Ndezvipi zvipundu izvi zviri pamutsipa nepahapwa? Ngatidzidzei nezveRosai-Dorfman Disease!

Zvakajairika kuti tinzwe kutya zvishoma patinoona shanduko mumiviri yedu, handiti? Kunyanya kana tikaona mapundu madiki pamutsipa, muhapwa, kana muzvidya, tinofunga kuti, 'Chii ichi?' Nhasi tichataura nezvechirwere chisingawanzoitiki chinogona kukonzera mapundu akadaro, asi vanhu vazhinji havasati vanzwa nezvazvo. Ichi chinonzi Rosai-Dorfman Disease.

Unoziva here kuti chirwere cheRoss-Dorffman (RDD) chii?

Zvichitaurwa zviri nyore, miviri yedu ine rudzi rwemasero machena eropa anonzi histiocytes . Aya akafanana nemapurisa ari mumiviri yedu, anorwisa utachiona hunokonzera zvirwere, uye chikamu chakakosha chemuviri wedu wekudzivirira chirwere. Zvisinei, kana ma histiocytes aya akakura zvakanyanya, anonzi chirwere cheRoss-Dorffmann (RDD). Ichi hachisi chirwere chegomarara (chisina njodzi) , zvichireva kuti hachisi chirwere chine njodzi chinopararira kubva kune imwe nhengo yemuviri kuenda kune imwe. Zvisinei, kana masero aya akaungana zvakanyanya, tinogona kusangana nekusagadzikana uye zviratidzo.

Kazhinji, ma "histiocytes" aya anoungana muma "lymph nodes" ari mumutsipa wedu. Zvadaro anozvimba semapundu ari mumutsipa. Vanachiremba dzimwe nguva vanoti kuzvimba kwema "nodes" ndiko "lymphadenopathy". Asi zvinogona kukanganisa kwete mutsipa chete, asiwo ma "nodes" ari mune dzimwe nzvimbo dzemuviri. Dzimwe nguva, pamusoro pema "nodes", masero aya anogonawo kuungana mune dzimwe nzvimbo dzemuviri (extranodal sites).

Chirwere ichi chinonziwo `sinus histiocytosis ine massive lymphadenopathy`. Ichi chirwere chisingawanzoitiki chiri muboka re `non-Langerhans cell histiocytosis`.

Ndeapi marudzi makuru echirwere cheRoss-Dorffmann (RDD)?

Chirwere ichi hachibate munhu wese zvakafanana. Vamwe vanhu vane kuzvimba panzvimbo imwe, vamwe vanogona kuzvimba munzvimbo dzakasiyana-siyana. Zvakare, ma histiocytes aya anogona kuungana kwete mumapundu chete asiwo mune dzimwe nhengo dzemuviri. Aya ndiwo maitiro aanoita.

• Chirwere cheRoss-Dorffmann (Chekumashure) (Classic/Nodal RDD): Ichi ndicho chirwere chinowanzoonekwa. Chinonyanya kusanganisira kuzvimba kwemapfundo ari mumutsipa. Zvisinei, zviratidzo zvinogona kusiyana zvichienderana nekuti mapfundo mangani abatwa.
• Extranodal RDD: Murudzi urwu, ma histiocytes aya anoungana mumatishu asiri ma lymph nodes. Ganda rinonyanya kukanganiswa. Izvi zvinonzi cutaneous RDD (CRDD) . Pamusoro pezvo, chirwere ichi chinogona kukanganisa sinuses dzedu, maziso nemaziso, mapfupa, uye central nervous system (CNS) - kureva, uropi nemusana. Dzimwe nguva kufema uye gastrointestinal system zvinogonawo kukanganiswa.

Vanhu vanosvika 40% vane RDD vanogona kunge vaine masero aya e-histiocyte akaunganidzwa mune dzimwe nhengo dzemuviri, pamusoro pemapundu, sezvamataura.

Ndiani ane mukana mukuru wekuwana chirwere ichi?

Chirwere cheRoss-Dorffmann chinonyanya kubata vana, vechidiki, nevechidiki . Chinowanzoonekwa muvanhu vane makore ekuma20. Zvisinei, vanhu vane makore ekuma70 vanonziwo vane chirwere ichi.

RDD, iyo inokanganisa machende, inowanikwa zvakanyanya muvarume vane dzinza reAfrica. Asi chirwere cheganda chataurwa kare chinonzi "CRDD" chinowanikwa zvakanyanya muvakadzi vane dzinza reAsia. "CRDD" inonyanya kuwanikwa muvanhu vane makore ari pakati pe20, 30, uye 40.

Chirwere cheRoss-Dorfman hachiwanzoitiki kangani?

Ichi chirwere chisingawanzoitiki . Chinobata munhu mumwe chete pavanhu mazana maviri ezviuru. Semuenzaniso, muUnited States, vanhu vanosvika zana vanobatwa nechirwere ichi gore rega rega. Izvi zvinoreva kuti muSri Lanka, chirwere ichi chakanyanya kuderera.

Ndezvipi zviratidzo zvechirwere cheRose-Dorfman (RDD)?

Zviratidzo zvaunoona zvinoenderana nekuti ma "histiocytes" akawandisa anoungana kupi mumuviri wako. Kana ma "lymph nodes" ari mumutsipa wako chete akakanganiswa, zviratidzo zvako zvinogona kunge zvisina kusimba kana kuti zvisipo zvachose. Zvisinei, kana masero aya akaungana uye akakanganisa mashandiro enhengo, zviratidzo zvako zvinogona kunge zvakanyanya.

Zviratidzo zvekare (zvine musoro)

MaHistiocyte anowanzo ungana mumitsipa yemutsipa. Saka, chiratidzo chinowanzoonekwa semapundu asingarwadzi, akazvimba kumativi ese emutsipa . Ungayeuka kuti dzimwe nguva unowana bundu diki mumutsipa wako kana uine dzihwa kana furuu, handiti? Ndizvozvo, asi zvingasarwadza. Zvisinei, zvichienderana nekuti nyama yakakanganiswa iri kupi, kuzvimba kunogonawo kuva mune dzimwe nzvimbo.

Nzvimbo huru dzekuzvimba dzinogona kuitika nekuda kwekuwedzera kwe histiocytes ndeidzi:

• Mutsipa wako
• Nzvimbo yegomarara
• Makwapa
• Pakati pechipfuva (mediastinum)

Ungasaona zviratidzo kunze kwekuzvimba, kana kuti ungaona zviratidzo zvakaita seizvi:

• Kupindwa nechando
• Ganda rakachenuruka
• Kuneta kwakanyanya
• Kudikitira husiku
• Mhino inoyerera
• Kuderedza uremu kusingatsananguriki

Zviratidzo zvekunze kwenododal

Chirwere cheRose-Dorffmann (`CRDD`) chinogona kukanganisa ganda uye chinogona kuoneka chero kupi zvako pamuviri. Mapundu aya, anoumbwa neboka remasero ehistiocyte, anowanzo kukura zvishoma nezvishoma. Zviratidzo zvinogona kuonekwa paganda zvinosanganisira:

• Mapundu akati sandara kana kuti akasimudzwa
• Mapundu akazara neurwa kana kuti akaomarara
• Mapundu eruvara rweyero, rwepepuru, rutsvuku, kana kuti rwebrown
• Inogona kupararira paganda rese kana kungogumira panzvimbo imwe chete.

Kana ma histiocyte aya akawandisa achikanganisa nhengo kana muviri, zviratidzo zvakasiyana-siyana zvinogona kuitika. Semuenzaniso, kana RDD ikakanganisa maziso, inogona kukonzera kuona kaviri. Kana ikakanganisa sisitimu yetsinga, inogona kukonzera pfari. Kana ikakanganisa mapapu, inogona kukonzera kukosora kusingaperi. Saizvozvowo, zviratidzo zvinosiyana zvichienderana nenzvimbo yakakanganiswa.

Chii chinokonzera chirwere cheRoss-Dorffman (RDD)?

Masayendisiti haasati aziva chikonzero chaicho chechirwere ichi. Sezvo RDD ichibata munhu mumwe nemumwe zvakasiyana, panogona kunge paine zvikonzero zvakawanda. Semuenzaniso, tsvakiridzo yazvino inoratidza kuti chirwere cheganda chinonzi "CRDD" chinokonzerwa nechimwe chinhu chisiri chimwe chete chinokonzera RDD yenguva dzose.

Vaongorori vachangobva kuona kuti mamwe majini anochinja anowanikwa mune mamwe marudzi eRDD kunze kweCRDD. Majini aya anogona kukonzera kukura kwemasero zvisingadzoreki.

Vanhu vazhinji vane RDD vane chirwere ichi pamwe chete nezvimwe zvirwere. Saka, panogona kunge paine hukama pakati pezvirwere izvozvo neRDD. Kutsvagurudza kwakawanda kunodiwa pane izvi.

RDD yakawanikwa iine chekuita nemamiriro ezvinhu anotevera:

• Utachiona hwemavhairasi: Mienzaniso inosanganisira herpes, Epstein-Barr virus, cytomegalovirus, uye hutachiona hweHIV.
• Kenza: Hodgkin lymphoma, non-Hodgkin lymphoma, uye kenza yeganda yakadai se cutaneous clear-cell sarcoma. (Asi yeuka, RDD haisi kenza.)
• Zvirwere zve autoimmune: Lupus, Juvenile Idiopathic Arthritis, Autoimmune Hemolytic Anemia.

Chirwere cheRoss-Dorffman (RDD) chinoonekwa sei?

Chiremba wako achatanga akuongorora muviri wako uye obvunza nezvezviratidzo zvako. Achatsvaga zviratidzo zveRDD, zvakaita sematumbu eganda akazvimba. Achabvunzawo nezvenhoroondo yako yehutano kuti aone kana wakambova kana kuti wakambova nematambudziko ane chekuita neRDD.

Uye zvakare, chiremba anogonawo kuita bvunzo idzi:

• Maitiro ekutora mifananidzo: Zvichienderana nerudzi rwenyama inofungidzirwa kuti ine histiocytes, chiremba anogona kuraira kuti apihwe X-ray, ultrasound, MRI, CT, PET, PET/CT, kana bone scan. Izvi zvinogona kushandiswa kuona zviri mukati memuviri.
• Kuongororwa kweropa: Kuongororwa kweropa kwakawanda, kwakadai sekuverengwa kweropa kwakazara (CBC) uye panel yakazara yemetabolism, kunogona kuitwa kuti uone shanduko dziri kuitika mumuviri.
• Biopsy: Iyi bvunzo inokosha zvikuru.Izvi zvinosanganisira kutora sampuli diki yenyama yakakanganiswa (semuenzaniso, lymph node yakazvimba) uye kuongorora masero pasi pemaikorosikopu kuti vaone kana aine hunhu hweRDD. Kuongororwa uku kunogonawo kuona kana chimwe chirwere chiri kukonzera kukura kwemasero kusina kujairika.

Chirwere cheRoss-Dorffmann (RDD) chinorapwa sei?

Dzimwe nguva, RDD inogona kupora pasina kurapwa. Izvi zvinoreva kuti chirwere ichi chichapora chega. Zvisinei, nguva yachinotora yakaoma kufanotaura. Zvinogona kutora mwedzi kana makore kuti chive nani. Zvisinei, mune dzimwe nguva, chingasapera chega, kana kuti chingadzoka. Kana chikasarapwa, chirwere ichi chingawedzera kuipa.

Kurapwa kwauchaitwa kunoenderana nekuti RDD yakakukanganisa sei.

• Kucherechedza: Kana usina zviratidzo zviri kukanganisa hupenyu hwako zvakanyanya, chiremba wako anogona kusarudza kutarisa mamiriro ako ezvinhu. Izvi zvakafanana nenzira yekumirira kwenguva refu.
• Kuvhiyiwa: Mapundu anogona kubviswa nekuvhiyiwa. Kana uine `CRDD` (cutaneous RDD), kana mapundu acho ari kuvhara nzira yekufema kana kukanganisa musana wako, kuvhiyiwa kunogona kuitwa.
• Kurapa nemwaranzi: Kana kuvhiyiwa kusingakwanise kubvisa ma histiocytes, kurapwa nemwaranzi kunogona kupiwa. Izvi zvinosanganisira kushandisa muchina kunangisa mwenje wemwaranzi pamasero, uchiaparadza.
• Kurapa nemakemikari: Kana chirwere chacho chakakomba, kana kuti zvimwe zvirwere zvakaita sekuvhiyiwa, zvisina kuderedza zviratidzo, chemotherapy inogona kufungwa nezvayo. Izvi zvinosanganisira kushandisa mishonga inouraya masero ari kukura nekukurumidza, akadai semasero ekenza.
• Corticosteroids: Corticosteroids, yakadai seprednisone, inogona kuderedza kuzvimba kwemajoini uye kuderedza zviratidzo. Iyi imhando yemishonga inoderedza kuzvimba.
• Kurapa Nema "Immunotherapy": Kurapa uku kunobatsira masoja emuviri wako kuwana nekuparadza masero e "histiocyte" akawandisa zvinobudirira.

Ramangwana remunhu ane chirwere cheRoss-Dorffman (RDD) rakaita sei?

Kufanotaura kwako kunoenderana nezvinhu zvakati wandei. Huwandu hwemasero akanganiswa, kuti ma histiocytes akawanda aripi mumuviri wako, uye kuti unopindura zvakanaka sei pakurapwa. Kazhinji, RDD inopera yega. Zvisinei, mune dzimwe nguva, kurapwa kunodiwa, kana kuti matambudziko akakomba anogona kuitika.

Kazhinji, kana masero akabatwa ari mashoma, mhedzisiro yacho inowedzera.Kana paine extranodal RDD, iyo inokanganisa ganda, chipfuva, kana nzira yekufema yepamusoro, mhedzisiro yacho inogona kuva yakanaka. Zvisinei, kana ma histiocytes ari munzira yekufema yepasi, itsvo, kana chiropa, mamiriro acho anogona kunge akaipa zvishoma.

Mibvunzo ipi yandinofanira kubvunza chiremba wangu?

Usakanganwa kutaura nachiremba wako nezvezvinhu izvi, nekuti zvakakosha kuti unzwisise mamiriro ako ezvinhu zvakanaka:

• "Chiremba, ndedzipi nhengo dzemuviri wangu dzakakanganiswa nechirwere changu cheRDD?"
• "Ndichafanira here kuedzwa mifananidzo (senge scans) kana kuongororwa ropa?"
• "Ndinoda kurapwa here, kana kuti zvinokwanisika here kuti dambudziko rangu reRDD rigadzirike roga?"
• "Ndinofanira kunetseka zvakadini nezvenyaya iyi yeRDD?"
• "Ndeipi nzira yakanakisisa yekurapa yandinorayirwa nachiremba?"
• "Ndeapi mamwe matambudziko andingatarisira mushure mekurapwa?"
• "Ndeipi mikana yekuti chirwere changu cheRDD chichadzoka mushure mekurapwa?"
• "Ndinofanira kuuya kuzoongororwa kangani kuti ndione mamiriro angu ezvinhu?"

Chirwere cheRoss-Dorffman (RDD) ikenza here?

Kwete. Uyu mubvunzo unobvunzwa nevanhu vazhinji. Chirwere cheRose-Dorffman hachisi kenza (isina kuipa) . Kureva kuti, hachipararire kubva kune imwe nhengo yemuviri kuenda kune imwe sekenza (kenza). Zvisinei, mapundu anoumbwa nekuda kweRDD anogona kukanganisa nhengo dzemuviri, kukanganisa mashandiro adzo, uye kukonzera matambudziko. Chiremba wako ndiye munhu akakodzera kuziva kuti chirwere chako cheRDD chiri kukanganisa sei muviri wako uye kana uchifanira kunetseka nacho.

Ndiani akawana chirwere cheRoss-Dorffman (RDD)?

Zvakanaka kuziva izvi zvakare. Muna 1965, musayendisiti ainzi Pierre Paul Louis Lucien Destombes akawana zviratidzo zveRDD muvarwere vana. Zvadaro, muna 1969, masayendisiti maviri ainzi Juan Rosai naRonald Dorfman vakaongorora rimwe boka revarwere vane zviratidzo zvakafanana. Chirwere ichi ikozvino chinozivikanwa sechirwere cheRosai-Dorfman, vachibatanidza mazita avo. Kashoma, chinonziwo chirwere cheRosai-Dorfman-Destombes, vachikudza masayendisiti ese matatu.

Pakupedzisira, zvinhu zvekurangarira (Mharidzo Yekuenda Kumba)

Chirwere cheRoss-Dorffmann (RDD) chinogona kuva chirwere chinonetsa nekuti hachiwanzoitiki uye chinobata munhu mumwe nemumwe zvakasiyana . Hapana gwara rimwe chete rekurapa RDD. Ndosaka zvichikosha kutaura pachena nachiremba wako, kunzwisisa chirwere chako, uye kuziva nzira dzekurapa dzaungasarudza.

Ungave uine RDD isina kusimba iyo inozopera yoga. Kana kuti, ungangoda mishonga yakasiyana-siyana. Bvunza chiremba wako kuti hurongwa hwako hwekurapa uye mhedzisiro inotarisirwa zvichagadziriswa sei zvinoenderana nemamiriro ako ezvinhu.

Yeuka kuti hausi wega . Paunenge uchitarisana nechirwere chakadai chisingawanzoitiki, zvakakosha kutevera mazano echiremba uye kuramba wakasimba mupfungwa. Kana uine mibvunzo kana kusava nechokwadi, kurukura nezvayo nachiremba wako. Usatya, nekuti kuziva ndicho chombo chakanakisa chekurwisa zvinhu zvakadaro.

` Rosai-Dorfman Disease, RDD, histiocytes, lymphadenopathy, mapundu emutsipa, zvirwere zveganda, CRDD, zvirwere zvisingawanzoitiki, immune system