Langerhans Sero histiocytosis: Zvinokonzera neMarapirwo

Langerhans masero - chena ropa masero kuti vari nhengo masoja emuviri uye chaiye ezvinhu basa rinokosha pakudzivirira muviri nemavhairasi, mabhakitiriya uye nezvimwe zvinokuvadza tusingaoneki. Zvinowanika paganda, node nodes, chirwatata, mwongo uye nemapapu.

Chii Langerhans muchitokisi histiocytosis?

In chirwere ichi, kare sezvo histiocytosis X, masero Langerhan vanotanga kuwanda mu pathological akamboona. Pane kuti vadzivirire muviri, guru kuwanda masero aya sei kuvimbika nyama uye kunyange kuvaparadza. Most vanowanzotambura kubva mapfupa, mapapu uye chiropa. Kunyange zvazvo izvi Sero kuwanda ane zvakawanda zvakafanana nekenza, vaongorori vakawanda havatendi histiocytosis X fomu kenza. The chikuru differentiating nzi iyi, vanoziva chakaipa maumbirwo masero hasha uye chete dambudziko iri kukura kwavo nokukurumidza. Pari, Langerhans sero histiocytosis (mu vana, zvinofanira kucherechedzwa, zvinonyanya akakurumbira kupfuura vakuru) rinozivikanwa chirwere of zvirwere, apo masero zvirwere muwande zvakare nokukurumidza uye kukonzera kuzvimba uye kukuvara akapoteredza tishu.

chirwere ichi anokwanisa kuva chero nhengo yomuviri uye chero nhengo. Unogona kuitika munzvimbo dzakasiyana siyana panguva imwe chete. Zvisinei, kazhinji, kuti zvokurwara kunokanganisa akawanda enyika muviri chete muvana pasi pemakore maviri. Roga maronda vanowanikwa vose vana nevakuru.

zvikonzero

It achiri kuzivikanwa nei pane zvokurwara yakadaro. Zvisinei, masayendisiti nezvezvaiitwawo ongororo inotevera pfungwa: Ungangove rudzi zvavo nyaya zvakatipoteredza, zvichida nemavhairasi chirwere inopa zvakasimbisa pokumuka zvinetso kukonzera zvakanyanyisa zvikuru maitiro achishandisa zvirwere. Zvisinei, mashoko zvakananga anokonzera chirwere panguva pasi zvitupa. Imwe anozivikanwa akataura unofadza: zvinenge varwere vose vemunyika chirwere ari zvemapapu zvinoshingaira kana vaimbova mudzanga vanosvuta. Asi, kunyange kana vakawanda kuparadzirwa kusvuta munzanga hariwanikwi chaizvo Langerhans histiocytosis. Zvikonzero zvinosanganisira kushandisa fodya iri pamazita zvinokonzera njodzi yakaita kwete, nokuti kazhinji chirwere uye asingauyi kukanganisa mumapapu. The ungangotarisa ndeyokuti akaipa Migumisiro nokusvuta vakaziva yokuwedzera Langerhans masero Varwere ane chisingawanzowaniki majini kutsunga kubatwa nayo. Maererano neimwe pfungwa, chinokonzera hosha iri tinozvarwa zvirwere zvirwere.

waridza

Vaitirei chirwere hariwanikwi uye rinowanikwa munenge imwe nyaya vana 250 chiuru uye nyaya imwe miriyoni mukuru. Kunyange zvingaratidza kuitika chero zera, izvozvo kazhinji chirwere muvana uye kuyaruka, uye 70% nyaya kuitika panoongororwa histiocytosis zera makore 17. In gotwe zera boka varwere, nhamba simuka makore kubva matatu.

chimiro chirwere

Langerhans Sero histiocytosis mune vana nevakuru kunosanganisira 3 nezvimiro kare vaiona akazvimirira pathologies. It eosinophilic granuloma, Henda chirwere - Shyullera - chirwere Christian uye Letterer - Siwa.

zviratidzo

The chirwere kungazviratidza parutivi anotevera zvinhu:

• Kuramba kurwadziwa uye kuzvimba ari mapfupa, kunyanya mune ruoko rwako kana gumbo;
• mitswi pfupa, kunyanya kana isina pashure pachedu kukuvara kana pashure chiedza, zvishomanana furidza;
• Makwanza Large pakati pameno muzviitiko apo Langerhans sero histiocytosis muvana rapararira kusvika mapfupa shaya;
• nzeve chirwere kana nzeve zvinoyerera, kana hosha kwakakanganisa mapfupa dehenya pedyo nzeve;
• ganda upundu preferentially vemunyika pamusoro magaro kana ganda romusoro;
• lymphadenopathy;
• chiropa nedumbu, izvo dzimwe nguva angaperekedzwa zviratidzo nhengo dysfunction (yellowing ganda uye meso (jaundice) kana pathological kuunganidza Urume mudumbu - ascites);
• exophthalmos - the misha yeziso mberi yakafanira histiocytosis pamwe localization kwemaziso;
• kupfupika mweya uye chikosoro, zvemapapu pamwe maronda;
• kunonoka kukura uye kunyanya weti nemhaka hormone kusaenzana.

Ishoma zviratidzo chirwere vari:

• fivhiri;
• uremu;
• kugumbuka;
• nekusagona abate uremu uye chengeta muviri chimiro.

Diagnostics

Sezvo Langerhans Sero histiocytosis (Photo kuratidzirwa chirwere ichi hachisi rakanaka pameso) rakanyatsojeka isingawanzowanikwi uye kunoratidzwa zviratidzo zviri nyore vakarasika kuti zviratidzo nezvimwe zvirwere zvakawanda, chirwere kazhinji ndiyo yakaoma uye zvinotora nguva. Ndokusaka chiremba zvingangowedzera kukubvunza mibvunzo chokuita nezvimwe zvirwere kuti zvinobudirira kusiyanisa anga histiocytosis.

The chikuru chirwere kuongorora chinhu chakaipa wenyama kuongororwa, apo chiremba Anoongororawo hombe utano uye anonyanya kukoshesa avo dzine zviratidzo chirwere kuonekwe. Kana murwere anosangana marwadzo ari mapfupa kana anonyunyuta kuti kuzvimba ari pfupa, chiremba anogona mishonga mureza maekisirei uye mapfupa scintigraphy. Maekisirei zvinoratidza kutsemuka mugove inonzi lytic maronda, uye scintigraphy achasarudza nzvimbo yokukuvadzwa apo pfupa vakazvimiririra anoedza apore.

Method computed Tomography (CT) rakashandiswa kudzidza musana uye chiuno muzviitiko apo chiremba kufungidzira kuti pangava muhomwe kuchinja zvinoparadza. Kana zviratidzo anoratidza chiropa dysfunction, vachada miedzo ropa. Kana pane zviratidzo kukuvara yemapapu, kana mune zviitiko apo nyanzvi pahwaro kwokutanga kuongororwa ezvokurapa kufungidzira kuti Langerhans sero histiocytosis anogona kuratidza nayo mumapapu, akarayira maekisirei kana computed tomography pechipfuva. CT scan musoro akaita kuti kuongorora mamiriro wouropi. The itsva kuedza kufungidzira michina, PET (positron achine tomography) kunokwanisa kubatsirawo.

Kana uchifungidzira munhu Langerhans sero histiocytosis nevana (ona mapikicha. Pakutanga nyaya) biopsy zvingava zvakakodzera, sezvo iyi ndiyo chete nzira kuzosimbisa presumptive zvawanikwa. In nzira iyi, chiremba inobvisa duku nzwisa zvinyama kana pfupa kuti akadzidza murabhoritari. Kazhinji, nzwisa zvipenyu zvinhu iri rakatorwa kunzvimbo zvakananga vemunyika Langerhans Sero histiocytosis: mapfupa, ganda uye node nodes. miedzo ropa anogona kukubatsira kuona kana mumwongo zvinovhiringidzika.

chinguva

Zvakaoma kufungidzira kuti nokukurumidza chirwere uye sei vacharatidza pacharo. Mune zvimwe zviitiko, zvokurwara haazombotaurwi roga uye haatarisiri zvokurapa kupindira. Izvi zvinoitika kana kutyora iri mupasi rimwe nzvimbo (mu pfupa kana ganda), kana kana uchisvuta kuchikuvadza yemapapu haizorambi zvinodya midzanga. Zvisinei, zvangoitikawo kuti chirwere rakaipa; rufu kunogona akarehwa Muzviitiko apo kuunganidza zvisinganzwisisiki kuwedzera masero kurwisa panguva dzakawanda dzakasiyana nadzo kana dzomuviri. Mamiriro hutano uchisvuta anotanga kuvandudza chete kana kurega.

dziviriro

Sezvo mhaka kutyorwa chinoramba haanzwisisiki, saka kure hapana zvinobudirira ekudzivirira matanho. Zvisinei, vakapiwa kuti mapapu vanokanganiswa chete panguva mafeni kuputa midzanga, kurega chimwe muropa anogona kuonekwa sechinhu chokudzivirira histiocytosis vemunyika mumapapu.

mabatiro

Kana murwere ane yasimbiswa chirwere kuti "histiocytosis pakati Langerhans masero", mishonga achaenderana kwokuomerwa uye kukura pakakoromoka.

Kana abnormality ari kuona imwe chete ino womuviri, chiremba vanonyorera corticosteroids, zvikurukuru "Prednisolone". Uyezve, unogona kuva narwo nzira mishonga. Mukuwedzera, njodzi nzvimbo pfupa kubviswa nokuda curettage - tichibvisa. Kana masero pathologically accrescent vari kusimbiswa imwe nzvimbo ganda, chirwere chinogona kurapwa kuburikidza kufukidza zvakakanganisa nzvimbo senitrogen yemasitadha - mishonga mumiririri. Rinoshandiswawo "Methotrexate". Radiotherapy chinotarisirwa muzviitiko apo pfupa chirwere (semuenzaniso, iri repamusoro gumbo kana ari nomuzongoza) zvakakonzera kuti kwakaderera apa yose chinyorwa. Musculo-epurasitiki kuvhiyiwa pamusoro musana - Fusion - inokurudzirwa kana mapfupa kusagadzikana mune remuromo nharaunda.

Generalized (systemic) Langerhans foni histiocytosis - a zvikuru chakakomba ezvinhu. Nokuti Mabatirwo mishonga kungashandiswawo Zvisinei, kushanda kwaro zvikuru wakaderedzwa kuenzaniswa kurapa pamwe chete lesion. Kunyange zvazvo nyanzvi amutsvaka kubudirira zvinodhaka zvakawanda, Zvakanyanya Kunaka Panguva Yepamuviri kurapwa haisati yawanikwa. Kunyange zvakadaro, kuti kuongorora kukura chirwere chiremba wako anogona mishonga mumwe anotevera nzira:

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

Mune zvimwe zviitiko, vanachiremba vanokurudzira chiropa transplantation, mumwongo kana zvemapapu, asi pakati vaongorori nekubvirana pasi Zvakanyanya Kunaka Panguva Yepamuviri nhengo transplantation pavarwere vane histiocytosis haisati dzamboparidzirwa.

Kana kuona chiremba?

Iva nechokwadi kuti kuonana chikuru hanya wevana vako kana uchifungidzira Langerhans sero histiocytosis vana. Ocular zviratidzo, kurwadziwa kana kuzvimba ari pfupa, chisingaperi ganda rashes ndivo zviratidzo chirwere ichi isingawanzowanikwi. Kana zvezviratidzo izvi kana zvokurwara nedzevakuru vanofanira kuona chiremba.