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Unyango lweLangerhans Cell Histiocytosis (®) –uPawu lwePatient

Ulwazi ngokubanzi malunga neLangerhans Cell Histiocytosis (LCH)

IINGONGOMA EZIPHAMBILI

  • I-Langerhans cell histiocytosis luhlobo lomhlaza olunokonakalisa izicwili okanye lubangele ukuba izilonda zenze indawo enye okanye nangaphezulu emzimbeni.
  • Imbali yosapho yomhlaza okanye ukuba nomzali ovezwe kwiikhemikhali ezithile kunokunyusa umngcipheko we-LCH.
  • Iimpawu kunye neempawu ze-LCH zixhomekeke apho ikhoyo emzimbeni.
  • Ulusu kunye neenzipho
  • Umlomo
  • Ithambo
  • Iimpawu ze-lymph kunye ne-thymus
  • Inkqubo ye-Endocrine
  • Iliso
  • Inkqubo ye-nervous central (CNS)
  • Isibindi kunye nodaka
  • Imiphunga
  • Ummongo wethambo
  • Uvavanyo oluvavanya amalungu kunye neenkqubo zomzimba apho i-LCH inokwenzeka khona zisetyenziselwa ukufumanisa i-LCH.
  • Izinto ezithile zichaphazela ukuxela kwangaphambili (ithuba lokufumana kwakhona) kunye nokhetho lonyango.

I-Langerhans cell histiocytosis luhlobo lomhlaza olunokonakalisa izicwili okanye lubangele ukuba izilonda zenze indawo enye okanye nangaphezulu emzimbeni.

ILangerhans cell histiocytosis (LCH) ngumhlaza onqabileyo oqala kwiiseli ze-LCH. Iiseli ze-LCH luhlobo lweseli ye-dendritic elwa nosulelo. Ngamanye amaxesha kukho utshintsho (utshintsho) kwiiseli ze-LCH njengoko zisenzeka. Oku kubandakanya utshintsho kwi-BRAF, MAP2K1, RAS kunye neARAF genes. Olu tshintsho lunokwenza iiseli ze-LCH zikhule kwaye zande ngokukhawuleza. Oku kubangela ukuba iiseli ze-LCH zakheke kwiindawo ezithile zomzimba, apho zinokonakalisa izicubu okanye zenze izilonda.

I-LCH ayisosifo seeseli zeLangerhans eziqhelekileyo ezivela kulusu.

I-LCH inokwenzeka nakweyiphi na iminyaka, kodwa ixhaphake kakhulu kubantwana abancinci. Unyango lwe-LCH ebantwaneni lwahlukile kunyango lwe-LCH kubantu abadala. Unyango lwe-LCH ebantwaneni kunye nonyango lwe-LCH kubantu abadala luchazwe kumacandelo ahlukeneyo esi sishwankathelo.

Imbali yosapho yomhlaza okanye ukuba nomzali ovezwe kwiikhemikhali ezithile kunokunyusa umngcipheko we-LCH.

Nantoni na eyandisa umngcipheko wokufumana isifo ibizwa ngokuba yingozi. Ukuba nomngcipheko akuthethi ukuba uza kuba nomhlaza; ukungabinamngcipheko oko akuthethi ukuba awuzukufumana umhlaza. Thetha nogqirha wakho ukuba ucinga ukuba usengozini.

Imiba yomngcipheko kwi-LCH ibandakanya oku kulandelayo:

  • Ukuba nomzali oye wachaphazeleka kwiikhemikhali ezithile.
  • Ukuba nomzali owayechaphazeleke kwisinyithi, igranite, okanye uthuli lomthi kwindawo yokusebenza.
  • Imbali yosapho yomhlaza, kubandakanya i-LCH.
  • Ukuba nembali yobuqu okanye imbali yosapho yesifo se-thyroid.
  • Ukuba nosulelo njengosana olusanda kuzalwa.
  • Ukutshaya, ngakumbi kubantu abadala abancinci.
  • Ukuba yiSpanishi.
  • Ukungagonywa njengomntwana.

Iimpawu kunye neempawu ze-LCH zixhomekeke apho ikhoyo emzimbeni.

Ezi kunye neminye imiqondiso kunye neempawu zinokubangelwa yi-LCH okanye ngezinye iimeko. Jonga ugqirha wakho ukuba wena okanye umntwana wakho unazo ezi zinto zilandelayo:

Ulusu kunye neenzipho

I-LCH kwiintsana inokuchaphazela ulusu kuphela. Kwezinye iimeko, i-LCH yolusu kuphela inokuba mandundu ngaphezulu kweeveki okanye iinyanga kwaye ibe yifom ebizwa ngokuba ngumngcipheko omkhulu we-LCH.

Kwiintsana, iimpawu okanye iimpawu ze-LCH ezichaphazela ulusu zingabandakanya:

  • Ukuntywizisa kwentloko enokhakhayi enokubonakala ngathi "kukuzala komntwana".
  • Ukuntywila kumanqanaba omzimba, anjenge-elbow yangaphakathi okanye i-perineum.
  • Ukuphakama kwesikhumba esikhulileyo, esimdaka okanye esimfusa naphina emzimbeni

Kubantwana nakubantu abadala, iimpawu okanye iimpawu ze-LCH ezichaphazela ulusu kunye nezipikili kunokubandakanya:

  • Ukuntywila entlonzeni okunokubonakala ngathi kukubhabha.
  • Ukuphakanyiswa, obomvu okanye obomdaka, ukuqubuka okukrakra kwindawo ye-groin, isisu, umqolo, okanye isifuba, esinokubangela okanye esibuhlungu.
  • Amaqhuma okanye izilonda entlonzeni.
  • Izilonda emva kweendlebe, phantsi kwamabele, okanye kummandla we-groin.
  • Iinzipho zeminwe eziwayo okanye ezimenyezelayo zemijelo eziwela isikhonkwane.

Umlomo

Iimpawu okanye iimpawu ze-LCH ezichaphazela umlomo zinokubandakanya:

  • Iintsini ezidumbileyo.
  • Izilonda kuphahla lomlomo, ngaphakathi kwezidlele, okanye kulwimi okanye emilebeni.

Amazinyo angalingani okanye awe.

Ithambo

Iimpawu okanye iimpawu ze-LCH ezichaphazela ithambo zingabandakanya:

  • Ukudumba okanye iqhuma phezu kwethambo, njengokhakhayi, umhlathi, iimbambo, isinqe, umqolo, ithambo lamathanga, ithambo lengalo ephezulu, ingqiniba, isokethi yamehlo, okanye amathambo ajikeleze indlebe.
  • Ubuhlungu apho kukho ukudumba okanye iqhuma ngaphezulu kwethambo.

Abantwana abanezilonda ze-LCH emathanjeni ajikeleze iindlebe okanye amehlo banomngcipheko omkhulu wesifo seswekile i-insipidus kunye nezinye izifo zenkqubo ye-neva.

Iimpawu ze-lymph kunye ne-thymus

Iimpawu okanye iimpawu ze-LCH ezichaphazela ii-lymph node okanye i-thymus zingabandakanya:

  • I-lymph nodes ezidumbileyo.
  • Ingxaki yokuphefumla.
  • Superior vena cava syndrome. Oku kunokubangela ukukhwehlela, ingxaki yokuphefumla, kunye nokudumba kobuso, intamo kunye neengalo ezingaphezulu.

Inkqubo ye-Endocrine

Iimpawu okanye iimpawu ze-LCH ezichaphazela i-pituitary gland inokubandakanya:

  • Isifo seswekile. Oku kunokubangela unxano olomeleleyo kunye nokuchama rhoqo.
  • Ukukhula kancinci.
  • Ukufikisa kwasekuqaleni okanye emva kwexesha.
  • Ukutyeba kakhulu.

Iimpawu okanye iimpawu ze-LCH ezichaphazela i-thyroid zingabandakanya:

  • Ukudumba idlala lengqula.
  • IHypothyroidism. Oku kunokubangela ukudinwa, ukunqongophala kwamandla, ukukhathalela ukubanda, ukuqhina, ulusu olomileyo, iinwele ezicothayo, iingxaki zememori, ingxaki yokugxila, kunye noxinzelelo. Kwiintsana, oku kunokubangela ukulahleka kwesidlo kunye nokukrakra ekudleni. Kubantwana nakwishumi elivisayo, oku kunokubangela iingxaki zokuziphatha, ukutyeba, ukukhula kancinci kunye nokufikisa emva kwexesha.
  • Ingxaki yokuphefumla.

Iliso

Iimpawu okanye iimpawu ze-LCH ezichaphazela iso zinokubandakanya:

  • Iingxaki zombono.

Inkqubo ye-nervous central (CNS)

Iimpawu okanye iimpawu ze-LCH ezichaphazela i-CNS (ingqondo kunye nentambo yomqolo) inokubandakanya:

  • Ukuphulukana nokulingana, ukungahambelani komzimba kunye nengxaki yokuhamba.
  • Ingxaki yokuthetha.
  • Ingxaki yokubona.
  • Intloko ebuhlungu.
  • Utshintsho kwindlela yokuziphatha okanye kubuntu.
  • Iingxaki zememori.

Ezi mpawu kunye neempawu zinokubangelwa zizilonda kwi-CNS okanye yi-CNS neurodegenerative syndrome.

Isibindi kunye nodaka

Iimpawu okanye iimpawu ze-LCH ezichaphazela isibindi okanye udakada kunokubandakanya:

  • Ukudumba esiswini okubangelwa kukwakheka kolwelo olongezelelekileyo.
  • Ingxaki yokuphefumla.
  • Mthubi ulusu kunye namhlophe ngamehlo.
  • Ukurhawuzelela.
  • Ukutyumza okulula okanye ukopha.
  • Ndiziva ndidiniwe.

Imiphunga

Iimpawu okanye iimpawu ze-LCH ezichaphazela umphunga zingabandakanya:

  • Umphunga owileyo. Le meko inokubangela iintlungu esifubeni okanye ukuqina, ingxaki yokuphefumla, ukuziva udiniwe, kunye nombala oluhlaza okwesibhakabhaka kulusu.
  • Ingxaki yokuphefumla, ngakumbi kubantu abadala abatshayayo.
  • Ukukhohlela owomileyo.
  • Iintlungu zesifuba.

Ummongo wethambo

Iimpawu okanye iimpawu ze-LCH ezichaphazela umongo wethambo zingabandakanya:

  • Ukutyumza okulula okanye ukopha.
  • Ifiva.
  • Rhoqo usulelo.

Uvavanyo oluvavanya amalungu kunye neenkqubo zomzimba apho i-LCH inokwenzeka khona zisetyenziselwa ukufumanisa i-LCH.

Ezi mvavanyo zilandelayo kunye neenkqubo zinokusetyenziselwa ukukhangela (ukufumana) kunye nokuchonga i-LCH okanye iimeko ezibangelwa yi-LCH:

  • Uvavanyo lomzimba kunye nembali yezempilo: Uvavanyo lomzimba ukukhangela iimpawu zempilo ngokubanzi, kubandakanya nokujonga iimpawu zesifo, ezinje ngamaqhuma okanye nayiphi na into ebonakala ingaqhelekanga. Imbali yemikhwa yempilo yesigulana kunye nezifo zangaphambili kunye nonyango ziya kuthathwa.
  • Uvavanyo lwe-Neurological: Uthotho lwemibuzo kunye novavanyo lokujonga ubuchopho, intambo yomqolo kunye nokusebenza kwentliziyo. Uvavanyo lujonga imeko yomntu ngokwengqondo, ulungelelwaniso, kunye namandla okuhamba ngesiqhelo, kunye nendlela esebenza ngayo izihlunu, imizwa kunye nengqondo. Oku kunokubizwa ngokuba luvavanyo lwe-neuro okanye uvavanyo lwe-neurologic.
  • Gcwalisa ubalo lwegazi (CBC) ngokwahluka-hlukeneyo: Inkqubo apho isampulu yegazi itsalwa kwaye ihlolwe oku kulandelayo:
  • Isixa sehemoglobin (iprotein ethwala ioksijini) kwiiseli ezibomvu zegazi.
  • Inxalenye yesampulu yegazi eyenziwe ziiseli ezibomvu zegazi.
  • Inani nohlobo lweeseli ezimhlophe zegazi.
  • Inani leeseli ezibomvu zegazi kunye neeplatelets.
  • Izifundo ze-chemistry yegazi: Inkqubo apho kuvavanywa khona isampulu yegazi ukulinganisa izixa zezinto ezithile ezikhutshelwe emzimbeni ngamalungu kunye nezicubu zomzimba. Inani elingaqhelekanga (eliphezulu okanye elisezantsi kunesiqhelo) lwento elinokuba ngumqondiso wesifo.
  • Uvavanyo lomsebenzi wesibindi: Uvavanyo lwegazi ukulinganisa amanqanaba egazi lezinto ezithile ezikhutshwe sisibindi. Inqanaba eliphezulu okanye elisezantsi lezi zinto zinokuba luphawu lwesifo esibindini.
  • Uvavanyo lwe-BRAF yemfuza: Uvavanyo lwaselebhu apho kuvavanywa khona isampulu yegazi okanye izicwili zotshintsho oluthile kuhlobo lweBRAF.
  • Urinalysis: Uvavanyo lokujonga umbala womchamo kunye neziqulatho zawo, ezinje ngeswekile, iproteni, iiseli ezibomvu zegazi kunye neeseli ezimhlophe zegazi.
  • Uvavanyo lokuncitshiswa kwamanzi: Uvavanyo lokujonga ukuba wenziwe kangakanani umchamo nokuba uyagxininiswa na xa unikwa amanzi amancinci okanye ungekho. Olu vavanyo lusetyenziselwa ukufumanisa isifo sikashukela i-insipidus, enokubangelwa yi-LCH.
  • Umnqweno wethambo kunye ne-biopsy: Ukususwa komongo wethambo kunye neqhekeza elincinci lethambo ngokufaka inaliti engenanto kumqolo. Ugqirha wezifo ujonga umongo wethambo kunye nethambo phantsi kwemicroscope ukukhangela imiqondiso ye-LCH.
Umnqweno wethambo lomnqweno kunye ne-biopsy. Emva kokuba indawo encinci yolusu iphonyiwe, inaliti yomongo wethambo ifakwa kwisithambo sethambo lesigulana. Iisampulu zegazi, ithambo, kunye nomongo wethambo ziyasuswa ukuze zihlolwe phantsi kwemicroscope.

Olu vavanyo lulandelayo lunokwenziwa kwizicubu ezazisuswe:

  • I-Immunohistochemistry: Uvavanyo lwaselebhu olusebenzisa ii-antibodies ukukhangela ii-antigen ezithile (iimpawu) kwisampulu yesicwili somguli. Amachiza omzimba ahlala enxulunyaniswa ne-enzyme okanye idayi ye-fluorescent. Emva kokuba ii-antibodies zibophelela kwi-antigen ethile kwisampulu yethishu, i-enzyme okanye idayi iyasebenza, kwaye i-antigen iya kuthi emva koko ibonwe phantsi kwemicroscope. Olu hlobo lovavanyo lusetyenziselwa ukufumanisa isifo somhlaza kunye nokunceda uxelele olunye uhlobo lomhlaza kolunye uhlobo lomhlaza.
  • Ukuhamba kwe-cytometry: Uvavanyo lwelebhu olulinganisa inani leeseli kwisampulu, ipesenti yeeseli eziphilayo kwisampulu, kunye neempawu ezithile zeeseli, ezinje ngobungakanani, imilo, kunye nobukho beempawu zethumba (okanye ezinye) umphezulu weseli. Iiseli ezivela kwisampulu yegazi lesigulana, umongo wethambo, okanye ezinye izicwili zinamabala edayi ye-fluorescent, ibekwe kulwelo, emva koko idlulise nganye nganye kwitampu yokukhanya. Iziphumo zovavanyo zisekwe kwindlela iiseli ezazinebala ledayi ye-fluorescent zisabela njani kwimbonakalo yokukhanya.
  • Ukuskena amathambo: Inkqubo yokujonga ukuba ngaba kukho ukwahlulahlula iiseli ngokukhawuleza kwithambo. Inani elincinci kakhulu lezinto ezisasazeka ngemitha lifakwa emthanjeni kwaye lihamba ngokuhamba kwegazi. Izinto ezinemitha eqhumayo ziqokelela emathanjeni anomhlaza kwaye zifunyenwe siskena.
Ukuskena amathambo. Inani elincinci lezinto ezinemitha eqhumayo lifakwe kumthambo womntwana kwaye lihamba ngegazi. Izinto ezinemitha yeathomathizi eziqokelela emathanjeni. Njengokuba umntwana elele phezu kwetafile eslayidayo phantsi kweskena, kufunyanwa into enemitha ye-radio kunye nemifanekiso yenziwe kwikhompyuter.
  • IX-reyi: X-reyi yamalungu namathambo ngaphakathi komzimba. IX-reyi luhlobo lwe-beam yamandla enokuhamba emzimbeni nakwifilimu, yenza umfanekiso weendawo ezingaphakathi komzimba. Ngamanye amaxesha kwenziwa uphando lwamathambo. Le yinkqubo yokwenza i-x-ray onke amathambo emzimbeni.
  • Ukuskena i-CT (ukuskena i-CAT): Inkqubo eyenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi emzimbeni, ezithathwe kwii-engile ezahlukeneyo. Imifanekiso yenziwe yikhompyuter edityaniswe kumatshini we-x-ray. Idayi inokufakwa ngaphakathi emthanjeni okanye iginywe ukunceda amalungu okanye izicwili zibonise ngokucacileyo. Le nkqubo ikwabizwa ngokuba yi-computed tomography, ikhompyuter ye-tomography, okanye ikhompyuter ye-axial tomography.
Ikhompyuter ye-tomography (CT) yesisu. Isigulana silele etafileni esisilayida ngomatshini we-CT, othatha imifanekiso ye-x-ray engaphakathi komzimba.
  • I-MRI (imaging resonance imaging): Inkqubo esebenzisa umazibuthe, amaza erediyo kunye nekhompyuter ukwenza uthotho lwemifanekiso eneenkcukacha zeendawo ezingaphakathi komzimba. Into ebizwa ngokuba yi-gadolinium inokufakwa ngaphakathi emthanjeni. I-gadolinium iqokelela ijikeleze iiseli ze-LCH ukuze zibonakale ziqaqambile emfanekisweni. Le nkqubo ikwabizwa ngokuba yimifanekiso yenyukliya yokujonga umfanekiso (i-NMRI).
Imagnetic resonance imaging (MRI) yesisu. Umntwana ulele phezu kwetafile etyibilika kwisikena se-MRI, esithatha imifanekiso engaphakathi komzimba. Iphedi esiswini somntwana inceda ukwenza imifanekiso icace gca.
  • Ukuskena i-PET (positron emission tomography scan) Inkqubo yokufumana iiseli zethumba emzimbeni. Inani elincinci leswekile ene-radioactive (iswekile) itofelwe emthanjeni. Iskena se-PET sijikeleza ujikeleze umzimba kwaye senze umfanekiso wendawo esetyenziswa kuyo iswekile emzimbeni. Iiseli zetyumzi ziyaqaqamba emfanekisweni kuba ziyasebenza kwaye zithatha iswekile eninzi kuneseli eziqhelekileyo.
Ukuskena i-Positron emission tomography (PET). Umntwana ulele phezu kwetafile etyibilika kwisikena sePET. Ukuphumla kwentloko kunye nomtya omhlophe kunceda umntwana ukuba alale. Inani elincinci leswekile ene-radioactive (iswekile) itofwe emthanjeni womntwana, kwaye iskena senza umfanekiso apho iswekile isetyenziswa khona emzimbeni. Iiseli zomhlaza zibonakala ziqaqambile emfanekisweni kuba zithatha iswekile eninzi kuneseli eziqhelekileyo.
  • Uvavanyo lwe-Ultrasound: Inkqubo apho amaza esandi aphembelela amandla aphezulu (i-ultrasound) akhutshelwa ngaphandle kwizicubu zangaphakathi okanye kwizitho zokwenza i-echoes. Iingqungquthela zenza umfanekiso wezicubu zomzimba ezibizwa ngokuba yi-sonogram. Umfanekiso unokuprintwa ukuze ujongwe kamva.
I-ultrasound yesisu. I-ultrasound transducer edityaniswe kwikhompyuter idlula ngaphezulu komphezulu wesisu. I-transducer ye-ultrasound igxotha amaza esandi kumalungu angaphakathi kunye nezicubu ukwenza ii-echoes ezenza i-sonogram (umfanekiso wekhompyuter).
  • Uvavanyo lomsebenzi wePulmonary (PFT): Uvavanyo lokujonga ukuba imiphunga isebenza njani. Ilinganisa ukuba ingakanani imiphunga onokuyibamba kwaye ukhawulezisa kangakanani ukungena nokuphuma kwemiphunga emiphungeni. Ikwalinganisa ukuba isetyenziswa kangakanani ioksijini kwaye ingakanani ikharbon diokside oyinikwayo xa uphefumla. Oku kukwabizwa ngokuba luvavanyo lomsebenzi wemiphunga.
  • I-Bronchoscopy: Inkqubo yokujonga ngaphakathi kwetrachea kunye neendlela ezinkulu zomoya kwimiphunga yeendawo ezingaqhelekanga. I-bronchoscope ifakwe ngempumlo okanye emlonyeni kwi-trachea kunye nemiphunga. I-bronchoscope sisixhobo esibhityileyo, esifana netyhubhu esinokukhanya kunye nelensi yokujonga. Inokuba nesixhobo sokususa iisampulu zethishu, ezijongwa phantsi kwemicroscope kwimpawu zomhlaza.
  • I-Endoscopy: Inkqubo yokujonga amalungu kunye nezicubu ngaphakathi komzimba ukujonga iindawo ezingaqhelekanga kumathambo okanye imiphunga. I-endoscope ifakwa ngokusikwa (ukusika) kulusu okanye ukuvula emzimbeni, njengomlomo. I-endoscope sisixhobo esibhityileyo, esifana netyhubhu esinokukhanya kunye nelensi yokujonga. Inokuba nesixhobo sokususa iisampulu zethishu okanye i-lymph node, ezijongwa phantsi kwemicroscope kwimpawu zesifo.
  • I-Biopsy: Ukususwa kweeseli okanye izicwili ukuze zijongwe ngemicroscope ngugqirha wezilwanyana ukujonga iiseli ze-LCH. Ukufumanisa i-LCH, i-biopsy yethambo, ulusu, ii-lymph node, isibindi, okanye ezinye iisayithi zesifo ezinokwenziwa.

Izinto ezithile zichaphazela ukuxela kwangaphambili (ithuba lokufumana kwakhona) kunye nokhetho lonyango.

I-LCH kumalungu afana nolusu, amathambo, ii-lymph node, okanye i-pituitary gland ihlala ibangcono kunyango kwaye ibizwa ngokuba "ngumngcipheko ophantsi". I-LCH kwipeni, isibindi, okanye umongo wethambo kunzima ukunyanga kwaye ubizwa ngokuba "ngumngcipheko omkhulu".

Ukuxela kwangaphambili kunye nokhetho lonyango luxhomekeke koku kulandelayo:

  • Umdala kangakanani umguli xa efunyaniswa ene-LCH.
  • Ngawaphi amalungu okanye iinkqubo zomzimba ezichaphazeleka yi-LCH.
  • Zingaphi izitho okanye iinkqubo zomzimba ezichaphazelekayo ngumhlaza.
  • Nokuba umhlaza ufumaneka esibindini, udakada, umongo wethambo, okanye amathambo athile kulukakayi.
  • Ngaba umhlaza uphendula ngokukhawuleza kangakanani kunyango lokuqala.
  • Nokuba kukho utshintsho oluthile kuhlobo lweBRAF.
  • Nokuba umhlaza usandula ukufumanisa ukuba ubuyile okanye ubuyile (uphindile).

Kwiintsana ukuya kunyaka omnye ubudala, i-LCH inokuhamba ngaphandle konyango.

Amanqanaba e-LCH

IINGONGOMA EZIPHAMBILI

  • Akukho nkqubo yokubeka inqanaba leLangerhans cell histiocytosis (LCH).
  • Unyango lwe-LCH lusekwe apho iiseli ze-LCH zifumaneka khona emzimbeni nokuba i-LCH inobungozi obuphantsi okanye ingozi ephezulu.
  • I-LCH yesiqhelo

Akukho nkqubo yokubeka inqanaba leLangerhans cell histiocytosis (LCH).

Ubungakanani okanye ukusasazeka komhlaza kuhlala kuchazwa njengezigaba. Akukho nkqubo yokumisela i-LCH.

Unyango lwe-LCH lusekwe apho iiseli ze-LCH zifumaneka khona emzimbeni nokuba i-LCH inobungozi obuphantsi okanye ingozi ephezulu.

I-LCH ichazwa njengesifo esikwinkqubo enye okanye isifo se-multisystem, kuxhomekeke ekubeni zingaphi iinkqubo zomzimba ezichaphazelekayo:

  • Inkqubo enye ye-LCH: I- LCH ifumaneka kwilungu elinye lomzimba okanye inkqubo yomzimba okanye ngaphezulu kwenxalenye yelungu okanye inkqubo yomzimba. Ithambo yeyona ndawo ixhaphakileyo yeLCH.
  • I-Multisystem LCH: I- LCH yenzeka kumalungu amabini okanye nangaphezulu okanye kwiinkqubo zomzimba okanye inokusasazeka emzimbeni wonke. I-Multisystem LCH ayiqhelekanga kunenkqubo enye ye-LCH.

I-LCH inokuchaphazela amalungu omngcipheko ophantsi okanye amalungu asemngciphekweni omkhulu:

  • Izitho ezinobungozi obuphantsi zibandakanya ulusu, ithambo, imiphunga, ii-lymph node, i-gastrointestinal tract, i-pituitary gland, i-thyroid gland, i-thymus kunye nenkqubo ye-nervous system (CNS).
  • Izitho ezinobungozi obuphezulu zibandakanya isibindi, udakada, kunye nomongo wethambo.

I-LCH yesiqhelo

I-LCH eqhubekayo ngumhlaza ophindaphindiweyo (ubuyile) emva kokuba unyangiwe. Umhlaza ungabuya ubuye endaweni enye okanye kwamanye amalungu omzimba. Ihlala iphinda ibuye emathanjeni, ezindlebeni, kulusu, okanye kwindlala yebhinqa. I-LCH ihlala iphinda ibuye unyaka emva kokuyeka unyango. Xa i-LCH iphinda ibuye, inokubizwa ngokuba kukusebenza kwakhona.

Unyango ngoNyango lweLCH

IINGONGOMA EZIPHAMBILI

  • Kukho iintlobo ezahlukeneyo zonyango kwizigulana ezine-Langerhans cell histiocytosis (LCH).
  • Abantwana abane-LCH kufuneka ukuba unyango lwabo lucwangciswe liqela lababoneleli ngezempilo abaziingcali kunyango lomhlaza wabantwana.
  • Iindidi ezisithoba zonyango olusetyenziswayo:
  • Unyango ngamayeza
  • Ugqirha
  • Unyango ngemitha
  • Unyango lwe-Photodynamic
  • Unyango lwe-Immunotherapy
  • Unyango ekujoliswe kulo
  • Olunye unyango lweziyobisi
  • Ukufakelwa kweseli
  • Ukujonga
  • Iindidi ezintsha zonyango ziyavavanywa kuvavanyo lweklinikhi.
  • Unyango lweLangerhans cell histiocytosis lunokubangela iziphumo ebezingalindelekanga.
  • Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi.
  • Izigulana zinokungena kuvavanyo lweklinikhi ngaphambi, ngexesha, okanye emva kokuqala unyango.
  • Xa unyango lwe-LCH luyeka, izilonda ezintsha zingavela okanye izilonda ezindala zingabuya.
  • Iimvavanyo zokulandelela zisenokufuneka.

Kukho iintlobo ezahlukeneyo zonyango kwizigulana ezine-Langerhans cell histiocytosis (LCH).

Iindidi ezahlukeneyo zonyango ziyafumaneka kwizigulana ezine-LCH. Olunye unyango lusemgangathweni (unyango olusetyenziswayo ngoku), kwaye olunye luvavanywa kwizilingo zonyango. Uvavanyo lweklinikhi yonyango luphononongo olwenzelwe ukunceda ukuphucula unyango lwangoku okanye ukufumana ulwazi kunyango olutsha kwizigulana ezinomhlaza. Xa uvavanyo lwezonyango lubonisa ukuba unyango olutsha lungcono kunonyango oluqhelekileyo, unyango olutsha kunokuba lunyango olusemgangathweni. Nanini na xa kunokwenzeka, abaguli kufuneka bathathe inxaxheba kuvavanyo lweklinikhi ukuze bafumane iintlobo ezintsha zonyango lwe-LCH. Olunye uvavanyo lwezonyango luvuleleke kuphela kwizigulana ezingaluqalanga unyango.

Uvavanyo lwezonyango lwenzeka kwiindawo ezininzi zelizwe. Ulwazi malunga novavanyo oluqhubekayo lwezonyango luyafumaneka kwiwebhusayithi ye-NCI. Ukukhetha olona nyango lufanelekileyo sisigqibo esibandakanya isigulana, usapho kunye neqela lokhathalelo lwempilo.

Abantwana abane-LCH kufuneka ukuba unyango lwabo lucwangciswe liqela lababoneleli ngezempilo abaziingcali kunyango lomhlaza wabantwana.

Unyango luya kujongwa yi-oncologist yabantwana, ugqirha ogxile ekunyangeni abantwana abanomhlaza. I-oncologist yabantwana isebenza nabanye ababoneleli ngezempilo babantwana abaziingcali ekunyangeni abantwana nge-LCH kwaye abagxile kwiindawo ezithile zonyango. Oku kunokubandakanya ezi ngcali zilandelayo:

  • Ugqirha wabantwana.
  • Ugqirha wabantwana.
  • I-hematologist yezilwanyana.
  • Imitha ye-oncologist.
  • Ingcali yonyango.
  • Ingcali yezigulana.
  • Ingcali yomongikazi wabantwana.
  • Ingcali yokuvuselela.
  • Ingcali yeengqondo.
  • Unontlalontle.

Iindidi ezisithoba zonyango olusetyenziswayo:

Unyango ngamayeza

I-Chemotherapy yonyango lomhlaza olusebenzisa iziyobisi ukunqanda ukukhula kweeseli zomhlaza, nokuba zibulale iiseli okanye uziyekise ekwahlukaneni. Xa i-chemotherapy ithathwa ngomlomo okanye ifakwe kwi-vein okanye kwimisipha, amachiza angena kwigazi kwaye anokufikelela kwiiseli zomhlaza kuwo wonke umzimba (systemic chemotherapy). Xa i-chemotherapy ibekwa ngqo eluswini okanye kwi-fluid ye-cerebrospinal, ilungu, okanye indawo yomzimba efana nesisu, amachiza achaphazela iiseli zomhlaza kwezo ndawo (i-chemotherapy yengingqi).

I-Chemotherapy inokunikwa ngenaliti okanye ngomlomo okanye ifakwe kulusu ukunyanga i-LCH.

Ugqirha

Utyando lunokusetyenziselwa ukususa izilonda ze-LCH kunye nenani elincinci lezicubu ezisondeleyo ezikufutshane. I-Curettage luhlobo lotyando olusebenzisa i-curette (isixhobo esibukhali, esimile okwecephe) ukukrwela iiseli ze-LCH kwithambo.

Xa kukho umonakalo omkhulu wesibindi okanye wemiphunga, ilungu lonke linokususwa kwaye kufakwe isibindi esempilweni okanye imiphunga kumxhasi.

Unyango ngemitha

Unyango ngemitha lunyango olusebenzisa amandla e-x-reyi okanye ezinye iintlobo zemitha yokubulala iiseli zomhlaza okanye ukuzigcina zikhula. Unyango lwangaphandle lwemitha lusebenzisa umatshini ongaphandle komzimba ukuthumela imitha kwindawo yomzimba enomhlaza. Unyango lwe-radiation ye-Ultraviolet B (UVB) lunokunikezelwa kusetyenziswa isibane esikhethekileyo esikhokelela kwimitha ye-LCH yolusu.

Unyango lwe-Photodynamic

Unyango lwe-Photodynamic lunyango lomhlaza olusebenzisa ichiza kunye nohlobo oluthile lokukhanya kwelaser ukubulala iiseli zomhlaza. Iyeza elingasebenziyo lide libonakaliswe kukukhanya lifakwe emthanjeni. Iyeza liqokelela ngakumbi kwiiseli zomhlaza kuneeseli eziqhelekileyo. Kwi-LCH, ukukhanya kwe-laser kujolise kulusu kwaye ichiza liyasebenza kwaye libulala iiseli zomhlaza. Unyango lwe-Photodynamic lubangela umonakalo omncinci kwizicubu ezisempilweni. Izigulana ezinonyango lwe-photodynamic akufuneki zichithe ixesha elininzi elangeni.

Kolunye uhlobo lonyango lwe-photodynamic, olubizwa ngokuba yi-psoralen kunye ne-ultraviolet A (PUVA), isigulana sifumana iyeza ekuthiwa yi-psoralen emva koko i-radiation ye-ultraviolet ijolise kulusu.

Unyango lwe-Immunotherapy

I-Immunotherapy lunyango olusebenzisa amajoni omzimba omzimba ukulwa nomhlaza. Izinto ezenziwe ngumzimba okanye ezenziwe elebhu zisetyenziselwa ukonyusa, ukuqondisa, okanye ukubuyisela ukhuseleko lwendalo lomzimba kumhlaza. Olu hlobo lonyango lomhlaza lukwabizwa ngokuba yi-biotherapy okanye unyango lwe-biologic. Kukho iintlobo ezahlukeneyo ze-immunotherapy:

  • I-Interferon isetyenziselwa ukunyanga i-LCH yolusu.
  • I-Thalidomide isetyenziselwa ukunyanga i-LCH.
  • I-immunoglobulin engena ngaphakathi (IVIG) isetyenziselwa ukunyanga i-CNS neurodegenerative syndrome.

Unyango ekujoliswe kulo

Unyango ekujoliswe kulo luhlobo lonyango olusebenzisa iziyobisi okanye ezinye izinto ukuhlasela iiseli zomhlaza. Unyango ekujoliswe kulo kunokubangela ingozi encinci kwiiseli eziqhelekileyo kunonyango lwechemotherapy okanye unyango lwemitha. Kukho iintlobo ezahlukeneyo zonyango olujolisiweyo:

  • I-Tyrosine kinase inhibitors ibhloko yeempawu ezifunekayo ekukhuleni kwamathumba. I-Tyrosine kinase inhibitors esetyenziselwa ukunyanga i-LCH ibandakanya oku kulandelayo:
  • I-Imatinib mesylate iyeka iiseli zeziqu zegazi ekubeni zijike zibe ziiseli ze-dendritic ezinokuba ziiseli zomhlaza.
  • I-BRAF inhibitors zivimba iiproteni ezifunekayo ekukhuleni kweseli kwaye zinokubulala iiseli zomhlaza. Uhlobo lwe-BRAF lufumaneka kwifom eguqulweyo (etshintshiweyo) kwenye i-LCH kwaye ukuyithintela kunokunceda ukugcina iiseli zomhlaza zingakhuli.
  • IVemurafenib kunye ne-dabrafenib zii-BRAF inhibitors ezisetyenziselwa ukunyanga i-LCH.
  • Unyango lwe-monoclonal antibody lusebenzisa ii-antibodies ezenziwe elebhu ezivela kuhlobo olunye lweeseli zomzimba. Ezi antibodies zinokuchonga izinto ezikumhlaza iiseli okanye izinto eziqhelekileyo ezinokunceda iiseli zomhlaza zikhule. Amachiza omzimba ancamathele kwizinto kwaye abulale iiseli zomhlaza, athintele ukukhula kwazo, okanye azigcine zisasazeka. Zingasetyenziselwa zodwa okanye ukuphatha iziyobisi, ityhefu, okanye izinto ezinemitha yeathom ngqo kwiiseli zomhlaza. Iintsholongwane zeMonoclonal zinikezelwa ngokufakwa.
  • I-Rituximab yi-monoclonal antibody esetyenziselwa ukunyanga i-LCH.

Olunye unyango lweziyobisi

Ezinye iziyobisi ezisetyenziselwa ukunyanga i-LCH zibandakanya oku kulandelayo:

  • Unyango lwe-Steroid, njenge-prednisone, isetyenziselwa ukunyanga izilonda ze-LCH.
  • Unyango lwe-bisphosphonate (njenge-pamidronate, i-zoledronate, okanye i-alendronate) isetyenziselwa ukunyanga izilonda zethambo kunye nokunciphisa iintlungu zethambo.
  • Iziyobisi ezichasayo ezichasayo ngamachiza (njenge-pioglitazone kunye ne-rofecoxib) aqhelekileyo asetyenziselwa ukunciphisa umkhuhlane, ukudumba, iintlungu nokubomvu. Iziyobisi ezichasayo kunye ne-chemotherapy zinokunikwa kunye nokunyanga abantu abadala ngethambo le-LCH.
  • I-Retinoids, efana ne-isotretinoin, iziyobisi ezinxulumene nevithamini A ezinokucothisa ukukhula kweeseli ze-LCH kulusu. Ii-retinoids zithathwa ngomlomo.

Ukufakelwa kweseli

Ukufakelwa kweseli kwistem yindlela yokunika i-chemotherapy kunye nokutshintsha iiseli ezenza igazi ezonakaliswe lunyango lwe-LCH. Iiseli zeziqu (iiseli zegazi ezingafakwanga) ziyasuswa egazini okanye kumongo wethambo wesigulana okanye somnikeli kwaye zibandisiwe kwaye zigcinwe. Emva kokuba i-chemotherapy igqityiwe, iiseli ezigciniweyo ezigciniweyo ziyanyibilika kwaye zibuyiselwe kwisigulana ngokufakwa. Ezi seli ze-stem ziphinde zafakwa zikhula ziye (kwaye zibuyisele) iiseli zegazi lomzimba.

Ukujonga

Ukujongwa kujongwa ngononophelo kwimeko yesigulana ngaphandle kokunika unyango de kuvele iimpawu okanye utshintsho.

Iindidi ezintsha zonyango ziyavavanywa kuvavanyo lweklinikhi.

Ulwazi malunga novavanyo lweklinikhi luyafumaneka kwiwebhusayithi yeNCI.

Unyango lweLangerhans cell histiocytosis lunokubangela iziphumo ebezingalindelekanga.

Ngolwazi malunga neziphumo ebezingalindelekanga eziqala ngexesha lonyango lomhlaza, jonga iphepha lethu Iziphumo ezingalunganga.

Iziphumo ebezingalindelekanga ezivela kunyango lomhlaza eziqala emva konyango kwaye ziqhubeke iinyanga okanye iminyaka zibizwa ngokuba ziziphumo ezifike kade. Iziphumo zokunyanga komhlaza zingabandakanya oku kulandelayo:

  • Ukukhula kancinci kunye nophuhliso.
  • Ukuphulukana nokuva.
  • Iingxaki zamathambo, izinyo, isibindi kunye nemiphunga.
  • Utshintsho kwiimvakalelo, imvakalelo, ukufunda, ukucinga, okanye inkumbulo.
  • Umhlaza wesibini, njenge-leukemia, i-retinoblastoma, i-Ewing sarcoma, ingqondo okanye umhlaza wesibindi.

Ezinye iziphumo ezinokufika emva kwexesha zinokunyangwa okanye zilawulwe. Kubalulekile ukuba uthethe noogqirha bomntwana wakho malunga nefuthe unyango lomhlaza olunokuba nalo emntwaneni wakho. (Jonga isishwankathelo se- malunga neZiphumo eziMva zaNyango zoMhlaza woMntwana ngolwazi oluthe kratya.)

Izigulana ezininzi ezine-LCH ye-multisystem ezineziphumo ezifike kade ezibangelwa lunyango okanye sisifo ngokwaso. Ezi zigulana zihlala zineengxaki zempilo zexesha elide ezichaphazela umgangatho wobomi babo.

Izigulana zinokufuna ukucinga ngokuthatha inxaxheba kuvavanyo lweklinikhi.

Kwezinye izigulana, ukuthatha inxaxheba kuvavanyo lweklinikhi kunokuba lolona khetho lonyango lufanelekileyo. Uvavanyo lwezonyango luyinxalenye yenkqubo yophando ngomhlaza. Uvavanyo lwezonyango luyenziwa ukufumanisa ukuba unyango lomhlaza olutsha lukhuselekile kwaye luyasebenza okanye lungcono kunonyango oluqhelekileyo.

Uninzi lonyango oluqhelekileyo lwanamhlanje lomhlaza lusekwe kuvavanyo lweklinikhi lwangaphambili. Izigulana ezithatha inxaxheba kuvavanyo lweklinikhi zingafumana unyango olusemgangathweni okanye zibe phakathi kwabokuqala ukufumana unyango olutsha.

Izigulana ezithatha inxaxheba kuvavanyo lweklinikhi zikwanceda ukuphucula indlela umhlaza oza kunyangwa ngayo kwixa elizayo. Nokuba uvavanyo lwezonyango alukhokeleli kunyango olusebenzayo, bahlala bephendula imibuzo ebalulekileyo kwaye bancedisa ukuqhubela phambili uphando.

Izigulana zinokungena kuvavanyo lweklinikhi ngaphambi, ngexesha, okanye emva kokuqala unyango.

Olunye uvavanyo lwezonyango lubandakanya kuphela izigulana ezingekafumani unyango. Olunye uvavanyo lokuvavanywa kunyango kwizigulana ezinomhlaza ongakhange ubengcono. Kukwakho nezilingo zeklinikhi ezivavanya iindlela ezintsha zokumisa umhlaza ekubuyeleni (ekubuyeni) okanye ukunciphisa iziphumo ebezingalindelekanga zonyango lomhlaza.

Uvavanyo lwezonyango lwenzeka kwiindawo ezininzi zelizwe. Ulwazi malunga novavanyo lweklinikhi oluxhaswe yi-NCI lunokufumaneka kwiphepha lewebhu le-NCI. Uvavanyo lwezonyango oluxhaswa yiminye imibutho lunokufumaneka kwiwebhusayithi yeClinicalTrials.gov.

Xa unyango lwe-LCH luyeka, izilonda ezintsha zingavela okanye izilonda ezindala zingabuya.

Izigulana ezininzi ezine-LCH ziba bhetele ngonyango. Nangona kunjalo, xa unyango luyeka, kungabonakala izilonda ezintsha okanye izilonda ezindala zinokubuya. Oku kubizwa ngokuba kukuphinda kwenziwe (ukuphinda-phinda) kwaye kungenzeka kwisithuba sonyaka omnye emva kokuyeka unyango. Izigulana ezinesifo se-multisystem ziyakwazi ukuphinda zisebenze. Iindawo eziqhelekileyo zokuvuselelwa kwethambo, iindlebe, okanye ulusu. Isifo seswekile i-insipidus nayo inokukhula. Iindawo ezincinci eziqhelekileyo zokuvuselela zibandakanya i-lymph nodes, umongo wethambo, i-spleen, isibindi, okanye imiphunga. Ezinye izigulana zinokuphinda zisebenze ngaphezulu kwesinye kwiminyaka eliqela.

Iimvavanyo zokulandelela zisenokufuneka.

Ngenxa yomngcipheko wokuphinda usebenze kwakhona, abaguli be-LCH kufuneka babekwe esweni iminyaka emininzi. Olunye uvavanyo olwenziweyo lokuchonga i-LCH lunokuphindwa. Oku kukubona ukuba unyango lusebenza kakuhle kangakanani kwaye ukuba ngaba kukho naziphi na izilonda ezitsha. Olu vavanyo lunokubandakanya:

  • Uvavanyo lomzimba.
  • Uvavanyo lwe-Neurological.
  • Uvavanyo lwe-Ultrasound.
  • IMRI.
  • Ukuskena i-CT.
  • Ukuskena iPET.

Olunye uvavanyo olunokufuneka lubandakanya:

  • Uvavanyo lwe-Brain stem oluvavanyileyo lokuphendula (i-BAER): Uvavanyo olulinganisa impendulo yengqondo ngokunqakraza izandi okanye iithoni ezithile.
  • Uvavanyo lomsebenzi wePulmonary (PFT): Uvavanyo lokujonga ukuba imiphunga isebenza njani. Ilinganisa ukuba ingakanani imiphunga onokuyibamba kwaye ukhawulezisa kangakanani ukungena nokuphuma kwemiphunga emiphungeni. Ikwalinganisa ukuba isetyenziswa kangakanani ioksijini kwaye ingakanani ikharbon diokside oyinikwayo xa uphefumla. Oku kukwabizwa ngokuba luvavanyo lomsebenzi wemiphunga.
  • I-x-ray yesifuba: IX-reyi yamalungu namathambo ngaphakathi kwesifuba. IX-reyi luhlobo lwe-beam yamandla enokuhamba emzimbeni nakwifilimu, yenza umfanekiso weendawo ezingaphakathi komzimba.

Iziphumo zolu vavanyo zingabonisa ukuba imeko yakho itshintshile okanye ukuba umhlaza ubuyile (buyela). Olu vavanyo ngamanye amaxesha lubizwa ngokuba luvavanyo olulandelayo okanye ukuhlolwa. Izigqibo malunga nokuqhubeka, ukutshintsha, okanye ukuyeka unyango zinokusekwa kwiziphumo zolu vavanyo.

Unyango lwe-LCH eseMngciphekweni waBantwana

Kweli Candelo

  • Izilonda zolusu
  • Izilonda kuMathambo okanye ezinye iiNgozi eziseMngciphekweni
  • Izilonda ze-CNS

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho.

Izilonda zolusu

Unyango lwezilonda zesikhumba seLangerhans cell histiocytosis (LCH) ezisanda kufunyaniswa zisenokubandakanya:

  • Ukujonga.

Xa kuvela irhashalala, iintlungu, izilonda, okanye ukopha, unyango lunokubandakanya oku kulandelayo:

  • Unyango lwe-Steroid.
  • I-Chemotherapy enikezwa ngomlomo okanye emthanjeni.
  • I-Chemotherapy isetyenziswe kulusu.
  • Unyango lwe-Photodynamic kunye ne-psoralen kunye ne-ultraviolet A (PUVA).
  • Unyango lwe-UVB.

Izilonda kuMathambo okanye ezinye iiNgozi eziseMngciphekweni

Unyango lwezilonda zamathambo ezisandula kufunyaniswa ebuntwaneni ngaphambili, emacaleni, okanye ngasemva kwekhakhayi, okanye kulo naliphi na elinye ithambo elinye linokubandakanya:

  • Utyando (i-curettage) kunye okanye ngaphandle kwonyango lwe-steroid.
  • Unyango lwe-radiation yedosi ephantsi yezilonda ezichaphazela amalungu akufutshane.

Unyango lwezilonda ezisandula ukufunyanwa zobuntwana ze-LCH emathanjeni ajikeleze iindlebe okanye amehlo kwenziwa ukwenzela ukunciphisa umngcipheko wesifo seswekile kunye nezinye iingxaki zexesha elide. Unyango lunokubandakanya:

  • I-Chemotherapy kunye nonyango lwe-steroid.
  • Ugqirha (unyango).

Unyango lwezilonda ezisandula ukufunyanwa komntwana kwi-LCH yomqolo okanye ithambo lethanga kunokubandakanya:

  • Ukujonga.
  • Unyango lwedosi ephantsi.
  • I-Chemotherapy, yezilonda ezisasazeka ukusuka kumqolo ukuya kwizicubu ezikufutshane.
  • Utyando lokomeleza ithambo elibuthathaka ngokudibanisa okanye ukudibanisa amathambo ngokudibeneyo.

Unyango lwezilonda zamathambo amabini okanye nangaphezulu kunokubandakanya:

  • I-Chemotherapy kunye nonyango lwe-steroid.

Unyango lwezilonda zamathambo amabini okanye nangaphezulu zidityaniswe nolusu, i-lymph node lesions, okanye isifo seswekile i-insipidus inokubandakanya:

  • I-Chemotherapy kunye okanye ngaphandle kwonyango lwe-steroid.
  • Unyango lwe-Bisphosphonate.

Izilonda ze-CNS

Unyango lwezilonda zesifo se-LCH esisembindini (CNS) esisandula ukufumanisa isifo sinokubandakanya:

  • I-Chemotherapy kunye okanye ngaphandle kwonyango lwe-steroid.

Unyango lwe-LCH CNS entsha ye-neurodegenerative syndrome inokubandakanya:

  • Unyango olujolise kwi-BRAF inhibitors (vemurafenib okanye dabrafenib).
  • Unyango ngamayeza.
  • Unyango olujoliswe kwi-anti-monoclonal antibody (rituximab).
  • Unyango lweRetinoid.
  • I-Immunotherapy (IVIG) kunye okanye ngaphandle kwe-chemotherapy.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

Unyango lwe-LCH ephezulu yomngcipheko kuBantwana

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho.

Unyango lwezifo zesifo se-LCH esandula ukufunyanwa sisifo esisesipeni, isibindi, okanye umongo wethambo kunye nelinye ilungu okanye indawo inokubandakanya:

  • I-Chemotherapy kunye nonyango lwe-steroid. Amayeza aphezulu e-chemotherapy angaphezulu kwesinye kunye nonyango lwe-steroid lunokunikwa kwizigulana ezinamathumba angaphenduliyo kwikhemotherapy yokuqala.
  • Unyango ekujoliswe kulo (vemurafenib).
  • Ukufakelwa kwesibindi kwizigulana ezinomonakalo omkhulu wesibindi.
  • Uvavanyo lweklinikhi olulungiselela unyango lomguli ngokusekwe kwiimpawu zomhlaza kunye nendlela asabela ngayo kunyango.
  • Uvavanyo lwezonyango lwe-chemotherapy kunye nonyango lwe-steroid.

Unyango lweLL eQhelekileyo, eKhanyayo, kunye neNkqubela phambili yoBuntwana kwi-LCH yaBantwana

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho.

I-LCH eqhubekayo ngumhlaza ongenakufunyanwa ixesha elithile emva konyango kwaye ubuye ubuye. I-Refractory LCH ngumhlaza ongangcono kunyango. Inkqubela phambili ye-LCH ngumhlaza oqhubeka ukhula ngexesha lonyango.

Unyango lwe-LCH ephindaphindayo, ephikisayo, okanye eqhubekayo yomngcipheko ophantsi inokubandakanya:

  • I-Chemotherapy kunye okanye ngaphandle kwonyango lwe-steroid.
  • Unyango lwe-Bisphosphonate.

Unyango lwezixhobo eziphindaphindayo, ezichasayo, okanye ezihamba phambili zomngcipheko ophezulu we-LCH unokubandakanya:

  • I-dose ephezulu ye-chemotherapy.
  • Unyango ekujoliswe kulo (vemurafenib).
  • Ukufakelwa kweseli.

Unyango olufundelwa ukuphinda-phinda, ukuphikisa, okanye ukuqhubeka kwe-LCH yobuntwana kubandakanya oku kulandelayo:

  • Uvavanyo lweklinikhi olulungiselela unyango lomguli ngokusekwe kwiimpawu zomhlaza kunye nendlela asabela ngayo kunyango.
  • Uvavanyo lweklinikhi olujonga isampulu yethumba lesigulana kutshintsho oluthile kwimfuza. Uhlobo lonyango ekujoliswe kulo oluya kunikwa isigulana luxhomekeke kuhlobo lotshintsho lwemfuza.

Unyango lwe-LCH kubantu abadala

Kweli Candelo

  • Unyango lwe-LCH yemiphunga kubantu abadala
  • Unyango lwe-LCH ye-Bone kuBantu abadala
  • Unyango lwe-LCH yolusu kubantu abadala
  • Unyango lweNkqubo eNye kunye neMultisystem LCH kubantu abadala

Ngolwazi malunga nonyango oludweliswe ngezantsi, jonga kwiCandelo loKhetho ngokujonga uKhetho

I-Langerhans cell histiocytosis (LCH) kubantu abadala ifana ne-LCH ebantwaneni kwaye inokwenza kumalungu afanayo kunye neenkqubo njengoko zisenza ebantwaneni. Oku kubandakanya iinkqubo ze-endocrine kunye ne-central nervous system, isibindi, udakada, umongo wethambo, kunye namathumbu esiswini. Kubantu abadala, i-LCH ixhaphake kakhulu emiphungeni njengesifo esinye senkqubo. I-LCH emiphungeni yenzeka rhoqo kubantu abadala abancinci abatshayayo. I-LCH yabantu abadala ikwafumaneka kwithambo okanye kulusu.

Njengakubantwana, iimpawu kunye neempawu ze-LCH zixhomekeke apho kufumaneka khona emzimbeni. Jonga icandelo loLwazi ngokubanzi malunga neempawu kunye neempawu ze-LCH.

Uvavanyo oluvavanya amalungu kunye neenkqubo zomzimba apho i-LCH inokwenzeka khona zisetyenziselwa ukufumana (ukufumana) kunye nokufumanisa i-LCH. Jonga icandelo loLwazi ngokubanzi malunga neemvavanyo kunye neenkqubo ezisetyenziselwa ukufumanisa i-LCH.

Kubantu abadala, alukho ulwazi oluninzi malunga nokuba loluphi unyango olusebenza kakuhle. Ngamanye amaxesha, ulwazi luvela kuphela kwiingxelo zokuxilongwa, unyango, kunye nokulandelelwa komntu omdala omnye okanye iqela elincinci labantu abadala abanikwe uhlobo olufanayo lonyango.

Unyango lwe-LCH yemiphunga kubantu abadala

Unyango lwe-LCH yemiphunga kubantu abadala inokubandakanya:

  • Ukuyeka ukutshaya kuzo zonke izigulana ezitshayayo. Ukulimala kwemiphunga kuya kuba kubi ngokuhamba kwexesha kwizigulana ezingayeki ukutshaya. Kwizigulana eziyeke ukutshaya, ukonakala kwemiphunga kunokuba ngcono okanye kunokuba kubi ngokuhamba kwexesha.
  • Unyango ngamayeza.
  • Ukufakelwa kwemiphunga kwizigulana ezinomonakalo omkhulu wemiphunga.

Ngamanye amaxesha i-LCH yemiphunga iya kuhamba okanye ingabi mandundu nokuba ayinyangwa.

Unyango lwe-LCH ye-Bone kuBantu abadala

Unyango lwe-LCH oluchaphazela kuphela ithambo kubantu abadala lunokubandakanya:

  • Ugqirha kunye okanye ngaphandle kwonyango lwe-steroid.
  • I-Chemotherapy kunye okanye ngaphandle kwonyango lweedosi ezisezantsi.
  • Unyango ngemitha.
  • Unyango lwe-Bisphosphonate, lwentlungu ebuhlungu yamathambo.
  • Iziyobisi Anti-ukudumba kunye chemotherapy.

Unyango lwe-LCH yolusu kubantu abadala

Unyango lwe-LCH oluchaphazela kuphela ulusu kubantu abadala lunokubandakanya:

  • Ugqirha.
  • I-Steroid okanye olunye unyango lweziyobisi olusetyenzisiweyo okanye olufakwe ulusu.
  • Unyango lwe-Photodynamic kunye ne-psoralen kunye ne-ultraviolet A (PUVA) radiation.
  • Unyango lwe-UVB.
  • I-Chemotherapy okanye i-immunotherapy enikezelwa ngomlomo, njenge-methotrexate, thalidomide, i-hydroxyurea, okanye i-interferon.
  • Unyango lwe-Retinoid lunokusetyenziswa ukuba ulusu lolusu aluphucuki nolunye unyango.

Unyango lwe-LCH oluchaphazela ulusu kunye nezinye iinkqubo zomzimba kubantu abadala lunokubandakanya:

  • Unyango ngamayeza.

Unyango lweNkqubo eNye kunye neMultisystem LCH kubantu abadala

Unyango lwe-single-system kunye ne-multisystem isifo kubantu abadala esingachaphazeli imiphunga, ithambo, okanye ulusu kunokubandakanya:

  • Unyango ngamayeza.
  • Unyango ekujoliswe kulo (imatinib, okanye vemurafenib).

Ngolwazi oluthe kratya malunga novavanyo lwe-LCH kubantu abadala, jonga iwebhusayithi ye-Histiocyte SocietyExit Disclaimer.

Sebenzisa uvavanyo lwethu lweklinikhi ukufumana ulingo lweklinikhi oluxhaswayo lwekliniki olwamkela izigulana. Unokukhangela izilingo ngokusekwe kuhlobo lomhlaza, ubudala besigulana, kwaye nokuba zenziwa phi izilingo. Ulwazi ngokubanzi malunga novavanyo lweklinikhi luyafumaneka.

Ukuze ufunde okungakumbi malunga neLangerhans Cell Histiocytosis

Ngolwazi oluthe kratya kwiZiko leSizwe loMhlaza malunga nonyango lweLangerhans cell histiocytosis, jonga oku kulandelayo:

  • Ikhompyuter yeTomography (CT) kunye noMhlaza
  • Unyango lwe-Photodynamic kuMhlaza
  • Unyango lwe-Immunotherapy ukunyanga uMhlaza
  • Iithagethi zoMhlaza ezijolise kuko
  • Utyando lweSeli oluQulunqwe ngegazi

Ngolwazi oluthe kratya lomhlaza wabantwana kunye nezinye izixhobo zomhlaza ngokubanzi, jonga oku kulandelayo:

  • Malunga noMhlaza
  • Umhlaza wabantwana
  • Unyango lokuKhangela uMhlaza waBantwanaPhuma kwiNgcazelo yokuzihlangula
  • Iziphumo zokugqibela zonyango lweCancer yoMntwana
  • Abafikisayo kunye naBantu abaDala abaDala abanomhlaza
  • Abantwana abanomhlaza: Isikhokelo sabazali
  • Umhlaza kuBantwana nakwishumi elivisayo
  • Ukuqokelela
  • Ukujamelana noMhlaza
  • Imibuzo yokubuza ugqirha wakho malunga neCancer
  • Abasindileyo kunye nabaNonopheli


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