Chii chinonzi Langerhans Cell Histiocytosis (LCH)? Mwana wako ari panjodzi here?

Wakambonzwa nezveLangerhans Cell Histiocytosis, kana kuti yatinoti LCH muchidimbu? Pamwe zita racho rinonzwika serakaoma uye rinotyisa. Asi muchokwadi, ichi chirwere chisingawanzoitiki chinonyanya kubata vana vedu vadiki, kunyanya vacheche nevana vari pasi pemakore gumi nemashanu ekuberekwa. Saka musazvidya mwoyo, ngatitaurei nezvazvo zviri nyore, muchiSinhala chamunogona kunzwisisa zvakadzama, sekunge muri kutaura neshamwari yepedyo.

Chii chaizvo chinonzi (LCH)?

Zvichitaurwa zviri nyore, (LCH) chirwere chinoitika kana muviri wemwana uine masero eLangerhans akawanda , rudzi rwemasero akakosha muimmune system yedu. Aya masero eLangerhans chaizvoizvo rudzi rwemasero machena eropa. Basa rawo guru nderekurwisa utachiona hunopinda mumuviri wedu uye kutidzivirira kubva kuzvirwere.

Masero aya anowanzo kuwanikwa mumiviri yedu yese, kunyanya muganda, mapapu, lymph nodes, bone morrow, spleen, nechiropa. Zvisinei, kana paine (LCH), masero aya eLangerhans anoungana akawandisa munzvimbo imwe chete kana kupfuura. Kana izvi zvikaitika, nhengo idzi dzinogona kukuvara uye maronda anogona kuumbwa.

Mafambiro echirwere ichi anogona kusiyana kubva pamunhu kusvika pamunhu. Vamwe vana vanopora zvachose kubva muchirwere ichi nekurapwa kwakakodzera. Zvinoshamisa kuti dzimwe nguva, kunyanya kana chikangokanganisa ganda chete, chinogona kunaya pasina kurapwa. Zvisinei, kana chirwere ichi (LCH) chikakanganisa nhengo dzinokosha dzakadai semupfupa wemwana, chiropa kana chiropa, kurapwa kwakasimba kunogona kudiwa.

Ko kenza (LCH) igovaniswa here?

Uyu mubvunzo unowanzo bvunzwa nevanhu vazhinji. Kutaura zvazviri, vaongorori vazhinji vanoona LCH sechirwere chegomarara, kureva kuti, bundu. Zvisinei, vamwe masayendisiti vava kuona chirwere ichi sechirwere chekuzvimba. Zvisinei, vanachiremba vekenza vanachiremba vane hunyanzvi mugomarara nezvirwere zveropa. Dzimwe nguva, mishonga yegomarara, yakadai sechemotherapy, inoshandiswawo kurapa chirwere ichi.

Ndiani anonyanya kubatwa nechirwere ichi (LCH)?

LCH inonyanya kuwanikwa muvacheche nevana vari pakati pemakore 1 ne15. LCH muvanhu vakuru haiwanzoitiki, asi hazvigoneki.

Izvi zvakajairika zvakadii?

Zvichienderana nehuwandu, mwana mumwe chete kana vaviri pavana vacheche vanobatwa neLCH gore rega rega. Zvakare, vana vanosvika vashanu pavana miriyoni vari pasi pemakore gumi nemashanu vanobatwa neLCH. Saka unogona kuona kuti ichi chirwere chisingawanzoitiki.

Ndezvipi zviratidzo zve (LCH)?

Zviratidzo zveLCH zvinosiyana zvikuru kubva pamunhu kusvika pamunhu. Zvinogona kukanganisa nzvimbo imwe chete yemuviri, kana kuti zvinogona kukanganisa nzvimbo dzakawanda. Saka, zviratidzo zvinoenderana nekuti ndeipi nhengo yemuviri wemwana inobatwa.

Kana mapfupa akanganiswa:

Vana vanosvika 80% vane LCH vanowana maronda mumapfupa avo rimwe kana kupfuura. Chii chinogona kukonzera izvi?

• Kuzvimba kana bundu rinogona kuitika munzvimbo dzakadai sedehenya, mapfupa akakomberedza maziso, mapfupa enzeve, mapfupa eshaya, makumbo, musana, chiuno, kana mbabvu. Kuzvimba uku kunogona kurwadza kana kupera.
• Musoro.
• Kurwadziwa kwemutsipa kana musana.
• Kutyoka kwemapfupa.
• Kuomerwa nekufamba.
• Kukamhina.

Paganda:

Mapundu anowanzoonekwa sezviratidzo zveganda (LCH).

• Muvana vadiki, mapundu anotaridzika sechivharo chemwana anogona kuoneka pamusoro peganda remusoro.
• Muvana vakuru nevakuru, zvinogona kukonzera mapundu anotsemuka anoita semapundu.
• Mapundu aya anogonawo kuonekwa kune dzimwe nhengo dzemuviri (kugomera, maoko, muhapwa, mudumbu, musana, chipfuva). Anogona kunge anorwadza, achikwenya, kana kuti akaomarara.
• Vamwe vana vanogona kubuda maronda anobuda.
• Zvinhu zvakaita sekushata kweruvara, kuomarara, kana kukweshwa kwezvipikiri zvinogonawo kuitika.

Nezvemuromo:

Zviratidzo zvinogona kuonekwa mumuromo zvinosanganisira:

• Kusununguka kwezino kana kurasikirwa nemazino.
• Zino rakadhonzwa.
• Kuzvimba kwematadza.
• Maronda pamiromo, parurimi, mukati mematama, kana pamusoro pemuromo.

Zvine chekuita nechiropa kana chirwatata:

Kana nhengo idzi dzikabatwa, zviratidzo zvakaita se:

• Kuzvimba kwedumbu nekuda kwekukura kwechiropa uye/kana spleen.
• Kutsvuka kweganda uye machena emaziso (jaundice).
• Kukwenya.
• Kuneta.
• Kukuvara zviri nyore uye/kana kubuda ropa.

Kana mupfupa wakanganiswa:

Mumwongo wemapfupa ndipo panogadzirwa masero eropa. Kana akanganiswa:

• Kushaya ropa, ganda rakachenuruka, kuneta, uye kushaya chido chekudya nekuda kwekudzikira kwemasero matsvuku eropa.
• Kurwara kakawanda uye fivha inokonzerwa nekushaikwa kwemasero machena eropa.
• Kutsvuka kuri nyore uye/kana kubuda ropa nekuda kwekushaya maplatelet.

Sisitimu yeEndocrine (Sisitimu yeEndocrine - Mahormone):

LCH inogona kukanganisa endocrine system yemwana, uko kunogadzirwa mahormone, akadai sepituitary gland nethyroid gland.

• Kana pituitary gland ikakanganiswa: nyota yakawandisa (polydipsia) uye kuita weti kakawanda (inonziwo diabetes insipidus), kutadza kukura, kutanga kana kunonoka kuyaruka, uye kuwedzera uremu.
• Kana thyroid gland ikakanganiswa: kuzvimba kwethyroid gland, zviratidzo zvemazinga akaderera emahormone ethyroid (hypothyroidism), uye kunetseka kufema.

Nezvenzeve:

• Kubatwa nezvirwere munzeve kakawanda.
• Mvura iri kubuda munzeve.
• Kutsvuka.
• Mapundu anokwenya.
• Kurwadziwa kwenzeve.
• Kurasikirwa nekunzwa.

Nezvemaziso:

• Maziso akatumbuka.
• Kuzvimba pamusoro pemaziso.
• Matambudziko ekuona.

MaNode eLymph:

• Ma lymph nodes akazvimba uye anorwadza mumutsipa, muhapwa, uye/kana muhudyu.

Sisitimu Yetsinga Yepakati:

Izvi zvinoreva kuti uropi uye/kana musana zvinokanganiswa.

• Musoro.
• Dzungu.
• Kurutsa.
• Nyota yakawandisa.
• Kuita weti kakawanda.
• Kuomerwa nekufamba.
• Kurasikirwa nekuenzana.
• Kusakwanisa kudzora mafambiro emuviri (ataxia).
• Kuomerwa nekutaura kana kuona.
• Kubatwa nepfari.
• Kuchinja kwemaitiro uye/kana ndangariro.

Mapapu:

Chirwere chemapapu chinonzi LCH, chirwere chinokanganisa mapapu, chinowanzoitika kuvanhu vakuru. Vanhu vanosvuta ndivo vari panjodzi huru.

• Kurwadziwa kwechipfuva.
• Kukosora kwakaoma.
• Kutadza kufema zvakanaka.
• Kukosora ropa.
• Kukundikana kwemapapu (pneumothorax).

Nzira yeMatumbu:

Kana dumbu remwana, ura hudiki, uye/kana ura hukuru hwakanganiswa:

• Kurwadziwa kwemudumbu.
• Kurutsa.
• Manyoka.
• Ropa riri mutsvina.
• Kukundikana kukura nekuda kwekushaya kudya kwakakwana.

Zvakakosha: Zviratidzo izvi zvinogona kukonzerwa nezvimwe zvirwere zvakasiyana-siyana, kwete LCH chete. Saka, kana mwana wako aine zviratidzo izvi, zvakakosha kutsvaga rubatsiro rwechiremba nekukasika kuti aonekwe zvakanaka.

Ndezvipi zvinokonzera chirwere che (LCH)?

Vanhu vanenge hafu chete vane LCH vane shanduko yemuviri mujini rinonzi BRAF. Shanduko dzemuviri kuchinja kunoitika mumasero mamwe mushure mekubatwa kwepamuviri. Hadzigamuchirwi kubva kuvabereki, asi dzinoitika zvisina tsarukano panguva yekukura kwemwana ari mudumbu.

Jini re (BRAF) rinogadzira puroteni inodzora kukura nekukura kwemasero. Kazhinji, puroteni iyi inogona kuvhurwa nekuvharwa zvichienderana nezviratidzo zvemakemikari. Zvisinei, kana paine shanduko mujini iri, puroteni inogara yakanamatira mu "on". Izvi zvinokonzera masero e (LCH) kukura nekupatsanurana zvakanyanya. Izvi ndizvo zvinokonzera kukuvara kwematishu uye bundu kuumbwa.

Masayendisiti akawanawo kuti kuchinja kwemajini akawanda kunogona kukonzerawo chirwere ichi. Semuenzaniso, majini (MAP2K1), (RAS), uye (ARAF). Vamwe vaongorori vanotenda kuti chepfu dzezvakatipoteredza nehutachiona hwemavhairasi zvinogonawo kukonzera chirwere ichi.

Ndezvipi zvinhu zvinokonzera njodzi ye (LCH)?

Zvimwe zvinhu zvinogona kuwedzera njodzi yemwana wako yekuwana chirwere cheLCH. Izvi zvinosanganisira:

• Kuva nenhoroondo yemhuri yeLCH.
• Kuva nerudzi rwechiHispanic (kunyange zvazvo izvi zvisina basa kunyika yedu, zvinotaurwa muzvinyorwa).
• Kusvuta (kunyanya kune vakuru vane kenza yemapapu (LCH)).
• Kusangana nemamwe makemikari panguva yekuzvitakura.
• Kusangana nesimbi, granite, kana guruva rehuni kubasa.
• Utachiona panguva yekuzvarwa.
• Kusawana majekiseni anokurudzirwa panguva yehudiki.

Ndezvipi zvinetso zvinogona kuitika nekuda kwe (LCH)?

Vana vanosvika 50% vane chirwere cheLCH vanowana matambudziko akasiyana-siyana nekuda kwechirwere ichi. Semuenzaniso:

• Mavanga.
• Kunonoka kukura.
• Kuremara kwetsandanyama nemapfupa.
• Chirwere chakaita sechirwere cheshuga (Diabetes insipidus).
• Kusawirirana kwemahormone.
• Kusanzwa zvakanaka.
• Matambudziko epfungwa (akadai sekuora mwoyo, kuzvidya mwoyo).
• Matambudziko emapfupa nemapapu.
• Kuoma kwechiropa.
• Kenza dzechipiri (semuenzaniso leukemia, lymphoma, Ewing sarcoma).

LCH inoonekwa sei?

Chiremba wemwana wenyu achatanga abvunza nezvenhoroondo yehutano hwemwana wenyu uye oita bvunzo yemuviri. Zvadaro, vacharaira bvunzo dzakawanda zvichienderana nezviratidzo zviri kusangana nemwana wenyu. Zvichienderana nemhedzisiro yebvunzo idzi, mwana wenyu anogona kutumirwa kuna chiremba weropa/oncologist wevana, uyo acharonga kurapwa nekutarisirwa kwemwana wenyu.

Ndedzipi bvunzo dzinoitwa kuti dziongororwe chirwere?

Nekuti LCH inogona kukanganisa nhengo dzakasiyana dzemuviri, bvunzo dzinoverengeka dzinodiwa kuti chirwere ichi chiongororwe nemazvo.

• Kuongororwa kweropa:
• Kuverengwa kweropa kwakazara (CBC): Izvi zvinoongorora huwandu hwemasero matsvuku eropa, masero machena eropa, uye maplatelet emwana.
• Kuongororwa kwemakemikari eropa: Izvi zvinoongorora huwandu hwezvimwe zvinhu zvinoburitswa nenhengo dzemuviri nematishu.
• Kuongororwa kwebasa rechiropa: Izvi zvinoongorora huwandu hwezvinhu zvinoburitswa nechiropa.

• Kuongororwa kweweti:
• Kuongororwa kweweti: Weti yemwana inoongororwa kuti ione huwandu hwemasero matsvuku eropa, masero machena eropa, mapuroteni, uye shuga.
• Kuongororwa kwekushaya mvura: Izvi zvinoongorora kuti mwana anoita weti yakawanda sei uye kana weti yacho ikanyanya kuzara kana asina kupiwa mvura.

• Kuongororwa kwemapfupa:
• Kuongororwa kwemuviri: Chiremba anotora sampuli yenyama, uye chiremba wezvirwere anoitarisa pasi pemaikorosikopu kuti aone kana paine masero eLCH.
• Kutsvaga mamongo emapfupa uye biopsy: Tsono ine buri inoshandiswa kubvisa mamongo emapfupa, ropa, nechidimbu chidiki chepfupa kubva papfupa rehudyu remwana. Izvi zvinoongororwawo nenyanzvi yezvirwere.

• Kuongororwa kwemajini:
• Ropa kana kuti tissue sampuro inoshandiswa kutarisa shanduko dziri mujini re (BRAF).

• Miedzo yemifananidzo:
• X-ray: Mifananidzo yemapfupa nenhengo dzemuviri inotorwa. Dzimwe nguva ongororo yemapfupa emuviri wese inoitwa kuti vaone kana paine zvisina kumira zvakanaka.
• Kuskena mapfupa: Chinhu chine radioactive chinopinzwa mutsinga uye scanner inoshandiswa kutsvaga marara asina kujairika mumapfupa. Izvi hazvichashandiswi zvakanyanya.
• CT scan (Computed tomography - CT scan): Mwana anopiwa mvura yakakosha yekunwa kana kubaiwa mutsinga, uye mifananidzo yakadzama inotorwa kubva kumativi akasiyana mukati memuviri.
• Positron emission tomography (PET scan): Shuga shoma ine radioactive (glucose) inopinzwa mutsinga, uye scanner inofambiswa mumuviri wese kuti itore mifananidzo yenzvimbo iri kushandiswa shuga. Masero ane chirwere anoonekwa akajeka pa scan iyi.
• Kuongororwa kweMRI (Magnetic resonance imaging - MRI scan): Chinhu chinonzi gadolinium chinopinzwa mutsinga uye mifananidzo yakatevedzana inotorwa uchishandisa magineti, mafungu eredhiyo, uye komputa. Nzvimbo dzine chirwere dzinoita sedzakajeka.
• Ultrasound: Inoshandisa mafungu eruzha ane simba guru kugadzira mifananidzo yemukati memuviri nekuratidza manzwi ari kubva munhengo nematishu.

LCH inorapwa sei?

Kurapwa kweLCH kunoenderana nekuti masero eLCH ari kupi mumuviri wemwana uye kuti chirwere chacho "chine njodzi shoma" here kana kuti "chine njodzi huru."

Nhengo dzemuviri dzine njodzi shoma ndedzinotevera:

• Ganda
• pfupa
• Mapapu
• Manodhi elymph
• Sisitimu yemudumbu
• Gland repituitary
• Gland re thyroid
• Thymus

Nhengo dzemuviri dzine njodzi huru ndedzi:

• Chiropa
• Chidendene
• Mupfupa
• Sisitimu yetsinga yepakati (CNS)

Vanachiremba vanoisa chirwere ichi muzvikamu zve "single-system LCH" uye "multi-system LCH." Izvi zvinoreva:

• Sisitimu imwe chete (LCH): Masero eLCH anowanikwa muchikamu chimwe chete chenhengo imwe chete kana sisitimu yemuviri. Rudzi rwakajairika iLCH inongobata mapfupa chete.
• Multisystem (LCH): Kune masero munhengo mbiri kana kupfuura, kana mumuviri wese (LCH). Izvi hazviwanzoitiki pane single-system (LCH).

Muzvimwe zviitiko, kunyanya izvo zvinongobata ganda kana mapfupa (LCH) chete, chirwere ichi chinogona kupera pasina kurapwa. Muzviitiko zvakadaro, vanachiremba vanoongorora chirwere ichi kuti vaone kana chichidzoka kana kuti chichipararira.

Sarudzo dzekurapa (LCH):

• Kurapa neSteroid: Chiremba wako anogona kushandisa steroid, dzakadai seprednisone, kunyanya yeLCH yeganda. Izvi zvinoderedza kushanda kwemasero machena eropa, izvo zvinogonawo kukanganisa masero eLCH.
• Kuvhiyiwa: Mabundu eLCH nenyama yakapoteredza zvinogona kubviswa nekuvhiyiwa. Nzira inonzi curettage inosanganisira kukwenya masero eLCH kubva mumapfupa uchishandisa chinhu chakapinza, chakaita sechipunu (curette).
• Kurapa nemakemikari: Izvi zvinosanganisira kupa masero egomarara mishonga yekuamisa kukura. Anouraya masero kana kuamisa kupatsanuka. Mishonga iyi inotorwa nemuromo, inobayiwa mutsinga kana tsandanyama, kana dzimwe nguva kuiswa paganda sekirimu.
• Kurapa nemwaranzi: MaX-ray ane simba guru kana mamwe marudzi emwaranzi anoshandiswa kuuraya masero ekenza kana kuamisa kukura nekupatsanuka.
• Kurapa Nezvirwere Zvemuviri: Nzira yekushandisa masoja emuviri emwana kurwisa gomarara. Dziviriro yemuviri inosimbiswa nekushandisa zvinhu zvakagadzirwa nemuviri pachawo kana izvo zvakagadzirwa murabhoritari.
• Kurapa kwakanangana: Kunoshandisa mishonga kana zvimwe zvinhu kuti zvionekwe uye zvirwise masero egomarara chaiwo. Semuenzaniso, mishonga inonzi BRAF inhibitors inogona kuuraya masero egomarara nekuvharira mapuroteni anodiwa kuti masero akure. Ma antibodies emonoclonal mapuroteni ekudzivirira muviri akagadzirwa murabhoritari.
• Kuisirwa kwemasero eropa: Kuisirwa kwemasero matsva kuti kutsiva masero anogadzira ropa akaparadzwa nechemotherapy.

LCH inogona kudzivirirwa here?

LCH inogona kudzivirirwa zvakanyanya nekuti inokonzerwa nekuchinja kwemajini. Hatigone kudzora zvimwe zvinhu zvinokonzera njodzi, zvakaita senhoroondo yemhuri uye rudzi. Zvisinei, pane zvimwe zvinhu zvatinogona kugadzirisa:

• Kusvuta hakubvumirwi.
• Kudzivirira mamwe makemikari anokuvadza panguva yekuzvitakura.
• Kuwana majekiseni ese anokurudzirwa.

Chii chinonzi fungidziro ye (LCH)?

Maonero eLCH anoenderana nezvinhu zvakawanda:

• Mangani masisitimu emuviri kana nhengo dzemuviri dzinokanganiswa?
• Ndeapi masisitimu emuviri kana nhengo dzemuviri dzinokanganiswa.
• Kuti chirwere ichi chinopindura sei kana charapwa.

Kazhinji, vana vane single-system (LCH) uye multisystem (LCH) isingakanganisi chiropa, spleen, kana bone marrow vanowira muchikamu che "low-risk". Nekurapwa, vana vari muchikamu ichi vane mukana wekurarama kusvika 100%. Zvisinei, chirwere ichi chinogona kudzoka uye/kana kuita mamwe matambudziko kwenguva refu.

Vana vane multisystemic leukemia (LCH) inokanganisa chiropa, spleen, kana bone marrow vanowira muchikamu che "high risk".

Mwana wangu anofanira kuona chiremba rini?

Kunyangwe mwana wako apedza kurapwa, chiremba anofanira kugara achiona mwana wako. Izvi zvinodaro nekuti njodzi yekuti chirwere ichi chidzokorore yakakura. Saka, mwana wako anofanira kutariswa kwemakore akati wandei. Munguva yekuongororwa uku, bvunzo dzakafanana dzakaitwa mwana paakaonekwa kuti ane chirwere, dzakadai se ultrasound, MRI, CT scan, uye PET scan, dzinofanira kudzokororwa. Chiremba achakuudza kuti unofanira kuuya nemwana wako kangani.

Mibvunzo ipi yandinofanira kubvunza chiremba wemwana wangu?

Ungave uine mibvunzo yakawanda nezvekuongororwa kwemwana wako. Zvakanaka kuinyora pasi wozouya nayo kunoona chiremba wako anotevera. Heano mimwe mibvunzo yaunogona kubvunza:

• Kupararira kwemasero eLangerhans kunokanganisa sei mwana wangu?
• Mwana wangu anoda kurapwa here?
• Ndezvipi zvirongwa zvekurapa zviripo kumwana wangu?
• Ndezvipi migumisiro yekurapwa?
• Mwana wangu achapora zvizere here nemishonga iyi?
• Chii chiri kutarisira chirwere chemwana wangu?
• Pane mapoka ekutsigirana aunogona kukurudzira here?

Pakupedzisira, Mharidzo Yekuenda Nayo Kumba

Langerhans Cell Histiocytosis (LCH) chirwere chisingawanzoitiki chinowanzobata vana. Kana mwana wako ari kusangana neizvi, ungave uchinzwa manzwiro akawanda. Ungave uchisuruvara uye uchitya mwana wako. Ungave uchitya mukati mako, asi ungave uchiedza kuva akasimba kunze. Manzwiro ako ese ane musoro. Asi yeuka, haufanirwe kupfuura nerwendo urwu uri wega. Chikwata chevanachiremba vemwana wako chinoziva zvauri kusangana nazvo. Varipo kuti vakubatsire nhanho dzese dzenzira, kuti vanzwisise mamiriro emwana wako, uye kuti vakupe simba rekutsungirira.

Yeukai, kuongororwa kwechirwere nekukurumidza uye kurapwa kwakakodzera kunogona kupa mwana wenyu hutano huri nani. Musatya, tarisanai neizvi neushingi.

` Langerhans Cell Histiocytosis, LCH, vana, kenza, majini ekuchinja, zviratidzo, kurapwa