Ngaba kukho ingxaki ngeeseli zakho ezimhlophe zegazi? Masifunde nge-Histiocytosis ngokulula!

Ubusazi ukuba ngamanye amaxesha iintlobo ezithile zeeseli emizimbeni yethu ziqala ukuziphatha ngendlela eyahlukileyo? Enye imeko engaqhelekanga enjalo ibizwa ngokuba yiHistiocytosis. Oku kunokuvakala kuyoyikisa kancinci, kodwa ungakhathazeki. Masithethe ngayo ngokulula nangendlela enengqiqo.

Yintoni i-Histiocytosis? Ngamazwi alula...

Umzimba wakho unohlobo lweeseli ezimhlophe zegazi ezibizwa ngokuba zii-histiocytes. Phakathi kwezo seli ezimhlophe zegazi, kukho uhlobo olukhethekileyo lweeseli ezibizwa ngokuba zii-histiocytes . Ezi zifana noonogada bomzimba wethu. Ngabo balwa neentsholongwane ezibangela izifo ezibizwa ngokuba zii-pathogens - iintsholongwane , iibhaktheriya kunye nefungi - kwaye banceda ukugcina inkqubo yethu yomzimba isempilweni.

Nangona kunjalo, ngamanye amaxesha ezi seli ze-histiocyte ziqala ukukhula ngendlela engaqhelekanga, oko kukuthi, ziba "zii- mutant " . Emva koko, ezi seli ze-histiocyte ezingaqhelekanga zikhula kakhulu kwaye ziqokelele kwiindawo ezahlukeneyo zomzimba wethu. Xa ziqokeleleka kakhulu, inkqubo yethu yomzimba inokuba neengxaki. Eyona nto iphambili kukuba ukudumba , oko kukuthi, imeko efana nokudumba, kwenzeka kwizicubu zomzimba. Oku kudumba ngamanye amaxesha kunokonakalisa amalungu ethu omzimba.

Ezi seli ze-histiocyte ezingaqhelekanga zinokuqokelelana kwii -lymph nodes zakho (ezikwabizwa ngokuba zii-lymph nodes), ulusu, amathambo, imiphunga, isibindi, i-spleen, okanye naphi na emzimbeni wakho. Ngamanye amaxesha ichaphazela inxalenye enye yomzimba wakho. Oku sikubiza ngokuba yimeko 'yenkqubo enye' . Ngamanye amaxesha kunokuchaphazela iindawo ezininzi zomzimba wakho. Oku sikubiza ngokuba yimeko 'yenkqubo ezininzi' .

Into ebalulekileyo kukuba i-histiocytosis ayingomhlaza . Nangona kunjalo, ngamanye amaxesha inokuziphatha njengomhlaza kwaye ibonise iimpawu ezifana nezomhlaza. Yiyo loo nto yimeko efuna ingqalelo ekhethekileyo. Ngenxa yokuba iimpawu zoku ziyahluka kumntu nomntu kwaye ubunzima bemeko buyahluka, ngamanye amaxesha kunokuba nzima ukufumanisa i-histiocytosis ngokukhawuleza.

Ziziphi iintlobo eziphambili ze-histiocytosis?

Ngoku mhlawumbi uyaqonda ukuba yintoni i-histiocytosis. Kukho iintlobo zayo ezingaphezu kwe-100. Kodwa kukho iintlobo ezintathu eziphambili esizibonayo nesithetha ngazo rhoqo. Makhe sizijonge.

1. I-Langerhans cell histiocytosis (LCH):

• Olu lolona hlobo luqhelekileyo lwe-histiocytosis.
• Le meko idla ngokuchaphazela abantwana abancinci . Ichaphazela abantwana abaphakathi kwe-5 ne-9 kwizigidi ngezigidi.
• Ixesha elininzi, oku akuyonto imbi kangako, kwaye kunokuba lula njengokuthi "akukho zimpawu" . Nangona kunjalo, kubalulekile ukukhumbula ukuba kwezinye iimeko, oku kunokuba nzima kwaye kubeke ubomi esichengeni.

2. Isifo se-Erdheim-Chester (ECD):

• Olu hlobo luchaphazela kakhulu abantu abadala .
• Le yimeko engaqhelekanga kakhulu .
• Njenge-LCH, i-ECD inokwahluka ukusuka kwencinci ukuya kweqatha kwaye isongela ubomi.

3. Isifo sikaRosai-Dorfman (RDD):

• Njenge-LCH, i-RDD idla ngokuvela ebantwaneni , kodwa inokwenzeka nakubantu abadala.
• Oku nako kunqabile kakhulu. Malunga nomntu omnye kumakhulu amabini amawaka ufunyaniswa enale sifo.
• Uhlobo oluphambili lwale nto yi -'classical RDD' . Oku kuxa ii-lymph nodes zakho (ii-glands) zivuvukala.
• Olunye uhlobo yi -'extranodal RDD' . Kule meko, amalungu angaphandle kwee-lymph nodes ayachaphazeleka. Oku kuthetha ukuba iiseli ze-histiocyte ezigqithisileyo zinokuqokelelana eluswini, emathanjeni, okanye kwezinye iindawo.

Ingaba esi sifo sibizwa ngokuba yiHistiocytosis sinqabile kangakanani?

Ewe, njengoko benditshilo ngaphambili, i-histiocytosis sisifo esingaqhelekanga . Enyanisweni, iintlobo zaso ezinobundlongondlongo nezinomhlaza zingaphantsi kwe-1% yazo zonke iintlobo zomhlaza ezifunyaniswe kwizicubu ezithambileyo kunye nee-lymph nodes. Ngoko ungacinga ukuba oku kunqabile kangakanani.

Zithini iimpawu ze-Histiocytosis?

Ngoku makhe sibone ukuba zeziphi iimpawu eziboniswayo kule meko ye-histiocytosis. Ezi mpawu zinokuhluka ukusuka komnye umntu ukuya komnye . Isizathu soku kukuba amalungu kunye nezicubu apho iiseli ze-histiocyte ezigqithisileyo ziqokelelana khona, njengoko benditshilo, kunye nobungakanani bomonakalo kwezo zicubu, kuyayichaphazela le nto.

Nazi ezinye zeempawu onokuzibona (kwaye zisenokuba zikhona nezinye):

• Iimpuku zesikhumba : Ezi zinokubonakala ngakumbi kwiindawo ezinoboya entlokweni yeentsana ezincinci (sizibiza ngokuba yi-'cradle cap' ) okanye kwindawo ye-nappy.
• Ifiva .
• Ukudinwa, ukudinwa .
• Intloko ebuhlungu.
• Ukukhohlela okanye ukuphefumla kancinci .
• Ukunciphisa umzimba ngendlela engachazekiyo .
• Intlungu yamathambo .
• Ii-lymph nodes ezivuvukileyo (oku kuthetha ukudumba).
• Ukutshintsha kombono okanye ukudumba kwamehlo .
• Ukuchama rhoqo kunye nokunxanwa kakhulu .
• Amaqhuma eluswini (angabonakala nakwiinkophe).
• Ubunzima bokugxila okanye ukukhumbula izinto .
• Iingxaki ngokulinganisela kunye nokulungelelanisa .

Zivela njani ezi mpawu? Kuxhomekeke ekubeni ii-histiocytes ziqokelelana phi!

Ezi mpawu zixhomekeke ekubeni ziqokelelana phi emzimbeni iiseli ze-histiocyte. Iindawo eziphambili zokuqokelelana ziyahluka ngokuxhomekeke kuhlobo lwe-histiocytosis.

Iindawo ezinokuchaphazeleka kwi-Langerhans cell histiocytosis (LCH):

• Ithambo
• Ulusu
• Ii-lymph nodes (ukudumba)
• Isibindi
• Ispleen
• Umlomo
• Imiphunga
• Inkqubo yemithambo-luvo ephakathi (oko kukuthi, ingqondo kunye nomqolo)

Iindawo ezinokuchaphazeleka kwisifo se-Erdheim-Chester (ECD):

• Amathambo amade (ingakumbi emilenzeni)
• Amehlo
• Inkqubo ye-nervous ephakathi
• Imiphunga
• Intliziyo
• Ivazi yegazi
• Izintso
• Izitho ezingasemva kwesisu (sizibiza ngokuba yi-'retroperitoneum' )

Iindawo ezinokuchaphazeleka sisifo iRosai-Dorfman (RDD):

• Ii-lymph nodes (ingakumbi entanyeni, kodwa zinokubakho nakwezinye iindawo)
• Ulusu
• Ithishu ethambileyo
• Indlela yokuphefumla ephezulu
• Ithambo
• I-Retroperitoneum (amalungu angasemva kwesisu)
• Amehlo

Yintoni ebangela i-Histiocytosis?

Izazinzulu azikayazi kakuhle into ebangela i-histiocytosis, kodwa zifumanise ukuba isenokuba inxulunyaniswa notshintsho lwemfuza .

Ngamafutshane, yonke iseli emzimbeni wethu inezakhi zofuzo, okanye iseti yemiyalelo. Le miyalelo imisela indlela ezo seli ezimele ziziphathe ngayo. Ngoko ke, xa utshintsho kwezi miyalelo, olubizwa ngokuba ziinguqu, lwenzeka, iiseli ziqala ukuziphatha ngendlela engaqhelekanga. Umzekelo, utshintsho lwezakhi zofuzo lunokubangela ukuba iseli efana ne-histiocyte izikope ize iqale ukusasazeka ngokungalawulekiyo.

Ukuchonga ezi nguqu zemfuza kuye kwavumela abaphandi ukuba baphuhlise iindlela ezintsha zonyango lwe-histiocytosis. Ezi ndlela zonyango zisebenza ngokuthintela utshintsho oluyingozi lweeseli oluvumela iiseli ze-histiocyte ukuba zikhule zingalawuleki.

Uyifumanisa njani i-histiocytosis?

Ukuba ukrokrela ukuba une-histiocytosis, ugqirha wakho uza kuqala enze uvavanyo lomzimba aze akubuze ngembali yakho yezonyango. Emva koko, uza kugqiba ukuba zeziphi iimvavanyo ekufuneka zenziwe ngokusekelwe kwiimpawu zakho kunye namalungu acingelwa ukuba achatshazelwe yi-histiocytes egqithisileyo.

Nazi ezinye iimvavanyo onokuzenza:

• Iinkqubo zokuthatha imifanekiso :
• Ugqirha wakho angayalela i-scan ekhethekileyo ebizwa ngokuba yi-FDG PET/CT scan . Oku kunokufumanisa izinto ezifana neethumba emzimbeni wonke.
• Ukongeza , iimvavanyo ezinje nge-CT scan, i-MRI scan, i-bone scan, i-ultrasound scan, i-X-ray, okanye i-echocardiogram (i-scan yentliziyo) zinokufuneka.

• Uvavanyo lwelebhu :
• Ugqirha wakho uza kuvavanya iisampulu zolwelo, njengegazi okanye umchamo wakho, ukuze abone ukuba kukho na iimpawu ze-histiocytosis.
• Inani leeseli zakho zegazi liyajongwa. Bakwakhangela iihomoni okanye iiproteni ezithile ezibonisa indlela amalungu akho asebenza ngayo. Ukuba ilungu alisebenzi kakuhle, oko kungabonisa ukuba kukho ii-histiocytes ezininzi kakhulu kuloo malungu.

• Uvavanyo lwe-Biopsy `(i-Biopsy)` :
• Oku kwenziwa kukuba ugqirha athathe isampuli yethishu aze ayihlole phantsi kwe-microscope ukuze abone ukuba unayo na i-histiocytosis.
• Ikwajonga utshintsho oluthile lwezakhi zofuzo oluhambelana ne-histiocytosis.
• Ngamanye amaxesha, ukuba i-biopsy ayinakwenzeka, izinto zemfuza ezisegazini lakho zinokuvavanywa. Oku kubizwa ngokuba yi-'liquid biopsy' .

Ziziphi iindlela zonyango lwe-Histiocytosis?

Unyango lwe-histiocytosis luxhomekeke kwizinto ezininzi . Ngokukodwa:

• Indlela ezibukhali ngayo iimpawu zakho .
• Nokuba unesilonda esinye okanye ezininzi (ezi ziindawo zezicubu ezingaqhelekanga).
• Ziphi ezi 'zilonda' emzimbeni ?
• Nokuba la maqhuqhuva anomhlaza okanye akunjalo .

Oogqirha bagqiba ngonyango olusekelwe kwezi zinto. Nazi ezinye iindlela zonyango:

• Bukela kwaye ulinde : Ukuba i-histiocytosis yakho ayinzima kwaye awunazo naziphi na iimpawu ezinkulu, ugqirha wakho unokukuxelela ukuba ujonge imeko yakho.
• UtyandoUkuba iiseli ze-histiocyte ziqokelelene kwinxalenye enye yomzimba kuphela, utyando lunokwenziwa ukuze kususwe loo nxalenye.
• Unyango ngemitha : Oku kuquka ukusebenzisa umatshini ukuqondisa imisebe yemitha enamandla aphezulu kwizilonda. Le mitha ibulala iiseli ezingaqhelekanga kwaye icothise ukukhula kweethumba. Ngamanye amaxesha unyango ngemitha lunokunceda ekunciphiseni iimpawu.
• Unyango lweKhemotherapy (olubizwa ngokuba yi-'chemo') : Oku kuquka ukuthumela iikhemikhali egazini lakho ukuze zitshabalalise iiseli ze-histiocyte ezigqithisileyo ezisasazeke emzimbeni wakho. Oogqirha bacebisa kuphela i-'chemo' ukuba iiseli ze-histiocyte zisasazeke emzimbeni wakho.
• Ii-Corticosteroids : Amayeza afana ne -prednisone awela kolu didi. La mayeza anokunciphisa ukudumba (ukudumba) okuhambisana ne-histiocytosis. La mayeza anokunikwa eyedwa okanye edibene nezinye iindlela zonyango, ezifana ne-chemotherapy.
• Unyango lwe-Immunotherapy : Oku kusebenza ngokuqinisa inkqubo yakho yomzimba yokuzikhusela, ukuyinceda ukuba ibone kwaye itshabalalise iiseli eziyingozi, ezifana ne-histiocytes ezingaqhelekanga.
• Unyango olujoliswe kulo : Olu lunyango olukhethekileyo. Olu nyango lusetyenziselwa iimeko ze-histiocytosis ezinxulumene notshintsho oluthile lwemfuza.

Ngaba i-Histiocytosis inganyangeka ngokupheleleyo?

Kunzima ukuthi 'ewe' okanye 'hayi' koku, kuba kuxhomekeke kwimeko. Abantu abaninzi abane-histiocytosis bangena kwinto ebizwa ngokuba yi -'durable remission'. Oko kukuthi, bayahamba bengenazo iimpawu okanye iimpawu zesifo.

Ezinye iintlobo ze-histiocytosis, ingakumbi xa ii-histiocytes zikwindawo enye emzimbeni, zinokunyangwa ngokupheleleyo ngotyando . Ugqirha wakho uza kukunika utyelelo olulandelayo ukuze ajonge ukuba aziphinde zenzeke na ezi zifo.

Nangona kunjalo, ukuba uhlobo lwe-histiocytosis onalo alunyangeki ngokupheleleyo, ugqirha wakho uya kukucebisa isicwangciso sonyango esinokukunceda ulawule le meko.

Ithini ingqikelelo yale meko?

Uqikelelo lwemeko yakho luxhomekeke kwizinto ezininzi. Kwabanye abantu, i-histiocytosis iba ngcono ngokwayo ngaphandle konyango . Umzekelo, malunga nama-40% abantu abane-RDD baba ngcono ngaphandle konyango. Kwakhona, xa i-LCH ichaphazela inxalenye enye yomzimba kuphela (esiyibiza ngokuba yi -'localized LCH') , idla ngokuba ngcono ngokwayo.

Kodwa, kwezinye iimeko, i-histiocytosis ifuna unyango olunzulu kunye nokujonga ngenyameko, kubantwana nakubantu abadala.Kwiimeko ezinzima ngakumbi, i-histiocytosis inokubangela umonakalo kwizitho zomzimba kwaye ide ibe yingozi ebomini .

Kungoko ke, kungcono ukubuza ugqirha wakho malunga nemeko ethile echaphazela ukuxilongwa kwakho.

Ngaba i-Histiocytosis ingathintelwa? Ingancitshiswa njani ingozi?

Inyaniso kukuba, akukho ndlela yokuthintela i-histiocytosis, kodwa unyango lunokukunceda ulawule iimpawu zakho.

Kodwa kukho into enye. Ukuyeka ukutshaya kunokunciphisa umngcipheko wokuba ne-LCH (i-Langerhans cell histiocytosis), umhlaza wemiphunga. Ukuyeka ukutshaya okanye ukuhlala ungatshayi kunokuphucula indlela osabela ngayo kunyango. Ukuba ufuna uncedo lokuyeka ukutshaya, buza ugqirha wakho malunga neenkqubo zokudlulisela ukuyeka ukutshaya.

Ndingayibuza yiphi imibuzo ugqirha wam?

Ukuba ufunyaniswe une-histiocytosis, kuyinto eqhelekileyo ukuba nemibuzo emininzi engqondweni yakho. Kubalulekile ukuthetha nogqirha wakho ngayo yonke le nto kwaye ufumane ukuqonda kakuhle imeko yakho. Nazi ezinye zemibuzo onokuyibuza:

• Luhlobo luni lwe-histiocytosis endinalo?
• Ngaba ndiza kufuna unyango?
• Zeziphi iindlela zonyango ozicebisayo?
• Zeziphi iziphumo ebezingalindelekanga ezinokulindelwa ngexesha lonyango?
• Zithini iziphumo ezinokubakho zonyango?
• Ingaba imeko yam inganyangeka ngokupheleleyo?

Umyalezo Wokugqibela Wokuya Ekhaya

Ngamafutshane, i-histiocytosis ligama elinikwa iqela leengxaki zegazi ezingaqhelekanga ezibangelwa kukuveliswa kakhulu kwee-histiocytes, uhlobo lweeseli ezimhlophe zegazi, kwizicubu. Ezinye zezi zinokuba lula kakhulu, zingabonakali zimpawu, ngelixa ezinye zinokuba yingozi ngokwaneleyo ukuba zibeke ubomi esichengeni .

Wonke umntu one-histiocytosis une-histiocytes ezininzi kwizicwili zakhe, nto leyo ebangela ukudumba (ukudumba). Nangona kunjalo, iimpawu, unyango, kunye nesiphumo sesifo zinokwahluka kakhulu, kwanaphakathi kwabantu abane-histiocytosis efanayo .

Ugqirha wakho ngumntu ongcono onokukuqonda ukuxilongwa kwakho kunye neemeko ezikhethekileyo ezikujikelezileyo. Ngoko ke, ukuze uqonde ngokupheleleyo ukuxilongwa kwakho kunye nesicwangciso sakho sonyango, ungaze woyike ukubuza imibuzo. Thetha nogqirha wakho ngayo yonke into esengqondweni yakho.

I- Histiocytosis, Iiseli zegazi ezimhlophe, ii-Histiocytes, Inkqubo yomzimba yokuzikhusela, i-LCH, i-ECD, i-RDD, Iimpawu, Unyango