Erdheim chirwere - chii munhu zvokurwara?

Pasinei chokwadi kuti mushonga kukura nokukurumidza, zvirwere zvakawanda havana kudzidza kusvikira pakuguma. Vamwe vavo vakanga kurondedzerwa kudaro kare. Saka, mashoko ari mamwe pathologies Panguva iyi haisi kukwana. Muenzaniso ndiye Erdheim chirwere. Kutambudzika yakazarukira chete XX remakore. Ndiye isingawanzowanikwi chirwere, saka hapana mukana kuti mberi kuongorora chirwere ichi zvakadzama. Zvinokonzera pathogenesis kuti zvinetso asingazivikanwi pakuguma. Kunyange zvakadaro, pane zvakawanda dzidziso chirwere. Vose nokushingaira vari kudzidza. To musi, 500 chete anozivikanwa zviitiko chirwere pasi pose. Sezvo zvokurwara anonzi zvisingawanzoitiki, hazvisi nyore kuziva nguva dzose.

Chii chirwere Erdheim?

Kokutanga pamusoro chirwere ichi, yakasvika pakuzivikanwa muna 1930. Chakazarurwa masayendisiti William Chester wake mudzidzisi - Jacob Erdheim. Vakashanda pamwe chete nokudzidza chirwere ichi. Saka chirwere unowanzonzi chirwere Erdheim Chester. Maererano data, izvo akakwanisa kuunganidza pamusoro kwemakore, zvokurwara zvikuru nevakawanda nevanhu. Kazhinji, chirwere kutanga anoratidza pacharo mu makore 50. Kunyange zvakadaro, pane zvakawanda zviitiko hwevanhu vanorwara pakati pavana. Zviratidzo hosha zvinosiyana. Kakawanda, zvinotevera zvinoratidza zvekuchipatara kuitika: mapfupa chirwere, chetsinga zvirwere uye chirwere cheshuga insipidus. Chirwere (chirwere) Erdheim-Chester hwokuratidza infiltration zvakasiyana siyana nyama dzomuviri nelangergansovymi histiocytes. Masero aya ndezvaJehovha zvinoumba dziviriro yezvirwere mumuviri. Kazhinji, vane unodzivirira basa. Asi pane unmotivated kubereka histiocytes, somugumisiro Dzinotibatsira siyana nhengo zvokurwara ichi.

Erdheim chirwere: chirwere zviratidzo

The kunotoda mufananidzo uyu isingawanzowanikwi chirwere angava akasiyana. It zvinoenderana iyo nhengo vakakundwapo ne histiocytes. Munenge zvose Muzviitiko Erdheim chirwere kwakaratidzwa nokuchinja mune marangwanda, tsinga uye ganda. Pakati zviratidzo chirwere ichi chinogona oga kutyorwa zvinotevera:

• Osteosclerosis periosteum. kuratidzwa Izvi aripo vakawanda varwere. Kunyange zvakadaro, kazhinji, chiratidzo ichi hazvirevi kunokanganisa varwere. Chete chikamu vanhu vanotambura nezvirwere ichi anotsutsumwa kurwadziwa mune nzvimbo lesion.
• Exophthalmos. chirwere ichi zviratidzo kuva nemhaka pokukundwa nzvimbo yaiva shure yeziso. Uyewo kuwedzera histiocytes kunogona kukonzera compression pamusoro optic etsinga uye tsandanyama. Saka, mune dzimwe varwere pane chiratidzo sezvo diplopia. Vamwe varwere yakashuma vasanyanye kutarisana acuity.
• Histiocytic infiltration of kwemakemikari anobudiswa nhengo. Kuratidzwa kuri kukura chirwere cheshuga insipidus (nyota, polyuria), metabolic nyonganyonga.
• Hydro uye uteronefroz. Zviratidzo izvi vanova somugumisiro compression nokuda histiocytic zvinyama etsvo uye ureters.
• The kukundwa kumwoyo uye nemapapu.
• Xanthelasma (mafuta) pamusoro namafungiro uye xanthoma. Neoplasms zvinogona iri yose muviri.

Kuongorora chirwere Erdheim urwere

Fungira Erdheim-Chester chirwere kwakaoma nokuda kwayo isingawanzowanikwi kuwanika uye vakawanda kuratidzwa kuti anogona kuitika nevamwe pathologies. Kakawanda, vanofanira kuteerera mubatanidzwa zviratidzo zvakadai proptosis uye kurwadziwa mapfupa, uyewo zvishoma nezvishoma nenyota uye polyuria. Foci kuti infiltration anogona kuonekwa nevanhu maekisirei nhengo dzake, dehenya. Uyewo, kana hosha yacho vakafunga kuti biopsy rinoitwa mitumbi kuti vari kusangana mwero infiltration. Uyezve, vaiita fundus ongororo, CT uye MRI of retroperitoneal nzvimbo. Kana paine rinoitwa munhu biopsy cutaneous kuratidzwa pathological nzvimbo (xanthomas). Anogona kusimbisa chirwere ane majini kuzviongorora.

Nzira kurapa chirwere Erdheim

Chokutanga mushonga kunofanira pakarepo pashure chirwere: Erdheim chirwere. Kurapa chirwere ichi panguva ipfupi kuti varwere "interferon". medicament dose kunobva kukura foci kuti infiltration. Sezvo ichangobva kuiswa zvinodhaka "vemurafenib", inova inhibitor pamusoro mutant protooncogene. Uyezve, zvichienderana kurapwa, symptomatic kurapa. Chirwere fungidziro kunotsamira ayo mwero kuyerera zvakavapo. Exophthalmos uye kukundwa ari kufema ino vanoonekwa zvisingafadzi. zviratidzo.