfocitos B basofílicos monomorfos de pequeño tamaño y citoplasma velloso en la pulpa roja esplénica, médula ósea y sangre periférica. Habitualmente se presenta en estadíos clínicos tardíos con esplenomegalia y linfocitosis moderada. Las citopenias son poco frecuentes y, por lo general, están asociadas a hiperesplenismo."},"it":{"name":"Linfoma splenico diffuso della polpa rossa","synonyms":["Linfoma B splenico diffuso della polpa rossa","SDRPL"],"definition":"Il linfoma splenico diffuso della polpa rossa a piccole cellule B è un linfoma non-Hodgkin a cellule B raro e silente, caratterizzato dalla proliferazione anomala di linfociti B piccoli, basofoli e monomorfi, con citoplasma villoso, nella polpa rossa della milza, nel midollo osseo e nel sangue periferico. Negli stadi clinici più avanzati si manifesta caratteristicamente splenomegalia e linfocitosi moderata. La citopenia è rara e si associa a ipersplenismo."},"nl":{"name":"Splenisch diffuus kleincellig B-cellymfoom van rode pulpa","synonyms":["Diffuus kleincellig B-cellymfoom van rode pulpa van milt","SDRPL","Splenisch diffuus lymfoom van rode pulpa"],"definition":"Splenisch diffuus kleincellig B-cellymfoom van de rode pulpa is een zeldzaam, indolent B-cel non-Hodgkinlymfoom en wordt gekarakteriseerd door abnormale proliferatie van kleine, monomorfe, basofiele B-lymfocyten met harig cytoplasma in de rode pulpa van de milt, het beenmerg en het perifeer bloed. Doorgaans vertoont het splenomegalie en matige lymfocytose in de late klinische fases. Cytopenieën zijn zeldzaam en vermoedelijk geassocieerd met hypersplenisme."},"pt":{"name":"Linfoma difuso da polpa vermelha do baço"},"pl":{"name":"Chłoniak rozlany miazgi czerwonej śledziony z małych komórek B","synonyms":["SDRpl","Chłoniak rozlany miazgi czerwonej śledziony"]},"cs":{"name":"Difuzní lymfom červené dřeně sleziny","synonyms":["SDRPL","Slezinný difuzní B-buněčný lymfom z červené dřeně"]},"uk":{"name":"Селезінкова дифузна червона пульпозна мала В-клітинна лімфома","synonyms":["SDRPL","Дифузна червона пульпозна лімфома селезінки"],"definition":"Splenic diffuse red pulp small B-cell lymphoma is a rare, indolent B-cell non-Hodgkin lymphoma characterized by abnormal proliferation of small, monomorphous, basophilic B-lymphocytes, with villous cytoplasm, in the splenic red pulp, bone marrow and peripheral blood. It typically presents in the late clinical stages with splenomegaly and moderate lymphocytosis. Cytopenias are rare and likely associated with hypersplenism."},"zh":{"name":"脾弥漫性红髓小B细胞淋巴瘤","synonyms":["脾弥漫性红髓淋巴瘤"],"definition":"脾弥漫性红髓小B细胞淋巴瘤是一种罕见的、无痛性B细胞非霍奇金淋巴瘤，其特征是脾脏红髓、骨髓和外周血中有小的单形嗜碱性B淋巴细胞异常增殖伴绒毛胞质。典型临床表现为疾病晚期脾肿大和中度淋巴细胞增多症。细胞减少罕见，可能与脾功能亢进有关。"},"tr":{"name":"Dalak difüz kırmızı pulpa küçük B-hücreli lenfoma","synonyms":["SDRPL","Dalak yaygın kırmızı pulpa lenfoması"]}},"xrefs":[{"source":"ICD-10","code":"C83.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"2A82.Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1970934986"},{"source":"ICD-11","code":"XH99V9","qualifier":"E","icd_relation":"specific","validated":true,"icd11_uri":"1691104199"},{"source":"MONDO","code":"0017599","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C2699508","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":250908,"name":"Rare neoplastic disease"},"parents":[{"orpha":300912,"name":"Marginal zone lymphoma","classifications":[194,202,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[],"phenotypes":null}