{"orpha":160,"name":"Castleman disease","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Angiofollicular ganglionic hyperplasia","Angiofollicular lymph hyperplasia"],"definition":"A rare lymphoid hemopathy characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, idiopathic multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-160/","orphanet_url":"https://www.orpha.net/en/disease/detail/160","names":{"fr":{"name":"Maladie de Castleman","synonyms":["Hyperplasie ganglionnaire angiofolliculaire","Hyperplasie lymphoïde angiofolliculaire","Pseudotumeur de Castleman"],"definition":"Maladie lymphoproliférative rare caractérisée par une atteinte des ganglions lymphatiques pouvant affecter toute partie du corps, le plus souvent le médiastin, l'abdomen, le cou ou la rate, se présentant sous la forme d'une maladie de Castleman unicentrique, multicentrique idiopathique ou multicentrique associée à KSHV/HHV8. Selon le type, les patients sont le plus souvent asymptomatiques ou présentent des symptômes d'atteinte systémique."},"de":{"name":"Castleman-Krankheit","synonyms":["Angiofollikuläre Lymphknotenhyperplasie","Castleman-Lymphom"],"definition":"Eine seltene lymphoide Hämopathie, die durch den Befall von Lymphknoten in allen Körperregionen gekennzeichnet ist, am häufigsten im Mediastinum, im Bauchraum, am Hals oder in der Milz, und die als unizentrische, idiopathische multizentrische oder KSHV/HHV8-assoziierte multizentrische Castleman-Krankheit auftritt. Je nach Typ sind die Patienten in der Regel asymptomatisch oder weisen systemische Symptome auf."},"es":{"name":"Enfermedad de Castleman","definition":"Es un trastorno linfoproliferativo poco frecuente caracterizado por la afectación de los ganglios linfáticos en cualquier parte del cuerpo, con mayor frecuencia en el mediastino, el abdomen, el cuello o el bazo, y que se presenta como enfermedad de Castleman unicéntrica, multicéntrica idiopática o multicéntrica asociada a KSHV/HHV8. Según el tipo, los pacientes pueden permanecer asintomáticos o presentar síntomas sistémicos."},"it":{"name":"Malattia di Castleman","synonyms":["Iperplasia ganglionaria angiofollicolare","Iperplasia linfatica angiofollicolare"]},"nl":{"name":"Ziekte van Castleman","synonyms":["Angiofolliculaire ganglionhyperplasie","Angiofolliculaire lymfehyperplasie"],"definition":"Een zeldzame lymfoïde hemopathie, gekenmerkt door betrokkenheid van lymfeklieren in eender welk deel van het lichaam, meestal mediastinum, abdomen, hals of milt, en optredend als unicentrische, idiopathische multicentrische, of KSHV/HHV8-geassocieerde multicentrische ziekte van Castleman. Afhankelijk van het type zijn patiënten meestal asymptomatisch of vertonen ze doorgaans systemische symptomen."},"pt":{"name":"Doença Castleman","synonyms":["Hamartoma linfóide","Hiperplasia ganglionar angiofolicular","Linfoma benigno"],"definition":"Hemopatia linfóide rara caracterizada pelo envolvimento de gânglios linfáticos em qualquer parte do corpo, mais frequentemente no mediastino, abdómen, pescoço ou baço, e ocorrendo como doença Castleman unicêntrica, multicêntrica idiopática ou multicêntrica associada ao KSHV/HHV8. Dependendo do tipo, os doentes são geralmente assintomáticos ou apresentam sintomas sistémicos."},"pl":{"name":"Choroba Castlemana","synonyms":["Angiofollicular ganglionic hyperplasia","Naczyniowogrudkowy przerost węzłów chłonnych"],"definition":"Rzadka hemopatia limfatyczna charakteryzująca się zajęciem węzłów chłonnych dowolnej części ciała, najczęściej śródpiersia, jamy brzusznej, szyi lub śledziony, występująca jako jednocentryczna, idiopatyczny multicentryczna lub multicentryczna związaną z KSHV/HHV8 choroba Castlemana. W zależności od typu, pacjenci są najczęściej nie maja objawów lub występują w nich objawy ogólnoustrojowe."},"cs":{"name":"Castlemanova choroba","synonyms":["Angiofolikulární ganglionická hyperplázie","Angiofolikulární hyperplázie uzlin"]},"uk":{"name":"Хвороба Кастлемана","synonyms":["Ангіофолікулярна гангліонарна гіперплазія","Ангіофолікулярна лімфатична гіперплазія"],"definition":"A rare lymphoid hemopathy characterized by involvement of lymph nodes in any part of the body, most frequently the mediastinum, abdomen, neck, or spleen, and occurring as unicentric, idiopathic multicentric, or KSHV/HHV8-associated multicentric Castleman disease. Depending on the type, patients are most commonly asymptomatic or typically present with systemic symptoms."},"zh":{"name":"Castleman病","synonyms":["血管滤泡性淋巴增生"]},"tr":{"name":"Castleman hastalığı","synonyms":["Anjiyofoliküler lenf hiperplazisi","Anjiyofoliküler ganglionik hiperplazi"]}},"xrefs":[{"source":"OMIM","code":"148000","qualifier":"BTNT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"D47.7","qualifier":"NTBT","icd_relation":"index","validated":true},{"source":"ICD-11","code":"4B2Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1940989685"},{"source":"MONDO","code":"0015564","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D005871","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0017531","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"12656","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10050251","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":93682,"name":"OBSOLETE: Pediatric Castleman disease","status":"inactive"},{"type":"Referred to","orpha":93686,"name":"OBSOLETE: Multicentric Castleman disease","status":"inactive"}],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":171898,"name":"Lymphoid hemopathy","classifications":[194,202,233]}],"children":[{"orpha":570438,"name":"HHV-8-associated multicentric Castleman disease","type":"Clinical subtype"},{"orpha":570431,"name":"Idiopathic multicentric Castleman disease","type":"Clinical subtype"},{"orpha":93685,"name":"Unicentric Castleman disease","type":"Clinical subtype"}],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"United States","status":"Validated","source":"ORPHANET_25120049[PMID]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-04-30","source":"30598916[PMID]_30746379[PMID]_30680887[PMID]","terms":[{"hpo":"HP:0002716","label":"Lymphadenopathy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001903","label":"Anemia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025142","label":"Constitutional symptom","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0011227","label":"Elevated circulating C-reactive protein concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003565","label":"Elevated erythrocyte sedimentation rate","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002729","label":"Follicular hyperplasia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030783","label":"Increased circulating interleukin 6 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100721","label":"Mediastinal lymphadenopathy","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003270","label":"Abdominal distention","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0031500","label":"Abdominal mass","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011024","label":"Abnormality of the gastrointestinal tract","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0025066","label":"Decreased mean corpuscular volume","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0030157","label":"Flank pain","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0008940","label":"Generalized lymphadenopathy","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000952","label":"Jaundice","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002017","label":"Nausea and vomiting","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012050","label":"Anasarca","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000790","label":"Hematuria","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0005214","label":"Intestinal obstruction","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0011974","label":"Myelofibrosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001723","label":"Restrictive cardiomyopathy","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0006000","label":"Ureteral obstruction","frequency":"Very rare (<4-1%)","criteria":null}]}}