Zithini ezi ziqhuma entanyeni nasemakhwapheni? Masifunde ngeSifo sikaRosai-Dorfman!

Kuqhelekile ukuziva usoyika kancinci xa sibona utshintsho emizimbeni yethu, akunjalo? Ingakumbi xa sibona amaqhuma amancinci entanyeni, emakhwapheni, okanye emathunjini, sicinga, 'Yintoni le?' Namhlanje siza kuthetha ngemeko engaqhelekanga enokubangela amaqhuma anjalo, kodwa abantu abaninzi abakhe beva ngayo. Oku kubizwa ngokuba yiRosai-Dorfman Disease.

Ngaba uyazi ukuba yintoni isifo sikaRoss-Dorffman (RDD)?

Ngamafutshane, imizimba yethu inohlobo lweeseli ezimhlophe zegazi ezibizwa ngokuba zii-histiocytes . Ezi zifana namapolisa emizimbeni yethu, zilwa neentsholongwane ezibangela izifo, kwaye ziyinxalenye ebalulekileyo yenkqubo yethu yokuzikhusela. Nangona kunjalo, xa ezi histiocytes zikhula kakhulu, zibizwa ngokuba sisifo sikaRoss-Dorffmann (RDD). Oku akusiyo umhlaza (ongengomhlaza) , oko kuthetha ukuba ayisosifo esiyingozi esisasazeka ukusuka kwelinye icala lomzimba ukuya kwelinye. Nangona kunjalo, xa ezi seli ziqokelelana kakhulu, sinokuziva singakhululekanga kwaye sineempawu.

Ixesha elininzi, la ma-histiocyte agqithisileyo aqokelelana kwii-lymph nodes entanyeni yethu. Emva koko avuvukala njengemibhobho entanyeni. Oogqirha ngamanye amaxesha babiza oku ngokuthi kukudumba kwama-nodes `(lymphadenopathy).` Kodwa oku kunokuchaphazela kungekuphela nje intamo, kodwa kunye nama-nodes kwezinye iindawo zomzimba. Ngamanye amaxesha, ukongeza kuma-nodes, la maseli anokuqokelelana nakwezinye iindawo zomzimba (ii-extranodal sites).

Esi sifo sikwabizwa ngokuba yi-'sinus histiocytosis ene-massive lymphadenopathy'. Esi sisifo esingaqhelekanga esikwiqela le-'non-Langerhans cell histiocytosis'.

Ziziphi iintlobo eziphambili zesifo iRoss-Dorffmann (RDD)?

Esi sifo asibachaphazeli bonke abantu ngokufanayo. Abanye abantu banokudumba kwindawo enye, ngelixa abanye banokuba nokudumba kwiindawo ezahlukeneyo. Kwakhona, ezi histiocytes zinokuqokelelana kungekuphela nje kumaqhuma kodwa nakwezinye iindawo zomzimba. Le yindlela ehlelwa ngayo.

• Isifo seRoss-Dorffmann (i-Nodal) esiqhelekileyo (i-Classic/Nodal RDD): Olu lolona hlobo luxhaphakileyo. Ngokuyintloko lubandakanya ukudumba kwamaqhuqhuva entanyeni. Nangona kunjalo, iimpawu zinokwahluka ngokuxhomekeke ekubeni zingaphi amaqhuqhuva achaphazelekileyo.
• I-Extranodal RDD: Kolu hlobo, la ma-histiocyte aqokelelana kwizicubu ezingezizo ii-lymph nodes. Ulusu luchaphazeleka kakhulu. Oku kubizwa ngokuba yi -cutaneous RDD (CRDD) . Ukongeza, le meko inokuchaphazela ii-sinuses zethu, amehlo kunye neenkophe, amathambo, kunye nenkqubo ye-nervous system (CNS) - oko kukuthi, ingqondo kunye nomgogodla. Ngamanye amaxesha inkqubo yokuphefumla kunye nenkqubo yesisu nazo zinokuchaphazeleka.

Malunga nama-40% abantu abane-RDD banokuba nezi seli ze-histiocyte eziqokelelwe kwezinye iindawo zomzimba, ukongeza kwiintsumpa, njengoko ukhankanyile.

Ngubani onokuthi afumane esi sifo?

Isifo sikaRoss-Dorffmann sichaphazela kakhulu abantwana, abantu abakwishumi elivisayo kunye nabantu abadala abaselula . Sidla ngokufunyaniswa kubantu abakwiminyaka yama-20. Nangona kunjalo, abantu abakwiminyaka yama-70 nabo baye baxelwa ukuba banaso esi sifo.

I-RDD, echaphazela amasende, ixhaphake kakhulu kumadoda anemvelaphi yaseAfrika. Kodwa imeko yolusu ekhankanyiweyo ngaphambili ethi "CRDD" ixhaphake kakhulu kubafazi abanemvelaphi yaseAsia. I-"CRDD" ixhaphake kakhulu kubantu abakwiminyaka yabo yama-20, yama-30, nama-40.

Isifo sikaRoss-Dorfman sinqabile kangakanani?

Esi sisifo esingaqhelekanga kakhulu . Sichaphazela malunga nomntu omnye kumakhulu amabini amawaka. Umzekelo, eMelika, malunga ne-100 lamatyala amatsha axelwa minyaka le. Oko kuthetha ukuba eSri Lanka, angaphantsi nangakumbi.

Zithini iimpawu zesifo sikaRose-Dorfman (RDD)?

Iimpawu ozifumanayo ziya kuxhomekeka ekubeni ziqokelelana phi emzimbeni wakho ii-histiocytes ezingaphezulu. Ukuba kuphela zii-lymph nodes ezikwintamo yakho ezichaphazelekileyo, iimpawu zakho zisenokuba zincinci okanye azikho kwaphela. Nangona kunjalo, ukuba ezi seli ziqokelelana kwaye ziphazamisana nomsebenzi wesitho, iimpawu zakho zinokuba zimbi ngakumbi.

Iimpawu zakudala (ezinamaqhuqhuva)

IiHistiocytes zihlala ziqokelelana kwizicubu zentamo. Ke ngoko, uphawu oluqhelekileyo kukubonakala kwamaqhuqhuva angenantlungu, avuvukileyo kumacala omabini entamo . Usenokukhumbula ukuba ngamanye amaxesha ufumana iqhuqhuva elincinci entanyeni yakho xa unomkhuhlane okanye umkhuhlane, akunjalo? Kunjalo, kodwa kusenokungabi buhlungu. Nangona kunjalo, kuxhomekeke ekubeni izicubu ezichaphazelekayo ziphi, ukudumba kunokuba nakwezinye iindawo.

Iindawo eziphambili zokuvuvukala ezinokwenzeka ngenxa yokwanda kwe-histiocytes zezi:

• Intamo yakho
• Indawo yokukhula
• Amakhwapha
• Umbindi wesifuba (mediastinum)

Usenokungaziboni ezinye iimpawu ngaphandle kokudumba, okanye unokuzibona ezi mpawu zilandelayo:

• Ifiva
• Ulusu olukhanyayo
• Ukudinwa kakhulu
• Ukubila ebusuku
• Impumlo enemifinya
• Ukunciphisa umzimba ngendlela engachazekiyo

Iimpawu ze-Extranodal

Isifo sikaRose-Dorffmann (`CRDD`) sinokuchaphazela ulusu kwaye sinokuvela naphi na emzimbeni. La maqhuqhuva, enziwa yingqokelela yeeseli ze-histiocyte, adla ngokukhula kancinci. Iimpawu ezinokubonakala kulusu ziquka:

• Amaqhuma atyatyekileyo okanye aphakanyisiweyo
• Amaqhuma azaliswe bubovu okanye aqinileyo
• Amaqhubu atyheli, amfusa, abomvu, okanye amdaka
• Ingasasazeka kulo lonke ulusu okanye inqunyelwe kwindawo enye.

Xa ezi histiocytes zigqithisileyo zichaphazela ilungu elithile okanye inkqubo yomzimba, iimpawu ezahlukeneyo zinokubakho. Umzekelo, ukuba i-RDD ichaphazela amehlo, inokubangela ukubona kabini. Ukuba ichaphazela inkqubo yemithambo-luvo ephakathi, inokubangela ukuxhuzula. Ukuba ichaphazela imiphunga, inokubangela ukukhwehlela okungapheliyo. Ngokufanayo, iimpawu ziyahluka ngokuxhomekeke kwindawo echaphazelekayo.

Yintoni ebangela isifo sikaRoss-Dorffman (RDD)?

Izazinzulu azikayazi eyona nto ibangela esi sifo. Ekubeni i-RDD ichaphazela umntu ngamnye ngendlela eyahlukileyo, kunokubakho izizathu ezininzi. Umzekelo, uphando lwakutshanje lubonisa ukuba imeko yolusu ethi "CRDD" ibangelwa yinto engeyiyo le nto ibangela i-RDD eqhelekileyo.

Abaphandi bafumanise kutshanje ukuba utshintsho oluthile lwezakhi zofuzo lufumaneka kwezinye iintlobo ze-RDD ngaphandle kwe-CRDD. Olu tshintsho lunokubangela ukuba iiseli zikhule ngendlela engalawulekiyo.

Abantu abaninzi abane-RDD banale meko kunye nezinye iimeko zonyango. Ngoko ke, kunokubakho unxibelelwano phakathi kwezo meko kunye ne-RDD. Kufuneka uphando oluthe kratya ngale nto.

Kufunyaniswe ukuba i-RDD inxulumene nezi meko zilandelayo:

• Usulelo lwentsholongwane: Imizekelo ibandakanya i-herpes, i-Epstein-Barr virus, i-cytomegalovirus, kunye nosulelo lwe-HIV.
• Umhlaza: I-Hodgkin lymphoma, i-non-Hodgkin lymphoma, kunye nomhlaza wolusu ofana ne-cutaneous clear-cell sarcoma. (Kodwa khumbula, i-RDD ayingomhlaza.)
• Iimeko zokuzikhusela komzimba: I-Lupus, i-Juvenile Idiopathic Arthritis, i-Autoimmune Hemolytic Anemia.

Isifo sikaRoss-Dorffman (RDD) sifunyanwa njani?

Ugqirha wakho uza kuqala enze uvavanyo lomzimba aze akubuze ngeempawu zakho. Baza kukhangela iimpawu ze-RDD, ezifana nokudumba kwamadlala kunye namaqhuma esikhumba. Baza kubuza nangembali yakho yezonyango ukuze babone ukuba unayo okanye unayo nayiphi na imeko enxulumene ne-RDD.

Ukongeza, ugqirha angenza olu vavanyo:

• Iinkqubo zokuthatha imifanekiso: Ngokuxhomekeke kuhlobo lwezicubu ekucingelwa ukuba ziqulethe ii-histiocytes, ugqirha unokuyalela ukuba kwenziwe i-X-ray, i-ultrasound, i-MRI, i-CT, i-PET, i-PET/CT, okanye i-bone scan. Ezi zingasetyenziswa ukubona ukuba yintoni ngaphakathi emzimbeni.
• Uvavanyo lwegazi: Uvavanyo lwegazi oluninzi, olufana nokubalwa kwegazi elipheleleyo (i-CBC) kunye nephaneli epheleleyo ye-metabolic, lunokwenziwa ukuze kubonwe ukuba loluphi utshintsho oluqhubekayo emzimbeni.
• I-Biopsy: Olu luvavanyo olubaluleke kakhulu.Oku kuquka ukuthatha isampuli encinci yezicubu ezichaphazelekileyo (umz., i-lymph node evuvukileyo) kunye nokuhlola iiseli phantsi kwe-microscope ukuze kubonwe ukuba zineempawu ze-RDD na. Olu vavanyo lwe-biopsy lunokufumanisa ukuba kukho esinye isifo esibangela ukukhula kweseli okungaqhelekanga.

Isifo sikaRoss-Dorffmann (RDD) sinyangwa njani?

Ngamanye amaxesha, i-RDD inokubuyela kwimeko yesiqhelo ngaphandle konyango. Oku kuthetha ukuba esi sifo siza kuba ngcono ngokwaso. Nangona kunjalo, ixesha elithathayo kunzima ukuliqikelela. Kungathatha iinyanga okanye iminyaka ukuba siphile. Nangona kunjalo, kwezinye iimeko, sisenokungapheli ngokwaso, okanye sinokubuya. Ukuba asinyangwa, esi sifo sinokuba sibi kakhulu.

Unyango olufumanayo luya kuxhomekeka kwindlela i-RDD ekuchaphazele ngayo.

• Ukuqwalaselwa: Ukuba awunazo iimpawu ezinefuthe elikhulu ebomini bakho, ugqirha wakho unokugqiba ekubeni ajonge imeko yakho. Oku kufana nendlela "yokulinda ngononophelo".
• Utyando: Amaqhuma anokususwa ngotyando. Ukuba une-`CRDD` (cutaneous RDD), okanye ukuba amaqhuma avala indlela yakho yomoya okanye achaphazela umqolo wakho, utyando lunokwenziwa.
• Unyango ngemitha: Ukuba utyando alukwazi ukususa ii-histiocytes, unyango ngemitha lunokunikwa. Oku kubandakanya ukusebenzisa umatshini ukuqondisa imisebe yemitha ekujoliswe kuyo kwiiseli, uzitshabalalise.
• Unyango lweKhemotherapy: Ukuba isifo sibi kakhulu, okanye ukuba ezinye iindlela zonyango, ezinje ngotyando, azizange zinciphise iimpawu, unyango lwekhemotherapy lunokuthathwa ingqalelo. Oku kubandakanya ukusetyenziswa kwamayeza abulala iiseli ezikhula ngokukhawuleza, ezifana neeseli zomhlaza.
• IiCorticosteroids: IiCorticosteroids, ezifana ne-prednisone, zinokunciphisa ukudumba kwamalungu kwaye zithomalalise iimpawu. Ezi luhlobo lwamayeza anciphisa ukudumba.
• Unyango lwe-Immunotherapy: Olu nyango lunceda inkqubo yakho yomzimba ifumane kwaye itshabalalise iiseli ze-histiocyte ezigqithisileyo ngempumelelo.

Liyintoni ikamva lomntu onesifo iRoss-Dorffman (RDD)?

Ingqikelelo yakho ixhomekeke kwizinto ezininzi. Inani leeseli ezichaphazelekileyo, apho kukho khona ii-histiocytes ezigqithisileyo emzimbeni wakho, kunye nendlela osabela ngayo kunyango. Uninzi lwexesha, i-RDD iyazigqibela ngokwayo. Nangona kunjalo, kwezinye iimeko, kufuneka unyango, okanye iingxaki ezinkulu zinokwenzeka.

Ngokubanzi, okukhona inani leeseli ezichaphazelekileyo lincinci, kokukhona iziphumo ziba ngcono.Ukuba kukho i-RDD engaphandle kwe-nodal, echaphazela ulusu, isifuba, okanye indlela yokuphefumla ephezulu, isiphumo sinokuba sihle ngakumbi. Nangona kunjalo, ukuba ii-histiocytes zikhona kwindlela yokuphefumla esezantsi, izintso, okanye isibindi, imeko inokuba mandundu kancinci.

Ndingayibuza yiphi imibuzo ugqirha wam?

Ungalibali ukuthetha nogqirha wakho ngezi zinto, kuba kubaluleke kakhulu ukuba uyiqonde kakuhle imeko yakho:

• "Gqirha, zeziphi iindawo zomzimba wam ezichaphazeleka yimeko yam ye-RDD?"
• "Ngaba kuya kufuneka ndiphinde ndenze ezinye iimvavanyo zokuthatha imifanekiso (ezifana neeskeni) okanye iimvavanyo zegazi?"
• "Ngaba ndifuna unyango, okanye kunokwenzeka ukuba imeko yam ye-RDD iza kuphelela ngokwayo?"
• "Ndifanele ndikhathazeke kangakanani ngale meko ye-RDD?"
• "Yeyiphi eyona ndlela ilungileyo yonyango ugqirha andicebisa yona?"
• "Zeziphi iziphumo ebezingalindelekanga endinokuzilindela emva konyango?"
• "Ngawaphi amathuba okuba imeko yam ye-RDD ibuyele emva konyango?"
• "Kufuneka ndize kangaphi ukuze ndihlolwe ukuze ndijonge imeko yam?"

Ngaba isifo sikaRoss-Dorffman (RDD) sisifo somhlaza?

Hayi. Lo ngumbuzo abantu abaninzi abawubuzayo. Isifo sikaRose-Dorffman asingomhlaza (ongengomhlaza) . Oko kukuthi, asisasazeki ukusuka kwelinye icala lomzimba ukuya kwelinye njengomhlaza (umhlaza). Nangona kunjalo, amaqhuma avela ngenxa ye-RDD anokuchaphazela amalungu omzimba, aphazamise ukusebenza kwawo, kwaye abangele iingxaki. Ugqirha wakho ngumntu ongcono ukwazi indlela imeko yakho ye-RDD echaphazela ngayo umzimba wakho kunye nokuba kufuneka ukhathazeke ngayo.

Ngubani owafumanisa isifo iRoss-Dorffman (RDD)?

Kulungile ukwazi oku. Ngo-1965, isazinzulu egama linguPierre Paul Louis Lucien Destombes safumanisa iimpawu ze-RDD kwizigulana ezine. Emva koko, ngo-1969, izazinzulu ezimbini ezigama linguJuan Rosai noRonald Dorfman bafunda elinye iqela lezigulana ezineempawu ezifanayo. Esi sifo ngoku saziwa njengesifo sikaRosai-Dorfman, sidibanisa amagama abo. Amaxesha amaninzi, sikwabizwa ngokuba sisifo sikaRosai-Dorfman-Destombes, ukuhlonipha zonke izazinzulu ezintathu.

Okokugqibela, izinto ekufuneka uzikhumbule (Umyalezo Wokuya Ekhaya)

Isifo iRoss-Dorffmann (RDD) sinokuba sisifo esinzima kuba asiqhelekanga kwaye sichaphazela umntu ngamnye ngokwahlukileyo . Akukho sikhokelo sinye sonyango lwe-RDD. Yiyo loo nto kubalulekile ukuthetha ngokukhululekileyo nogqirha wakho, ukuqonda isifo osifumeneyo, kwaye uqaphele iindlela zakho zonyango.

Usenokuba ne-RDD encinci eza kuzilungisa ngokwayo. Okanye, ungadinga indibaniselwano yonyango. Buza ugqirha wakho ukuba isicwangciso sakho sonyango kunye neziphumo ezilindelekileyo ziya kulungiswa njani ngokwemeko yakho.

Khumbula, awuwedwa . Xa ujongene nemeko engaqhelekanga kangaka, kubaluleke kakhulu ukulandela iingcebiso zonyango kwaye uhlale uqinile engqondweni. Ukuba unemibuzo okanye amathandabuzo, xoxa ngawo nogqirha wakho. Musa ukoyika, kuba ukuqonda sesona sixhobo silungileyo sokulwa nezinto ezinjalo.

Isifo sikaRosai -Dorfman, i-RDD, ii-histiocytes, i-lymphadenopathy, amaqhuma entamo, izifo zolusu, i-CRDD, izifo ezingaqhelekanga, inkqubo yomzimba yokuzikhusela