Ingabe ingane yakho ike yaba neqhubu elincane endaweni ethile emzimbeni wayo? Noma ukuqubuka kwesikhumba noma ukuqubuka okulumayo okungeke kuphole? Ngezinye izikhathi lokhu kujwayelekile, kodwa akuvamile ukuba kube izimpawu zesimo esingavamile esibizwa ngokuthi i-Langerhans Cell Histiocytosis (LCH). Kuvamile ukuzizwa wesaba uma uzwa leli gama, ngoba akuyona into esijwayele ukuyizwa. Kodwa ungakhathazeki, ake sixoxe ngayo ngamazwi alula.
Kalula nje, iyini i-LCH?
Cabanga ngemizimba yethu njengezwe elikhulu. Kukhona ibutho lokuvikela leli lizwe, futhi yilo isimiso sethu somzimba sokuzivikela . Uhlobo olukhethekile lwesosha kuleli butho lubizwa ngamaseli e-Langerhans . Lawa maseli awuhlobo lweseli elimhlophe legazi. Umsebenzi wawo oyinhloko ukulwa namagciwane kanye nokutheleleka okungena emizimbeni yethu futhi kusivikele ezifweni. Lawa masosha atholakala kuwo wonke umzimba wethu, ikakhulukazi esikhumbeni, emaphashini, kuma-lymph node, emnkantsheni, espleen nasesibindini.
Kodwa uma kwenzeka i-LCH, la maseli e-Langerhans ayanda ngokungalawuleki futhi aqoqana ezingxenyeni ezahlukene zomzimba. Kufana nomhlambi wamasosha omi nje endaweni eyodwa ngaphandle kokulwa. Uma eqoqana kanje, aqala ukulimaza izicubu ezinempilo kulezo zindawo. Kuvela izilonda kulezo zindawo.
Izindaba ezinhle ngalesi simo ukuthi izingane eziningi zingalulama ngokuphelele kulesi sifo ngokwelashwa okufanele. Ngezinye izikhathi, ikakhulukazi uma kuthinta isikhumba kuphela, singase siphele sodwa ngaphandle kokwelashwa. Kodwa-ke, uma la maseli ethinta izitho ezibalulekile njengomongo wethambo, ubende noma isibindi, kungadingeka ukwelashwa okunamandla kakhulu.
Ingabe umdlavuza we-LCH?
Lona umbuzo abantu abaningi abanawo. Eqinisweni, kusekhona ukungavumelani phakathi kodokotela ngalokhu. Abacwaningi abaningi babheka i-LCH njengesimo esifana nomdlavuza, okungukuthi, i-neoplasm . Okungukuthi, ukukhula okungavamile kwamaseli. Kodwa abanye bacabanga ukuthi yisifo sokuvuvukala kwesimiso somzimba sokuzivikela.
Kodwa-ke, i-LCH yelashwa odokotela bomdlavuza, abangochwepheshe bomdlavuza nezifo zegazi. Ngezinye izikhathi, ukwelashwa okulwa nomdlavuza njenge -chemotherapy nakho kuyasetshenziswa ukuyilapha.
Ubani onamathuba amaningi okuthola lesi sifo?
I-LCH ivame ukubonakala ezinsaneni ezisanda kuzalwa nasezinganeni eziphakathi kweminyaka engu-1 no-15. Abantu abadala bangavamile kakhulu, kodwa akuyona into engenakwenzeka.
Ngokwezibalo, yingane eyodwa noma ezimbili kuphela kweziyisigidi ezizalwa zinalesi sifo minyaka yonke. Lokhu kusho ukuthi lesi yisimo esingavamile kakhulu .
Ziyini izimpawu ze-LCH?
I-LCH ayithinti yonke ingane ngendlela efanayo. Nakuba ezinye izingane zingase zibe nendawo eyodwa kuphela yomzimba wazo ethintekile, ezinye zingase zibe nezindawo eziningi zomzimba wazo ezithintekile. Ngakho-ke, izimpawu ziyahlukahluka kuye ngokuthi ingxenye yomzimba ithintekile. Ake sibheke ukuthi ziyini.
| Ingxenye yomzimba ethintekile | Izimpawu ezingabonakala |
|---|---|
| Amathambo | I-LCH ithinta amathambo cishe ku-80% weziguli. Iqhubu noma ukuvuvukala kungavela ezindaweni ezifana nogebhezi, amathambo azungeze amehlo, ngemuva kwezindlebe, umhlathi, izitho, umgogodla kanye nezinqe. Ubuhlungu bungaba khona noma bungabi khona. Ngaphezu kwalokho, ikhanda elibuhlungu, ubuhlungu bentamo/umhlane, ubunzima bokuhamba, kanye nokuxhuga kungabonakala. |
| Isikhumba | Ukuvuvukala okuvame kakhulu ukuvuvukala kwesikhumba. Isembozo sengane singaba yisimo esingapholi esibonakala ekhanda lomntwana. Amabala abomvu, anamabala angavela embotsheni, emakhwapheni, esiswini, esifubeni nasemhlane. Lokhu kungase kuphume futhi kulume noma kube buhlungu. Izinzipho zingase zishintshe umbala noma ziwe. |
| Umlomo | Amazinyo axegayo noma axegayo, amazinyo anciphayo, izinsini ezivuvukele, izilonda ezindebeni, olimini, ngaphakathi ezihlathini, noma ophahleni lomlomo. |
| Isibindi noma i-spleen | Ukwanda kwesibindi noma ubende kungabangela ukuvuvukala kwesisu, ukuphuzi kwamehlo nesikhumba (i-jaundice), ukulunywa, ukukhathala okukhulu, kanye nokulunywa noma ukuphuma kwegazi kalula. |
| Umkantsha | Ukuntuleka kwegazi emzimbeni, ukuphaphatheka, ukukhathala, kanye nokulahlekelwa yisifiso sokudla ngenxa yokwehla kwamaseli abomvu egazi. Ukutheleleka njalo kanye nomkhuhlane ngenxa yokwehla kwamaseli amhlophe egazi. Ukuphuka okulula ngenxa yokwehla kwama-platelet. |
| Uhlelo Lwe-Endocrine (Uhlelo Lwe-Endocrine - AmaHormone) | Uma indlala ye-pituitary ithintekile: ukoma ngokweqile kanye nokuchama njalo (isifo sikashukela insipidus), ukukhula okubambezelekile, ukuthomba kusenesikhathi noma ukubambezeleka, ukukhuluphala. Uma indlala ye-thyroid ithintekile: ukuvuvukala kwendlala, ubunzima bokuphefumula. |
| Izindlebe | Ukutheleleka njalo kwendlebe, ukuphuma koketshezi endlebeni, ukubomvu kwendlebe, ukulunywa endlebeni, ubuhlungu bendlebe, kanye nokulahlekelwa ukuzwa. |
| Amehlo | Amehlo avuvukele, ukuvuvukala ngaphezu kwamehlo, ukukhubazeka kokubona. |
| Ama-Lymph Node | Ukuvuvukala nobuhlungu emaqhubu (amaqhubu) entanyeni, emakhwapheni, noma embotsheni. |
| Isistimu Yezinzwa Ephakathi | Ikhanda elibuhlungu, isiyezi, ukuhlanza, ubunzima bokuhamba, ukulahlekelwa yibhalansi (i-ataxia), ubunzima bokukhuluma noma bokubona, ukuxhuzula, izinguquko ekuziphatheni nasekukhumbuleni. |
| Amaphaphu | Lokhu kuvamile kubantu abadala, ikakhulukazi ababhemayo. Ubuhlungu besifuba, ukukhwehlela okomile, ubunzima bokuphefumula, ukuwa kwamaphaphu (i-pneumothorax). |
| Indlela Yokugaya Isisu | Ubuhlungu besisu, ukuhlanza, isifo sohudo, igazi esitulweni, kanye nokukhula kabi ngenxa yokushoda kokudla okunempilo. |
Into ebalulekile ukuthi lezi zimpawu zingabonakala nakwezinye izifo eziningi ezivamile. Ngakho-ke ungesabi i-LCH ngoba nje unesinye noma ezimbili zalezi zimpawu. Kodwa uma lezi zimpawu ziqhubeka, kubaluleke kakhulu ukubona udokotela bese uthola ukuxilongwa okufanele.
Iyini imbangela eqondile ye-LCH?
Kalula nje, amangqamuzana ethu anezakhi zofuzo eziwatshela ukuthi "ahlukane manje, yeka manje." Cishe ingxenye yezingane ezine-LCH inoshintsho oluncane, noma ukuguquka, ku-gene yazo ebizwa ngokuthi 'i-BRAF .' Le gene yenza iphrotheni elawula ukukhula kwamangqamuzana. Ngenxa yalokhu kuguquka, leyo phrotheni ayitholi umyalo "wokuyeka ukwahlukana." Kufana nokuthi inkinobho ethi 'on' inamathele. Ngakho-ke amangqamuzana e-Langerhans ayaqhubeka nokwahlukana futhi ehlangana.
Lokhu akuyona into ezuzwa ngabazali ezinganeni. Lokhu kuguquka kwezakhi zofuzo kwenzeka ngokungahleliwe ngemva kokuba ingane ikhulelwe esibelethweni sikanina.
Ngaphezu kwezakhi zofuzo ze-'BRAF', abacwaningi bathole ukuthi lesi sifo singabangelwa futhi izinguquko kwezinye izakhi zofuzo ezifana ne-'MAP2K1`, `RAS`, kanye ne-'ARAF`.
Ukuthola kanjani lokhu, Dokotela?
Uma uyisa ingane yakho kudokotela, izoqala ikubuze ngezimpawu zengane yakho. Ngemuva kwalokho, izohlola ingane yakho ngokucophelela. Ngenxa yokuthi i-LCH ingathinta izingxenye eziningi zomzimba, kungadingeka ukuhlolwa okuningana ukuqinisekisa ukuxilongwa.
| Uhlobo lokuhlola | Kalula nje... |
|---|---|
| Ukuhlolwa Kwegazi | Ukubalwa kwegazi okuphelele (i-CBC) kulinganisa inani lamaseli abomvu, amaseli amhlophe, nama-platelet egazini. Ukuhlolwa kwegazi kwenziwa futhi ukuhlola izinto ezifana nokusebenza kwesibindi. |
| Ukuhlolwa kwebhayoloji | Lena yindlela ecacile kakhulu yokuqinisekisa i-LCH.Kuthathwa ingxenye encane yezicubu esigaxeni noma esilondeni ngaphansi kwe-anesthesia bese kuhlolwa ngaphansi kwe-microscope ukuqinisekisa ukuthi akhona yini amaseli e-LCH. Uma kukhona okusolakala ukuthi kukhona umnkantsha, i-bone marrow biopsy nayo ingenziwa. |
| Ukuhlolwa Kwezakhi Zofuzo | Isampula yezicubu ezithathwe ku-biopsy isetshenziselwa ukuhlola ukuguquka kwezakhi zofuzo ze-'BRAF'. |
| Ukuhlolwa Kwezithombe | Ukuhlolwa okufana ne-X-ray, i-CT scan, i-MRI scan, kanye ne-PET scan kunganquma izinga lemiphumela ye-LCH ezithweni zangaphakathi njengamathambo, ubuchopho namaphaphu. |
Ngokusekelwe emiphumeleni yalezi zivivinyo, udokotela wakho uzodlulisela ingane yakho kudokotela wezifo zegazi/umdlavuza wezingane.
Yiziphi izindlela zokwelapha i-LCH?
Akuzona zonke izingane ezine-LCH ezidinga uhlobo olufanayo lokwelashwa. Ukwelashwa kuncike endaweni kanye nobunzima besifo.
Odokotela bahlukanisa i-LCH ngezinhlobo ezimbili eziyinhloko:
1. I-LCH yohlelo olulodwa: Lesi sifo sithinta uhlelo olulodwa lwesitho emzimbeni (isib., amathambo kuphela noma isikhumba kuphela).
2. I-LCH enezinhlelo eziningi: Lesi sifo sithinta izinhlelo zomzimba ezimbili noma ngaphezulu (isib. isikhumba nesibindi).
Futhi, izitho ezifana nesibindi, udebe, kanye nomnkantsha zibhekwa njengezitho "eziyingozi kakhulu", kuyilapho izitho ezifana nesikhumba, amathambo, namaphaphu zibhekwa "njengezingozi ezincane." Lokhu kuhlukaniswa kunquma uhlelo lokwelashwa.
Kunezindlela eziningana zokwelapha eziyinhloko:
- Ukwelashwa ngama-steroid: Ikakhulukazi uma isikhumba kuphela sithintekile, imithi yama-steroid efana ne-prednisone isetshenziswa njengamaphilisi noma amafutha okugcoba.
- Ukuhlinzwa: Ukuhlinzwa okufana ne -curettage kwenziwa ukuze kususwe isimila se-LCH ethanjeni.
- Ukwelashwa ngamakhemikhali: Lolu uhlobo lomuthi olunikezwa ukubulala amangqamuzana omdlavuza. Ngenxa yokuthi amangqamuzana e-LCH ahlukana ngokushesha, le mithi iyawavimba ekukhuleni. Le mithi inganikezwa njengamaphilisi, imijovo, noma njengokhilimu osetshenziswa esikhumbeni.
- Ukwelashwa ngemisebe: Kusebenzisa imisebe enamandla aphezulu ukubhubhisa amaseli e-LCH. Lokhu manje akusasetshenziswa kakhulu.
- Ukwelashwa okuqondiwe:Ezinganeni ezine-'BRAF' gene mutation, kunemithi (i-BRAF inhibitors) ehlose lokho kuguqulwa. Le mithi ayinawo umonakalo omkhulu kumaseli anempilo.
- Ukwelashwa Ngezifo Ezingalawuleki: Ukuvuselela amasosha omzimba engane ukuze alwe namaseli e-LCH.
- Ukufakelwa kwe-stem cell: Inketho yokwelapha ecatshangelwa ezimweni ezinzima kakhulu ezingamelani nezinye izindlela zokwelapha.
Sinjani isimo ngemva kokwelashwa?
Ukubikezela kwe-LCH kuncike ezintweni eziningana, okuhlanganisa ukuthi yiziphi izingxenye zomzimba lesi sifo esithintekile, ukuthi sesisakazeke kangakanani, nokuthi sisabela kahle kangakanani ekwelashweni.
- Izinga lokuphulukiswa kwezingane ezinezitho "ezinengozi encane" kuphela (zombili uhlelo olulodwa kanye nohlelo oluningi) licishe libe yi-100% . Lokhu kusho ukuthi akukho bungozi bokufa. Kodwa-ke, kunengozi yokuphinda kwenzeke noma ezinye izinkinga zesikhathi eside.
- Isimo sezingane ezinezitho "ezisengozini enkulu", njengesibindi, ubende, kanye nomnkantsha, singase sibe sibi kakhulu.
Ngakho-ke, kubaluleke kakhulu ukulandela udokotela wakho iminyaka eminingi, ngisho nangemva kokwelashwa. Udinga ukuhlolwa njalo ukuthi unesifo esibuye sibuye yini.
Umlayezo Wokuya Nawe Ekhaya
- I-Langerhans Cell Histiocytosis (LCH) yisifo esingavamile esibangelwa ukukhula okungalawuleki kohlobo lweseli lokuzivikela komzimba. Sivame ukubonakala ezinganeni.
- Nakuba lokhu kungabhekwa njengomdlavuza, odokotela bezifo zomdlavuza bayakwelapha ngokusebenzisa izindlela zokwelapha umdlavuza.
- Izimpawu (izilonda zesikhumba, amaqhubu ethambo, ukutheleleka okuvamile) ziyahlukahluka kakhulu kuye ngokuthi ingxenye yomzimba ethintekile yilesi sifo.
- I-biopsy ibalulekile ukuze kutholakale ukuthi lesi sifo sikhona ngempela.
- Ezinganeni eziningi, ikakhulukazi lezo ezisesigabeni "esisengozini encane", ukwelashwa kungasiqeda ngokuphelele lesi sifo.
- Ngisho nangemva kokuba ukwelashwa sekuqediwe, kubaluleke kakhulu ukuba nokuhlolwa kwezokwelapha iminyaka eminingana ukuze kubonakale ukuthi lesi sifo siyaphinda yini.
- Xoxani nganoma yimiphi imibuzo noma ukukhathazeka onakho ngesimo sengane yakho ngokukhululekile nodokotela wakho.
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