{"orpha":450322,"name":"Polyclonal hyperviscosity syndrome","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare hematologic disease characterized by high serum viscosity due to polyclonal expansion of immunoglobulins, most commonly in the context of Waldenström's macroglobulinemia, as well as a variety of disorders of immune dysregulation. Patients present with signs and symptoms involving multiple organs, such as bleeding diathesis, mucosal bleeding, retinal hemorrhage, headache, stroke, pulmonary hypertension, and congestive heart failure.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-450322/","orphanet_url":"https://www.orpha.net/en/disease/detail/450322","names":{"fr":{"name":"Syndrome d'hyperviscosité polyclonale","definition":"Maladie hématologique rare caractérisée par une viscosité sérique élevée due à l'expansion polyclonale des immunoglobulines, le plus souvent dans le cadre d'une macroglobulinémie de Waldenström, ainsi que de diverses maladies du dérèglement immunitaire. Les patients présentent des signes et des symptômes en lien avec l'atteinte de plusieurs organes, notamment, la diathèse hémorragique, un saignement des muqueuses, une hémorragie rétinienne, des céphalées, un accident vasculaire cérébral, une hypertension pulmonaire et une insuffisance cardiaque congestive."},"de":{"name":"Polyklonales Hyperviskositätssyndrom","definition":"Eine seltene hämatologische Erkrankung, die durch eine hohe Serumviskosität aufgrund einer polyklonalen Vermehrung von Immunglobulinen, am häufigsten im Zusammenhang mit der Waldenström-Makroglobulinämie, und einer Vielzahl von Störungen der Immunregulation gekennzeichnet ist. Die Patienten zeigen Anzeichen und Symptome, die mehrere Organe betreffen, wie Blutungsdiathese, Schleimhautblutungen, Netzhautblutungen, Kopfschmerzen, Schlaganfall, pulmonale Hypertonie und Herzinsuffizienz."},"es":{"name":"Síndrome de hiperviscosidad policlonal","definition":"Es una enfermedad hematológica poco frecuente caracterizada por una viscosidad sérica elevada debido a la expansión policlonal de inmunoglobulinas, más comúnmente en el contexto de la macroglobulinemia de Waldenström, así como en diversos trastornos de disregulación inmunológica. Los pacientes se presentan con signos y síntomas que afectan a múltiples órganos, tales como diátesis hemorrágica, sangrado de las mucosas, hemorragia retiniana, cefaleas, accidente cerebrovascular, hipertensión pulmonar e insuficiencia cardíaca congestiva."},"it":{"name":"Sindrome da iperviscosità policlonale","definition":"Si tratta di una malattia ematologica rara, caratterizzata da un'elevata viscosità del siero causata dall'espansione policlonale delle immunoglobuline, di solito nel quadro della macroglobulinemia di Waldenström, e da altre anomalie della regolazione immunitaria. I pazienti presentano segni clinici che interessano vari organi, come la diatesi emorragica, l'emorragia delle mucose e della retina, la cefalea, l'ictus, l'ipertensione polmonare e l'insufficienza cardiaca congestizia."},"nl":{"name":"Polyklonale hyperviscositeitssyndroom","definition":"Een zeldzame hematologische aandoening, gekarakteriseerd door serum met hoge viscositeit als gevolg van polyklonale expansie van immunoglobulinen, doorgaans in de context van macroglobulinemie van Waldenström, alsook allerlei aandoeningen met immuundisregulatie. Patiënten presenteren zich met verschijnselen en symptomen met betrokkenheid van meerdere organen, zoals bloedingsneiging, mucosale bloeding, retinale hemorragie, hoofdpijn, beroerte, pulmonale hypertensie, en congestief hartfalen."},"pt":{"name":"Síndrome de hiperviscosidade policlonal"},"pl":{"name":"Zespół nadlepkości poliklonalnej"},"cs":{"name":"Polyklonální hyperviskózní syndrom"},"uk":{"name":"Синдром поліклональної гіпервязкості","definition":"A rare hematologic disease characterized by high serum viscosity due to polyclonal expansion of immunoglobulins, most commonly in the context of Waldenström's macroglobulinemia, as well as a variety of disorders of immune dysregulation. Patients present with signs and symptoms involving multiple organs, such as bleeding diathesis, mucosal bleeding, retinal hemorrhage, headache, stroke, pulmonary hypertension, and congestive heart failure."},"zh":{"name":"多克隆高粘度综合征"},"tr":{"name":"Poliklonal hiperviskozite sendromu"}},"xrefs":[{"source":"ICD-10","code":"D89.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0018678","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5681102","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97992,"name":"Rare hematologic disease"},"parents":[{"orpha":97992,"name":"Rare hematologic disease","classifications":[194]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adolescent","Adult","All ages","Elderly"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Worldwide","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}