{"orpha":542,"name":"Primary cutaneous lymphoma","type":"Category","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"A group of rare primary organ-specific lymphoma characterized by heterogeneous T-cell and B-cell non-Hodgkin lymphomas present in the skin. Clinical and pathological features, evolutive profile, prognosis, molecular etiology and response to therapy are highly variable between the entities belong to this group.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-542/","orphanet_url":"https://www.orpha.net/en/disease/detail/542","names":{"fr":{"name":"Lymphome cutané primitif","definition":"Groupe de lymphomes primitifs rares spécifiques à un organe, caractérisés par des lymphomes non hodgkiniens hétérogènes à lymphocytes T et B cutanés. Les caractéristiques cliniques et pathologiques, le profil évolutif, le pronostic, l'étiologie moléculaire et la réponse au traitement varient fortement suivant les entités appartenant à ce groupe."},"de":{"name":"Lymphom, kutanes primäres","definition":"Eine Gruppe seltener primärer organspezifischer Lymphome, die durch heterogene T-Zell- und B-Zell-Non-Hodgkin-Lymphome in der Haut gekennzeichnet sind. Klinische und pathologische Merkmale, Entwicklungsprofil, Prognose, molekulare Ätiologie und Ansprechen auf die Therapie sind zwischen den Entitäten, die zu dieser Gruppe gehören, sehr unterschiedlich."},"es":{"name":"Linfoma cutáneo primario","definition":"Es un grupo poco frecuente de linfomas primarios órgano-específicos caracterizados por linfomas no-Hodgkin heterogéneos de células T y B presentes en la piel. Las características clínicas y patológicas, el perfil evolutivo, el pronóstico, la etiología molecular y la respuesta al tratamiento son muy variables entre las entidades pertenecientes a este grupo."},"it":{"name":"Linfoma cutaneo primitivo"},"nl":{"name":"Primair cutaan lymfoom","definition":"Een groep van zeldzame primaire orgaanspecifieke lymfomen, gekenmerkt door aanwezigheid in de huid van heterogene T-cel en B-cel non-Hodgkin-lymfomen. Klinische en pathologische kenmerken, evolutie, prognose, moleculaire etiologie en respons op therapie zijn hoogst variabel tussen de entiteiten die tot deze groep behoren."},"pt":{"name":"Linfoma cutâneo primário","definition":"Um grupo raro de linfomas primários específicos de órgão, caracterizados por linfomas não-Hodgkin heterogéneos de células T e B presentes na pele. As características clínicas e patológicas, o perfil evolutivo, o prognóstico, a etiologia molecular e a resposta à terapêutica são altamente variáveis ​​entre as entidades clínicas pertencentes a este grupo."},"pl":{"name":"Pierwotny chłoniak skóry","definition":"Grupa rzadkich pierwotnych chłoniaków narządowo-specyficznych, które charakteryzują się występowaniem heterogennych chłoniaków nieziarniczych z komórek T i B występujących w skórze. Cechy kliniczne i patologiczne, profil ewolucyjny, rokowanie, podłoże molekularne i odpowiedź na terapię są bardzo zmienne dla poszczególnych jednostek należących do tej grupy."},"cs":{"name":"Primární kožní lymfom"},"uk":{"name":"Первинна шкірна лімфома","definition":"Cutaneous lymphoma is a heterogeneous entity with respect to its clinical and pathological features, evolutive profile, prognosis, molecular aetiology and response to therapy. These specifications have been taken into account in recent classifications, which have placed particular importance on the prognostic implications of these different entities."},"zh":{"name":"原发性皮肤淋巴瘤"},"tr":{"name":"Primer kutanöz lenfoma"}},"xrefs":[{"source":"MONDO","code":"0018898","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18898","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C1302772","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10051708","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":79386,"name":"Rare skin tumor or hamartoma","classifications":[187,202]}],"children":[{"orpha":178563,"name":"Primary cutaneous B-cell lymphoma","type":"Category"},{"orpha":171901,"name":"Primary cutaneous T-cell lymphoma","type":"Category"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":[],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 1 000 000","value":0.75,"area":"Europe","status":"Not yet validated","source":"15692063[PMID]_16192622[PMID]_ ORPHANET"},{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 100 000","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}