Ziyini lezi ziqhubu entanyeni nasemakhwapheni? Ake sifunde ngesifo iRosai-Dorfman!

Kuvamile ukuzizwa wesaba kancane uma sibona izinguquko emizimbeni yethu, akunjalo? Ikakhulukazi uma sibona amaqhubu amancane entanyeni yethu, emakhwapheni, noma emisipheni, sicabanga ukuthi, 'Kuyini lokhu?' Namuhla sizokhuluma ngesimo esingavamile esingabangela amaqhubu anjalo, kodwa abantu abaningi abake bezwa ngaso. Lokhu kubizwa ngokuthi i-Rosai-Dorfman Disease.

Uyazi ukuthi siyini isifo sikaRoss-Dorffman (RDD)?

Kalula nje, imizimba yethu inohlobo lwamaseli amhlophe egazi abizwa ngokuthi ama-histiocytes . Lawa afana namaphoyisa emizimbeni yethu, alwa namagciwane abangela izifo, futhi ayingxenye ebalulekile yesimiso sethu somzimba sokuzivikela. Kodwa-ke, lapho la ma-histiocytes ekhula kakhulu, abizwa ngokuthi isifo sikaRoss-Dorffmann (RDD). Lokhu akuyona ngempela umdlavuza (ongeyingozi) , okusho ukuthi akusona isifo esiyingozi esisakazeka sisuka kwenye ingxenye yomzimba siye kwenye. Kodwa-ke, lapho la maseli eqoqana ngokweqile, singaba nokungakhululeki kanye nezimpawu.

Esikhathini esiningi, lawa ma-histiocyte amaningi aqoqana kuma-lymph node entanyeni yethu. Bese evuvukala njengezigaxa entanyeni. Odokotela ngezinye izikhathi babiza lokhu ngokuthi ukuvuvukala kwama-node `(lymphadenopathy).` Kodwa kungathinta hhayi intamo kuphela, kodwa nama-node kwezinye izingxenye zomzimba. Ngezinye izikhathi, ngaphezu kwama-node, lawa maseli angaqoqana nakwezinye izingxenye zomzimba (izindawo zangaphandle kwama-node).

Lesi sifo sibizwa nangokuthi 'i-sinus histiocytosis ene-massive lymphadenopathy'. Lesi yisifo esingavamile esingeseqembu elithi 'i-non-Langerhans cell histiocytosis'.

Yiziphi izinhlobo eziyinhloko zesifo iRoss-Dorffmann (RDD)?

Lesi sifo asithinti wonke umuntu ngendlela efanayo. Abanye abantu banokuvuvukala endaweni eyodwa, kanti abanye bangase babe nokuvuvukala ezindaweni eziningana. Futhi, lawa ma-histiocyte anganqwabelana hhayi kuphela ezigaxeni kodwa nakwezinye izingxenye zomzimba. Yile ndlela ehlukaniswa ngayo.

• Isifo Esijwayelekile (I-Nodal) Ross-Dorffmann (i-Classic/Nodal RDD): Lolu uhlobo oluvame kakhulu. Luhilela kakhulu ukuvuvukala kwama-node entanyeni. Kodwa-ke, izimpawu zingahluka kuye ngokuthi mangaki ama-node athintekile.
• I-Extranodal RDD: Kulolu hlobo, lawa ma-histiocyte aqoqana ezicutshini ezingezona ama-lymph node. Isikhumba sivame ukuthinteka. Lokhu kubizwa ngokuthi i-cutaneous RDD (CRDD) . Ngaphezu kwalokho, lesi simo singathinta ama-sinuses ethu, amehlo namajwabu amehlo, amathambo, kanye nesistimu yezinzwa ephakathi (CNS) - okungukuthi, ubuchopho kanye nomgogodla. Ngezinye izikhathi uhlelo lokuphefumula kanye nohlelo lokugaya ukudla nakho kungathinteka.

Cishe abantu abangu-40% abane-RDD bangase babe nala maseli e-histiocyte aqoqwe kwezinye izingxenye zomzimba, ngaphezu kwama-warts, njengoba ushilo.

Ubani onamathuba amaningi okuthola lesi sifo?

Isifo sikaRoss-Dorffmann sithinta kakhulu izingane, intsha kanye nabantu abadala abasebasha . Sivame ukutholakala kubantu abaseminyakeni yabo yama-20. Kodwa-ke, abantu abaseminyakeni yabo yama-70 nabo kubikwe ukuthi banalesi sifo.

I-RDD, ethinta amasende, ivame kakhulu kwabesilisa abadabuka e-Afrika. Kodwa isimo sesikhumba esishiwo ngaphambilini esithi "CRDD" sivame kakhulu kwabesifazane abadabuka e-Asia. I-"CRDD" ivame kakhulu kubantu abaneminyaka engama-20, 30, kanye nengama-40.

Isifo sikaRoss-Dorfman singavamile kangakanani?

Lesi yisifo esingavamile kakhulu . Sithinta cishe umuntu oyedwa kwabayizinkulungwane ezingamakhulu amabili. Isibonelo, e-United States, kubikwa amacala amasha angaba yi-100 minyaka yonke. Lokho kusho ukuthi eSri Lanka, sincane kakhulu.

Ziyini izimpawu zesifo iRose-Dorfman (RDD)?

Izimpawu ozibonayo zizoncika ekutheni ama-histiocyte amaningi aqongelela kuphi emzimbeni wakho. Uma kuthinteka ama-lymph node entanyeni yakho kuphela, izimpawu zakho zingase zibe zincane noma zingabi khona nhlobo. Kodwa-ke, uma la maseli eqongelela futhi ephazamisa ukusebenza kwesitho, izimpawu zakho zingase zibe zimbi kakhulu.

Izimpawu zakudala (ezinamaqhubu)

Ama-Histiocyte avame ukuqongelela ezicutshini zentamo. Ngakho-ke, uphawu oluvame kakhulu ukubonakala kwamaqhubu angenabuhlungu, avuvukele ezinhlangothini zombili zentamo . Ungase ukhumbule ukuthi ngezinye izikhathi uthola iqhubu elincane entanyeni yakho uma unomkhuhlane noma umkhuhlane, akunjalo? Kunjalo, kodwa kungase kungabi buhlungu. Kodwa-ke, kuye ngokuthi izicubu ezithintekile zikuphi, ukuvuvukala kungaba nakwezinye izindawo.

Izindawo eziyinhloko zokuvuvukala okungenzeka ngenxa yokwanda kwama-histiocytes yilezi:

• Intamo yakho
• Indawo yokukhula
• Amakhwapha
• Iphakathi kwesifuba (i-mediastinum)

Ungase ungaboni ezinye izimpawu ngaphandle kokuvuvukala, noma ungase uqaphele izimpawu ezifana nalezi:

• Imfiva
• Isikhumba esikhanyayo
• Ukukhathala okukhulu
• Ukujuluka ebusuku
• Impumulo egobhozayo
• Ukwehlisa isisindo okungachazeki

Izimpawu zangaphandle kwe-node

Isifo sikaRose-Dorffmann (`CRDD`) singathinta isikhumba futhi singavela noma kuphi emzimbeni. Lawa maqhubu, akhiwa yiqoqo lamaseli e-histiocyte, avame ukukhula kancane. Izimpawu ezingabonakala esikhumbeni zifaka:

• Amaqhubu ayisicaba noma aphakanyisiwe
• Amaqhubu agcwele ubomvu noma aqinile
• Amaqhubu aphuzi, onsomi, obomvu, noma onsundu
• Kungase kusabalale esikhumbeni sonke noma kukhawulelwe endaweni eyodwa.

Uma la ma-histiocyte amaningi ethinta isitho esithile noma uhlelo lomzimba, kungavela izimpawu ezahlukahlukene. Isibonelo, uma i-RDD ithinta amehlo, ingabangela ukubona okubili. Uma ithinta uhlelo lwezinzwa oluphakathi, ingabangela ukuquleka. Uma ithinta amaphaphu, ingabangela ukukhwehlela okuqhubekayo. Ngokufanayo, izimpawu ziyahlukahluka kuye ngendawo ethintekile.

Yini ebangela isifo sikaRoss-Dorffman (RDD)?

Ososayensi abakayazi imbangela eqondile yalesi sifo. Njengoba i-RDD ithinta umuntu ngamunye ngendlela ehlukile, kungaba nezimbangela eziningana. Isibonelo, ucwaningo lwamuva lubonisa ukuthi isimo sesikhumba "i-CRDD" sibangelwa yinto ehlukile kunezinye izinto ezibangela i-RDD evamile.

Abacwaningi basanda kuthola ukuthi izinguquko ezithile zezakhi zofuzo zitholakala kwezinye izinhlobo ze-RDD ngaphandle kwe-CRDD. Lezi zinguquko zingabangela ukuba amangqamuzana akhule ngendlela engalawuleki.

Abantu abaningi abane-RDD banalesi simo kanye nezinye izimo zezokwelapha. Ngakho-ke, kungase kube nokuxhumana phakathi kwalezo zimo ne-RDD. Kudingeka ucwaningo olwengeziwe ngalokhu.

Kutholakale ukuthi i-RDD ihlotshaniswa nalezi zimo ezilandelayo:

• Ukutheleleka ngegciwane: Izibonelo zifaka phakathi i-herpes, igciwane le-Epstein-Barr, i-cytomegalovirus, kanye nokutheleleka nge-HIV.
• Umdlavuza: I-Hodgkin lymphoma, i-non-Hodgkin lymphoma, kanye nomdlavuza wesikhumba njenge-cutaneous clear-cell sarcoma. (Kodwa khumbula, i-RDD ayiwona umdlavuza.)
• Izimo zokuzivikela komzimba: I-Lupus, I-Juvenile Idiopathic Arthritis, I-Autoimmune Hemolytic Anemia.

Isifo sikaRoss-Dorffman (RDD) sitholakala kanjani?

Udokotela wakho uzoqala ngokukuhlola umzimba bese ebuza ngezimpawu zakho. Uzobheka izimpawu ze-RDD, njengezindlala ezivuvukele kanye namaqhubu esikhumba. Uzobuza nangomlando wakho wezokwelapha ukuze abone ukuthi unazo noma uke waba nazo yini izimo ezihlobene ne-RDD.

Ngaphezu kwalokho, udokotela angenza nalezi zivivinyo:

• Izinqubo zokuthatha izithombe: Kuye ngohlobo lwezicubu okusolwa ukuthi ziqukethe ama-histiocyte, udokotela angase acele i-X-ray, i-ultrasound, i-MRI, i-CT, i-PET, i-PET/CT, noma i-bone scan. Lokhu kungasetshenziswa ukubona ukuthi yini engaphakathi emzimbeni.
• Ukuhlolwa kwegazi: Ukuhlolwa kwegazi okuningana, njengokubalwa kwegazi okuphelele (i-CBC) kanye nephaneli ephelele ye-metabolic, kungenziwa ukuze kubonwe ukuthi yiziphi izinguquko ezenzeka emzimbeni.
• I-Biopsy: Lolu vivinyo olubaluleke kakhulu.Lokhu kuhilela ukuthatha isampula encane yezicubu ezithintekile (isib., i-lymph node evuvukele) nokuhlola amaseli ngaphansi kwesibonakhulu ukuze kubonakale ukuthi anezici ze-RDD. Lokhu kuhlolwa kwegazi kunganquma nokuthi ngabe esinye isifo sibangela ukukhula kweseli okungavamile.

Isifo sikaRoss-Dorffmann (RDD) selashwa kanjani?

Ngezinye izikhathi, i-RDD ingadlula ngokushesha ngaphandle kokwelashwa. Lokhu kusho ukuthi lesi sifo sizoba ngcono ngokwaso. Kodwa-ke, isikhathi esizothathwa kunzima ukusibikezela. Kungathatha izinyanga noma ngisho neminyaka ukuba ngcono. Kodwa-ke, kwezinye izimo, singase singapheli sodwa, noma singabuya. Uma singelashwa, lesi sifo singase sibe sibi kakhulu.

Ukwelashwa okutholayo kuzoncika ekutheni i-RDD ikuthinte kanjani.

• Ukuqaphela: Uma ungenazo izimpawu ezithinta kakhulu impilo yakho, udokotela wakho angase anqume ukuqapha isimo sakho. Lokhu kufana nendlela "yokulinda ngokuqapha".
• Ukuhlinzwa: Amaqhubu angasuswa ngokuhlinzwa. Uma une-`CRDD` (cutaneous RDD), noma uma amaqhubu evimba indlela yakho yokuphefumula noma ethinta umgogodla wakho, ukuhlinzwa kungenziwa.
• Ukwelashwa ngemisebe: Uma ukuhlinzwa kungakwazi ukususa ama-histiocyte, ukwelashwa ngemisebe kunganikezwa. Lokhu kuhilela ukusebenzisa umshini ukuqondisa imisebe yemisebe eqondiwe emaseli, uwabhubhise.
• Ukwelashwa ngamakhemikhali: Uma lesi sifo sibi kakhulu, noma uma ezinye izindlela zokwelapha, njengokuhlinzwa, zinganciphisanga izimpawu, kungacatshangwa ukuthi kwenziwa ngamakhemikhali. Lokhu kuhilela ukusetshenziswa kwemithi ebulala amangqamuzana akhula ngokushesha, njengamaseli omdlavuza.
• Ama-Corticosteroids: Ama-Corticosteroids, njenge-prednisone, anganciphisa ukuvuvukala kwamalunga futhi aqede izimpawu. Lawa uhlobo lomuthi onciphisa ukuvuvukala.
• Ukwelashwa Ngezifo Ezingalawuleki: Lokhu kwelashwa kusiza amasosha omzimba wakho ukuthola nokubhubhisa amaseli e-histiocyte amaningi ngempumelelo enkulu.

Liyini ikusasa lomuntu onesifo iRoss-Dorffman (RDD)?

Ukubikezela kwakho kuncike ezintweni eziningana. Inani lamaseli athintekile, ukuthi ama-histiocyte amaningi emzimbeni wakho akuphi, nokuthi usabela kahle kangakanani ekwelashweni. Esikhathini esiningi, i-RDD iyazixazulula ngokwayo. Kodwa-ke, kwezinye izimo, kudingeka ukwelashwa, noma kungaba nezinkinga ezinkulu.

Ngokuvamile, uma inani lamaseli athintekile lincipha, kuba ngcono umphumela.Uma kukhona i-RDD engaphandle kwe-nodal, ethinta isikhumba, isifuba, noma umgudu wokuphefumula ophezulu, umphumela cishe uzoba muhle. Kodwa-ke, uma ama-histiocyte ekhona emgudwini wokuphefumula ongezansi, izinso, noma isibindi, isimo singase sibe sibi kakhulu.

Yimiphi imibuzo okufanele ngiyibuze udokotela wami?

Ungakhohlwa ukukhuluma nodokotela wakho ngalezi zinto, ngoba kubaluleke kakhulu ukuthi uqonde kahle isimo sakho:

• "Dokotela, yiziphi izingxenye zomzimba wami ezithintekile yisimo sami se-RDD?"
• "Ingabe kuzodingeka ngenze ezinye izivivinyo zezithombe (ezifana nokuskena) noma ukuhlolwa kwegazi?"
• "Ingabe ngidinga ukwelashwa, noma kungenzeka ukuthi isimo sami se-RDD sizozixazulula sodwa?"
• "Kufanele ngikhathazeke kangakanani ngalesi simo se-RDD?"
• "Yikuphi ukwelashwa okungcono kakhulu udokotela angincomayo?"
• "Yimiphi imiphumela emibi engingayilindela ngemva kokwelashwa?"
• "Yimaphi amathuba okuba isimo sami se-RDD siphinde sibuye ngemva kokwelashwa?"
• "Kudingeka ngize kangaki ukuzohlolwa ukuze ngiqaphe isimo sami?"

Ingabe isifo sikaRoss-Dorffman (RDD) siwumdlavuza?

Cha. Lona umbuzo abantu abaningi abawubuzayo. Isifo sikaRose-Dorffman asisona umdlavuza (ongemuhle) . Okusho ukuthi, asisabalali sisuka kwenye ingxenye yomzimba siye kwenye njengomdlavuza (umdlavuza). Kodwa-ke, amaqhubu akheka ngenxa ye-RDD angathinta izitho zomzimba, aphazamise ukusebenza kwazo, futhi abangele izinkinga. Udokotela wakho ungumuntu ongcono kakhulu ukwazi ukuthi isimo sakho se-RDD sithinta kanjani umzimba wakho nokuthi kufanele ukhathazeke ngaso.

Ubani owathola isifo sikaRoss-Dorffman (RDD)?

Kuhle ukwazi lokhu futhi. Ngo-1965, usosayensi ogama lakhe linguPierre Paul Louis Lucien Destombes wathola izimpawu ze-RDD ezigulini ezine. Kwabe sekuthi ngo-1969, ososayensi ababili ogama lakhe linguJuan Rosai noRonald Dorfman bahlola elinye iqembu leziguli ezinezimpawu ezifanayo. Lesi sifo manje saziwa kakhulu ngokuthi isifo sikaRosai-Dorfman, sihlanganisa amagama abo. Akuvamile ukuthi sibizwe nangokuthi isifo sikaRosai-Dorfman-Destombes, ukuhlonipha bonke ososayensi abathathu.

Okokugcina, izinto okufanele uzikhumbule (Umyalezo Wokuya Ekhaya)

Isifo iRoss-Dorffmann (RDD) singaba ukuxilongwa okunzima ngoba asivamile futhi sithinta umuntu ngamunye ngendlela ehlukile . Akukho siqondiso esisodwa sokwelashwa kwe-RDD. Yingakho kubalulekile ukukhuluma ngokukhululekile nodokotela wakho, ukuqonda ukuxilongwa kwakho, nokuqaphela izinketho zakho zokwelashwa.

Ungase ube ne-RDD encane ezophela yodwa. Noma, ungase udinge inhlanganisela yokwelashwa. Buza udokotela wakho ukuthi uhlelo lwakho lokwelashwa kanye nemiphumela okulindelekile kuzokwenziwa kanjani ngokuvumelana nesimo sakho.

Khumbula, awuwedwa . Uma ubhekene nesimo esingavamile kangaka, kubaluleke kakhulu ukulandela iseluleko sezokwelapha futhi uhlale uqinile engqondweni. Uma unemibuzo noma ukungabaza, xoxa ngakho nodokotela wakho. Ungesabi, ngoba ukuqwashisa kuyisikhali esingcono kakhulu sokulwa nezinto ezinjalo.

Isifo sikaRosai -Dorfman, i-RDD, ama-histiocyte, i-lymphadenopathy, amaqhubu entamo, izifo zesikhumba, i-CRDD, izifo ezingavamile, isimiso somzimba sokuzivikela