Zvaunofanira kuziva nezveCastleman Disease

Wakambova nebundu here, ratinoti "bundu," pamutsipa wako, muhapwa, kana pahudyu? Dzimwe nguva, mapundu akadai anozvimba kana uine dzihwa kana chimwe chirwere. Izvozvo zvakajairika. Asi kana uine mapundu akazvimba seizvi pasina chikonzero, kwenguva yakareba, uye uchinzwawo kuneta uye kudzikira uremu, unofanira kunetseka zvishoma. Nhasi, tichataura nezvechirwere chisingawanzoitiki chinogona kufungidzirwa panguva dzakadaro. Icho chirwere cheCastleman.

Zvichitaurwa zviri nyore, chii chinonzi Castleman Disease?

Fungidzira kuti muviri wedu wakaita senyika. Saka masoja edu ekudzivirira muviri ndiwo anodzivirira nyika iyoyo. Kana utachiona (muvengi) hukapinda mumuviri huchibva kunze, uto iri rinoenda kuhondo rokunda muvengi iyeye. Kana hondo yapera, uto rinodzokera kukamba yemauto robva ranyarara. Izvi ndizvo zvinoitika mumuviri wemunhu ane hutano.

Asi kune munhu ane chirwere cheCastleman, zvakasiyana. Chinoitika pano ndechekuti mauto akaenda kuhondo, masoja ekudzivirira muviri, haanyarari kunyangwe hondo yapera. Anoramba achishanda. Zvinoita sekunge anogara akagadzirira hondo. Izvi zvinokonzera kuzvimba kwenguva refu mumuviri. Izvi zvinogona kukuvadza nhengo dzedu.

Panguva iyi, ma lymph nodes edu, zvinhu zvidiki izvozvo, zvakaita semasoja. Anoita basa rakawanda, sekubvisa utachiona nekuchengetedza masero ekudzivirira muviri. Iye zvino, nekuti immune system iri kushanda nguva dzose, ma lymph nodes aya ari kushandawo nguva dzose. Nekuda kweizvozvo, masero ari maari anotanga kukura zvakanyanya. Ndosaka ma lymph nodes achikura muchirwere cheCastleman. Hakusi kuzvimba chete, asi kunokonzera shanduko munyama yema lymph nodes iwayo.

Ndeapi marudzi makuru echirwere cheCastleman?

Chirwere cheCasselman chakakamurwa kuita mhando mbiri huru. Zviratidzo nekurapwa kwechimwe nechimwe zvakasiyana zvikuru. Saka, zvakakosha kunzwisisa mhando mbiri idzi nemazvo.

Pane mhando dzechirwere cheCastleman chinonzi multicentric disease (MCD) here?

Ehe, MCD yakakamurwazve kuita mapoka akasiyana-siyana. Kupatsanurwa uku kunoitwa zvichibva pane chinokonzera uye zvimwe zvirwere zvinoitika nayo.

• MCD inobatanidzwa neNhetembo: Nhetembo chirwere cheropa chisingawanzoitiki. Dzimwe nguva MCD inogona kuitika pamwe chete neNhetembo.
• HHV-8-associated MCD: Izvi zvinokonzerwa nehutachiona hunonzi Human Herpes Virus-8 (HHV-8). Rudzi urwu runonyanya kuwanikwa muvanhu vane HIV kana kuti vane dziviriro yemuviri inovakanganisa nekuda kwezvimwe zvikonzero.
• Idiopathic MCD (iMCD): Izwi rezvekurapa rekuti " idiopathic " rinoreva kuti "hapana chikonzero chakawanikwa." Ruzhinji rwevarwere veMCD vane rudzi urwu. Izvi zvinoreva kuti chikonzero chaicho hachisati chazivikanwa.

Rudzi urwu rwe iMCD rwakakamurwazve kuita mapoka matatu madiki:

1. iMCD ine chekuita neTAFRO: TAFRO izita rinobva mukubatanidzwa kwezviratidzo zvakasiyana-siyana. Kureva kuti, Thrombocytopenia (maplatelet eropa mashoma), Anasarca (kuzvimba kwemuviri nemvura), Fivha (fivha), Kusashanda zvakanaka kweitsvo (kusashanda zvakanaka kweitsvo) uye Organomegaly (kukura kwechiropa kana spleen) ndizvo zviratidzo zvinogona kuonekwa mune izvi.

2. iMCD ine idiopathic plasmacytic lymphadenopathy (iMCD-IPL): Murudzi urwu, huwandu hwemaplatelet hunogona kukwira. Panogonawo kuve nekuwanda kwemasero machena eropa anogadzira ma antibodies.

3. iMCD, isina kutaurwa neimwe nzira (iMCD-NOS): Varwere vane iMCD vasina hukama neTAFRO uye vasina chimwe chikonzero chaicho chinowanikwa vanowira muchikamu ichi.

Ndezvipi zviratidzo zvechirwere ichi?

Zviratidzo zvinosiyana zvichienderana nerudzi rwechirwere cheCastleman chaunacho.

Chirwere cheCasselman chinowanzova chisina zviratidzo . Chiratidzo chega i lymph node yakazvimba (adenoid). Dzimwe nguva, zviratidzo (semuenzaniso, kurwadziwa, kufema zvishoma) zvinongoitika chete kana dumbu rakazvimba rakadzvanya pane imwe nhengo.

Zvisinei, muchirwere cheCastleman chinonzi multicentric (MCD), zviratidzo zvacho zvinonyanya kuoneka. Pamusoro pekuzvimba kwemagland, unogona kusangana ne:

• Fivha inowanzo itika
• Kunzwa kuneta zvakanyanya uye kuneta pasina chikonzero (izvi zvinogonawo kunge zvichikonzerwa nekushaya ropa)
• Kudikitira zvakanyanya usiku
• Kusvotwa nekurutsa
• Kuderedza uremu kusingatarisirwi
• Kuzvimba kwemakumbo, zvitsitsinho, kana dumbu
• Chiropa chakakura (splenomegaly) kana chiropa chakakura (hepatomegaly)
• Kusanzwa zvakanaka mumaoko nemakumbo (peripheral neuropathy)

Sei chirwere ichi chichiitika? Pane zvinhu zvinogona kukonzera njodzi here?

Kutaura zvazviri, masayendisiti haasati awana chikonzero chaicho chechirwere cheCastleman (UCD) chimwe chete .

Chirwere cheMulticentric Castleman (MCD) chakabatana nehutachiona hweHHV-8 hwambotaurwa. Zvisinei, vaongorori vachiri kuongorora kana zvimwe hutachiona, kushanduka kwemajini, kana maitiro ekudzivirira muviri zvingave zviri izvo zvinokonzera rudzi rusingazivikanwe rwe iMCD.

Kana zvasvika pazvinhu zvine njodzi, hapana zvinhu zvinozivikanwa zvinogona kukonzera UCD ne iMCD. Zvisinei, kana uine hutachiona hweHIV kana imwe immune system isina simba, uri panjodzi huru yekubatwa neHHV-8-associated MCD.

Kunyangwe chirwere ichi chichigona kuitika muvanhu vemazera ese, chinonyanya kuitika muvanhu vane makore ari pakati pe30 ne60 .

Ndezvipi zvinetso zvinogona kukonzera chirwere ichi?

Chirwere cheCastleman chinogona kuwedzera njodzi yako yekubatwa nezvimwe zvirwere, kunyanya kenza , dzakadai se lymphoma, kenza ye lymphatic system.

Pamusoro pezvo, vanhu vane UCD vari panjodzi yekubatwa nechirwere cheganda chisingawanzoitiki asi chakakomba chinonzi paraneoplastic pemphigus (PNP). Vanhu vane MCD vari panjodzi yakakura yekubatwa nezvirwere zvakasiyana-siyana. Kana zvikasarapwa, hutachiona uhu hunogona kukonzera kukuvara kwenhengo dzemuviri uye kutouraya.

Asi musazvidya mwoyo, chiremba wenyu acharamba achitarisa dambudziko iri uye achitora matanho anodiwa kudzivirira matambudziko aya zvakanyanya sezvinobvira.

Chiremba anonyatsoongorora sei chirwere ichi?

Nekuti zviratidzo zvechirwere cheCastleman zvakafanana nezvezvimwe zvirwere, zvakaita sedzihwa kana furuu, chiremba anotanga aongorora zvirwere zvakajairika. Ndipo chete pavanozofungidzira chirwere cheCastleman uye voita bvunzo dzakati.

Miedzo inotevera inonyanya kuitwa:

• Kuongororwa kwerabhoritari: Ropa rako richaongororwa uchishandisa maikorosikopu kuti rione kana paine kusarongeka muhuwandu hwemasero eropa rako nezvimwe zvirwere. Kana paine kufungidzirwa kuti HHV-8-associated MCD, kuongororwa kweHIV kunogona kuitwawo.
• Kuongororwa kwemifananidzo: Kuongororwa kweCT nePET kunogona kubatsira kuona kuti ma lymph nodes akazvimba ari kupi mumuviri. Vanogonawo kutarisa kana nhengo dzemuviri dzakadai sechiropa nespleen dzakakura.
• Kuongororwa kwemasero eropa: Iyi ndiyo nzira chete uye inonyanya kujeka yekusimbisa chirwere . Izvi zvinosanganisira kutora chidimbu chidiki chenyama kubva pamasero eropa akazvimba wochiongorora uchishandisa maikorosikopu. Izvi zvinozobva zvaratidza kana paine shanduko dzakati wandei mumasero dzine chekuita nechirwere cheCasselman.

Ndezvipi zvinorapwa izvi?

Nzira dzekurapa dzinosiyana zvachose zvichienderana nerudzi rwechirwere.

Kurapwa kweUnicentric Casselman Disease (UCD)

Kurapa kukuru uye kunobudirira zvikuru kweUCD kubviswa kwe lymph node yakakanganiswa . Kazhinji, chirwere chinorapwa zvachose mushure mekuvhiyiwa uku.

Dzimwe nguva, kana bundu rakakura, kurapwa nemwaranzi kana kuti immunotherapy kunopihwa vasati vavhiyiwa kuti ritetepe uye zvive nyore kubvisa.

Kana bundu riri panzvimbo isingabviswi nekuvhiyiwa, uye iwe usina zviratidzo, chiremba wako anogona kusarudza kusarirapa asi kungoritarisa. Zvisinei, kune mumwe munhu asiri kuda kuvhiyiwa asi achiri nezviratidzo, kurapwa kwakafanana nekweMCD kunogona kupihwa.

Kurapwa kwechirwere cheCasselman chine mativi akawanda (MCD)

Nekuti MCD chirwere chinopararira muzvikamu zvakawanda zvemuviri, hachigone kurapwa nekuvhiyiwa kana kurapwa nemwaranzi. Saka, mamwe marapirwo anoshandiswa. Kurapa kunoenderana nekuti une HHV-8 here uye kuti chirwere chacho chakakomba sei.

Mishonga inotevera inonyanya kupihwa:

• Corticosteroids: Mishonga iyi inobatsira kudzora zviratidzo nekuderedza kuzvimba mumuviri.
• Mishonga yekurapa nemakemikari: Iyi inoshanda nekudzora mwero wekukura kwemasero ari mu lymphatic system nekukurumidza. Rituximab inowanzoshandiswa pakurapa HHV-8-associated MCD.
• Kurapa Nezvirwere Zvinodzivirira Muviri: Kurapa uku kunoshanda nekudzikamisa masoja emuviri wako anodzivirira muviri zvakanyanya. Siltuximab (Sylvant®) ndiyo yega mushonga unotenderwa kushandiswa ne iMCD.
• Mishonga inorwisa mavhairasi: Kana uine HHV-8-associated MCD, unogona kupihwa mishonga iyi kudzora hutachiona hweHIV kana HHV-8.

Ndinofanira kuona chiremba nguvai?

Kana ukaona bundu kana kuti dumbu riri pamutsipa wako, muhapwa, kana pahudyu raunogona kunzwa, iva nechokwadi chekuudza chiremba wako nezvazvo.

Uyewo, kana zviratidzo zvataurwa munyaya ino, zvakaita sefivha isina kutsanangurwa, kudzikira kwehuremu, uye kuneta zvakanyanya, zvikaramba zviripo kwemavhiki akati wandei, iva nechokwadi chekutsvaga rubatsiro rwechiremba.

Zvinokwanisika here kurarama nechirwere ichi?

Mhinduro yemubvunzo uyu inoenderana nerudzi rweCasselman yaunayo.

Munhu ane UCD anogona kutarisira mhedzisiro yakanaka kwazvo . Kana bundu rikabviswa nekuvhiyiwa, chirwere ichi chinowanzo pora zvachose. Mushure mekurapwa, izvi hazvizokanganisi hupenyu hwako hwemazuva ese.

Kufanotaura kwevanhu vane MCD kwakaoma zvishoma . Zvinosiyana zvichienderana nerudzi uye kuoma kwechirwere. Kune vamwe, zviratidzo zvinogona kungoerekana zvanyanya uye zvichiisa upenyu panjodzi. Kune vamwe, zviratidzo zvinogona kugara kwenguva yakareba, asi pane tariro. Pakati pe65% ne75% yevanhu vanoonekwa vaine MCD vanorarama kwemakore anopfuura mashanu . Izvi zviri kuvandudzika nekurapwa kutsva.

Hapana mhinduro iri nyore, inokwana zvese kuchirwere cheCasselman. Zvinoenderana neruzivo rwako, rudzi rwechirwere chaunacho, uye kana uine zvimwe zvirwere, zvakaita seHIV, TAFRO, kana POEMS. Dzimwe nguva kuvhiyiwa uye kugara uchiongororwa nachiremba zvinenge zvakakwana. Kana kuti ungangoda kurapwa kakawanda muhupenyu hwako hwese. Saka zvakanaka kutaura pachena nachiremba wako nezvechirwere chako, kurapwa kwako, uye remangwana rako.