Langerhans selo histiocytosis: Zoyambitsa ndi Chithandizo

Langerhans maselo - maselo oyera amene ali mbali ya chitetezo cha m'thupi ndi zinthu zachilendo mbali yofunika kuteteza thupi tizilombo, tizilombo ena tizilombo zoipa. Zikupezeka mu khungu, lymph mfundo, ndulu, mafupa ndi mapapo.

Kodi Langerhans selo histiocytosis?

Mu matenda, poyamba ankatchedwa histiocytosis X, maselo Langerhan kuyamba zikupitiriza mu lonse pathological. M'malo mothandiza thupi, woipa yaikulu ya maselo amenewa bwanji kukhulupirika kwa zimakhala ndi kuwononga iwo. Nthawi zambiri amadwala mafupa, mapapo ndi chiwindi. Ngakhale kuchuluka selo amafananadi ndi khansara, akatswiri ambiri sakhulupirira histiocytosis X khansa. Waukulu differentiating mbali mu nkhani iyi, amazindikira kapangidwe yachibadwa maselo ankhanza ndi vuto yekha ndi kukula mofulumira. Panopa Langerhans selo histiocytosis (ana, tisaiwale, ndi ambiri kuposa akuluakulu) anazindikira matenda a chitetezo cha m'thupi, umene maselo mthupi kuchulukitsa mofulumira kwambiri ndipo chifukwa kutupa ndi kuwonongeka kwa minofu ozungulira.

Matenda akhoza kukhala mu gawo lirilonse la thupi ndi chiwalo chilichonse. Angathe zimachitika m'madera osiyanasiyana imodzi. Nthawi zambiri Komabe, kudwala amakhudza machitidwe angapo a thupi okha ana osaposera zaka ziwiri. zotupa Yekhayekha zimapezeka ana ndi akulu omwe.

zifukwa

Iwo akadali osadziwika chifukwa pali kudwala. Komabe, asayansi lolunjika pa yotsimikiza ya amanena izi: zambiri mtundu wa nkhani wamba mu chilengedwe, mwina tizilombo wakupatsa kutsogolera chitukuko cha matenda kuchititsa kwambiri kwambiri anachita ndi chitetezo cha m'thupi. Komabe, gwero mwachindunji matenda panthawi phunziro kwa Chizindikirocho. Munthu wodziwika kuonerera ndi chidwi: pafupifupi odwala onse ndi matenda isanafalikire mu phapo zachangu kapena kale osuta ndudu. Komabe, ngakhale kupezeka kwa kusuta anthu chosowa kwambiri Langerhans histiocytosis. zifukwa monga ntchito fodya mndandanda wa mfundo zoopsa ngati ayi, chifukwa nthawi zambiri matenda ndi sikumangotikhudza m'mapapo. The kungoganizira kwambiri N'zachidziwikire kuti mavuto obwera chifukwa cha kusuta anazindikira kuchuluka kwa maselo Langerhans odwala ndi osowa chibadwa anatsimikiza kutengera za izo. Malinga ndi chiphunzitso china, chimayambitsa matenda lagona titadziwa matenda chitetezo cha m'thupi.

inafikira

Akuti matenda ali osowa ndi amapezeka pafupifupi umodzi ana 250 zikwi ndi chitsanzo chimodzi pa miliyoni wamkulu. Ngakhale zizindikiro kumachitika pa msinkhu wina uliwonse, kawirikawiri kwambiri matenda ana ndi achinyamata, ndi 70% milandu zimachitika kudziwa histiocytosis zaka ndi zaka 17. Mu msinkhu wamng'ono odwala, ziwerengero adzauka mu zaka chaka chimodzi kufikira zitatu.

mawonekedwe a matenda

Langerhans selo histiocytosis ana ndi akulu kumafuna mitundu 3 musanayambe zochitikazi pathologies palokha. Iwo eosinophilic granuloma, Henda matenda - Shyullera - matenda Mkhristu ndi Letterer - Siwa.

zizindikiro

Matendawa angasonyeze ndi mbali zotsatirazi:

• kulimbikira ululu ndi kutupa mu mafupa, makamaka dzanja lanu kapena mwendo;
• ndi mng'alu mu fupa, makamaka ngati si yoyamba ndi choipa kapena yoyamba kuunika, pang'ono nkhonya;
• mipata Large pakati pa mano milandu kumene Langerhans selo histiocytosis ana wakafika mafupa a pachibwano;
• khutu matenda kapena kumaliseche khutu, ngati matenda chatithandizira mafupa a Chigaza pafupi khutu;
• ziwengo preferentially isanafalikire pa matako kapena khungu;
• lymphadenopathy;
• chiwindi kukulitsa, zomwe zina akhozanso kukopeka ndi zizindikiro za chiwalo kukanika (chikasu pathupi ndi m'maso (matenda a chikasu ndiyo) kapena pathological kudzikundikira madzimadzi pamimba - ascites);
• exophthalmos - ndi kusamuka kwa diso patsogolo chifukwa histiocytosis ndi kutanthauzira za maso,
• kufupika kwa mpweya ndi chifuwa, m'mapapo ndi zotupa;
• kukula pang'onopang'ono ndipo pokodza kwambiri chifukwa vutoli m'thupi.

zizindikiro zochepa wamba matenda ndi:

• malungo;
• kuwonda;
• irritability;
• kulephera kugwira thupi ndi thupi mu mawonekedwe.

diagnostics

Popeza Langerhans selo histiocytosis (Photo kuwonekera kwa matenda si pamaso wokongola) ali osowa kwambiri ndipo chimaoneka ndi zizindikiro zimene mosavuta kolakwika kwa zizindikiro za matenda ena ambiri, matenda zambiri zovuta zimatenga nthawi. N'chifukwa chake dokotala mosakayika ndikufunseni inu mafunso okhudza matenda ena bwino kusiyanitsa ndi histiocytosis anafuna.

Kufufuza chachikulu matenda ndi thupi Kupenda wabwinobwino, imene adokotala amaweruza thanzi akukhudzidwira ndi amalipira chidwi kwambiri m'madera amene zizindikiro za matenda kuonekera. Ngati wodwala amakhala kupweteka mafupa kapena kudandaula za kutupa mu fupa, adokotala akhoza mankhwala muyezo X-ray ndi fupa scintigraphy. X-ray awulule wovulala gawo lotchedwa zotupa lytic, ndi scintigraphy adzaona m'dera kuvulazidwa kumene fupa paokha amayesetsa kuti achire.

Njira yopangidwa Tomography (CT) ntchito mu kafukufuku wa kumbuyo ndi mafupa a chiuno pa nthawi imene dokotala akuganiza kuti pangakhale matumba a kusintha zowononga. Zizindikiro zimasonyeza kukanika chiwindi, tifunika MAGAZI. Ngati pali zizindikiro za kuwonongeka m'mapapo, kapena pa nthawi imene katswiri pa maziko a kuona koyamba mankhwala akuganiza kuti Langerhans selo histiocytosis angasonyeze mu mapapo, mankhwala X-ray kapena yopangidwa tomography a chifuwa. CT jambulani mutu anachita kuwunika boma la ubongo. The watsopano Kuona luso, PET (positron umuna tomography) kungakhale kothandiza.

Ngati mukukayikira kuti munthu Langerhans selo histiocytosis ana (onani zithunzi. Kumayambiriro kwa nkhani) biopsy kungakhale kofunika, ili njira yokhayo yotsimikizira matenda presumptive. Mu njirayi, dokotala amachotsa chitsanzo ka minofu kapena fupa kuti ankaphunzira zasayansi. Nthawi zambiri, chitsanzo cha zinthu zamoyo ndi yotengedwa m'madera mwachindunji isanafalikire Langerhans selo histiocytosis: mafupa, khungu ndi lymph mfundo. MAGAZI angakuthandizeni kudziwa ngati mafupa zimakhudzira.

Kutalika

N'zovuta kulosera mmene msanga matendawa ndi mmene kuonekera. Mu zina, kudwala Mwamsanga palokha ndipo sikutanthauza alowererepo zachipatala. Izi zimachitika pamene kuphwanya kwa isanafalikire m'dera lina (mu fupa kapena khungu), kapena pamene wosuta ndi kuwonongeka m'mapapo ikutha kudya ndudu. Komabe, zimachitika kuti matenda ndi koopsya; imfa akhoza ananeneratu milandu anthu pamene kudzikundikira maselo abnormally proliferating ali pomenyana nthawi yomweyo angapo ziwalo zosiyana kapena ziwalo. Ukupeza ya wosuta umayamba kusintha kokha pamene kusiya.

kupewa

Popeza chifukwa cha kuyan'anila akhala chinsinsi, mpaka palibe miyeso ogwira kupewa. Komabe, anapatsidwa kuti mapapo amakhudzidwa yekha pa mafani kusuta fodya, kusiya khalidweli Tingaone monga histiocytosis njira isanafalikire mu mapapo.

chithandizo

Ngati wodwalayo anatsimikizira matenda a "histiocytosis maselo Langerhans", chithandizo kudzadalira zovuta ndi mmene pakati.

Ngati chosayenerera ndi wapezeka mu kachitidwe kamodzi kokha za thupi, dokotala mankhwala corticosteroids, makamaka "Prednisolone". Komanso, inu mukhoza kulandira njira mankhwala amphamvu. Komanso, zinkakhala m'dera la fupa anachotsa curettage - kuchipala. Ngati maselo pathologically accrescent wambiri M'dera lina la khungu, matenda kuchiritsidwa ndi kuphimba zinkakhala m'dera asafe mpiru - Morning wothandizira. Amagwiritsidwanso ntchito "Methotrexate". Radiotherapy ndi kutumikiridwa mu nthawi imene matenda fupa (mwachitsanzo, mu mwendo chapamwamba kapena msana) zinachititsa kuti chifooke lonse mafupa mpukutu. Musculo-pulasitiki opaleshoni msana - maphatikizidwe - tikulimbikitsidwa pamene fupa Kusakhazikika m'dera khomo lachiberekero.

Zowombetsa mkota (zokhudza zonse) Langerhans selo histiocytosis - chikhalidwe lalikulu kwambiri. Mankhwala mankhwala amphamvu Angagwiritsidwenso ntchito Komabe, nchito zake ndi yafupika poyerekeza mankhwala ndi chotupa umodzi. Ngakhale akatswiri anafufuza mphamvu ya mankhwala ambiri, mulingo woyenera mankhwala silinakwaniritsidwe wapezeka. Komabe, kuti kuwunika chitukuko cha matenda dokotala anu angakupatseni imodzi mwa njira zotsatirazi:

• "Vinblastine";
• "Etoposide";
• "Mercaptopurine";
• "Cladribine";
• "Cytarabine";
• "Methotrexate".

Nthawi zina, madokotala amalangiza chiwindi kupatsidwa zina, mafupa, kapena m'mapapo, koma mwa ofufuza kugwirizana pa mulingo woyenera limba kupatsidwa zina odwala ndi histiocytosis silinakwaniritsidwe anafika.

Pamene kuwona dotolo?

Onetsetsani kuti funsani chisamaliro chanu dokotala yaikulu ngati mukukayikira Langerhans selo histiocytosis ana. Zizindikiro Ocular, ululu kapena kutupa mu fupa, khungu aakulu totupa ndi zizindikiro za matenda osowa. Ngati aliyense wa zizindikiro kapena kudwala akuluakulu ayenera kufunsa dokotala.