Langerhans cell histiocytosis: Oonobangela and Treatment

iiseli Langerhans - iiseli ezimhlophe zegazi eziyinxalenye kwamajoni omzimba kwaye kwiimeko eziqhelekileyo zidlala indima ebalulekileyo ekukhuseleni umzimba nxamnye iintsholongwane, iintsholongwane kunye namanye amagciwane eziyingozi. Bona afumaneka eluswini, iindawo nkovu, udakada, lomongo wethambo kunye nemiphunga.

Yintoni Langerhans cell histiocytosis?

Kule sifo, eyayisaziwa ngokuba histiocytosis X, iiseli Langerhan uqale ukwanda yandile yezifo. Kunokuba ekukhuseleni umzimba, zoqhaphelo olukhulu ezi seli kuchaphazela ukuthembeka izicwili nkqu awatshabalalise. Amaninzi ubunzima ukusuka amathambo, imiphunga kunye zesibindi. Nangona oku kwanda zeeseli zininzi izinto esifana ngazo umhlaza, inkoliso abaphandi musani ukukholwa ifomu histiocytosis X lomhlaza. Eyona umehluko eziphambili kulo mongo, uliqonda isakhiwo okuqhelekileyo iiseli ndlongondlongo kunye ingxaki kuphela ukukhula kwabo yanda. Okwangoku, Langerhans iseli histiocytosis (ebantwaneni, kufanele kuphawulwe, kuqhelekile ngaphezu abadala) ibonakaliswe isifo kwamajoni omzimba, apho iiseli omzimba ndikwandise kakhulu ngokukhawuleza ibangele nangokurhala, ukonakala inyama ezingqongileyo.

Esi sifo uyakwazi ukuphuhlisa kuyo nayiphi na ilungu lomzimba kunye naliphi na iziko likarhulumente. Kusenokuba nokuba kwenzeka kwiindawo ezininzi ezahlukeneyo ngaxeshanye. Noko ke, kwiimeko ezininzi, ngezifo kuchaphazela iinkqubo ezininzi omzimba kuphela abantwana abangaphantsi kweminyaka emibini ubudala. izilonda zodwa afumaneka abantwana kunye nabantu abadala.

izizathu

Kuyinto okungaziwayo kutheni kukho ngezifo enjalo. Noko ke, izazinzulu ziye swi lokungqinisisa hypothesis zilandelayo: kokuqhelekileyo uhlobo umcimbi eziqhelekileyo kokusingqongileyo, mhlawumbi igciwane lohlobo ukuba ikhuthaze uphuhliso ukuphazamiseka ezibangela basabela kakhulu ngokugqithiseleyo yi amajoni omzimba. Noko ke, umthombo ngqo sifo ngalo mzuzu phantsi yesazisi. Enye ubonwa eyaziwayo umdla: phantse zonke izigulane kunye nesifo eyendayo kwemiphunga ezi ugwayi osebenzayo okanye bangaphambili. Noko ke, enoba ezisasazwayo ukutshaya eluntwini Langerhans ezinqabileyo kakhulu histiocytosis. Ezi zizathu zibandakanya ukusetyenziswa yecuba kuluhlu ubungozi ayakunanza, ngenxa yokuba kaninzi banesigulo kwaye aluchaphazeli emiphungeni. Eyona Kungenzeka hypothesis kukuba imiphumo emibi zokutshaya ukwanda ngabona iiseli Langerhans kwizigulane kunye yofuzo zemfuza kumiselwa ezinqabileyo kuyo. Ngokutsho kwenye inkolelo, ingcambu unobangela sifo siyifumana ukuphazamiseka omzimba angaphakathi.

ukwanda

Njengento sifo enqabileyo yaye kwenzeka malunga elinye icala 250 amawaka abantwana icala elinye ngalinye million omdala. Nangona iimpawu kunokwenzeka nakweliphi na ubudala, kakhulu sivame ukuxilongwa abantwana nabo bakwishumi elivisayo, 70% amatyala kwenzeka ekuchongeni yobudala histiocytosis ukuya kwiminyaka eli-17. Kwi qela lobudala omncinane izigulane, amanani kuvuka ngeminyaka ukusuka kwiminyaka yokuqala ukuya kweyesithathu.

uhlobo sifo

Langerhans cell histiocytosis abantwana nabantu abadala ibandakanya iintlobo 3 ngaphambili ingqalelo ezimbi ezizimeleyo. It granuloma eosinophilic, isifo Henda - Shyullera - isifo abangamaKristu Letterer - Siwa.

iimpawu

Isifo ngazo ngokwawo ezi mpawu zilandelayo:

• ubuhlungu zokunyanzelisa ukudumba emathanjeni, ingakumbi ingalo yakho okanye umlenze;
• sibange kwi ithambo, ingakumbi ukuba akukho lwandulelwe yokwenzakala emzimbeni okanye kwakhokelwa ukukhanya, nibethe kancinane;
• izikhewu ezinkulu phakathi kwamazinyo kwiimeko apho Langerhans iseli histiocytosis ebantwaneni kwafikela amathambo emhlathini;
• indlebe usuleleko okanye indlebe obhobhozayo, ukuba esi sifo olubachaphazela amathambo intamo kufutshane nendlebe;
• irhashalala preferentially localized ezinqeni okanye entlonzeni;
• lymphadenopathy;
• kwesibindi yesinye, nto leyo kwezinye iimeko sikhatshwe ngemiqondiso yelungu kwekhono (yellowing ulusu kunye namehlo (senyongo) okanye ukuqokelelana zophendlo lwezifo kolwelo esiswini - ascites);
• exophthalmos - ukubana lweliso lakho phambili histiocytosis ngenxa kunye kwasendaweni yamehlo;
• ukuphelelwa umoya kunye ukukhohlela, umphunga ne izilonda;
• ukukhula kade kunye Ukuchama ngokugqithisileyo ngenxa ukungalingani hormone.

Iimpawu Ngaphantsi oqhelekileyo sifo zezi:

• umkhuhlane;
• komzimba;
• ucaphuka;
• ekungakwazini ukubamba ubunzima uze ugcine umzimba imilo.

ekuxilongweni

Ekubeni Langerhans cell histiocytosis (Ifoto abonakalisa kwesi sifo ayisiso emehlweni entle) Kuyinto enqabileyo kubonakaliswa iimpawu ukuba lula ngokuphosakeleyo iimpawu nezinye izifo ezininzi, uphethwe kudla nto inzima kwaye kuthatha ixesha. Kungenxa yoko le nto ugqirha abuze imibuzo enxulumene nezinye izifo ukuba umahluko ngempumelelo ngakumbi histiocytosis ekujongwe.

Uphando lokuqala diagnostic yi uviwo eziqhelekileyo emzimbeni, apho ugqirha oluvavanya impilo jikelele kwaye sinika ingqalelo ngokukodwa kwezo ndawo apho kuvela iimpawu zesifo. Ukuba isigulana lifumana intlungu kwi amathambo okanye ekungxolisa of Ukudumba apha ithambo, ugqirha unakho ukumisela umgangatho-X-reyi kwaye scintigraphy ithambo. -X-reyi ibonisa ngokwaphulwa isahlulo ekuthiwa izilonda lytic, yaye scintigraphy uya lijonge indawo yokwenzakala apho ithambo ngokuzimeleyo uzama kwakhona.

Indlela ngekhompyutha Itomography (CT) ezisetyenziswa ekufundeni emqolo isinqe kwiimeko apho ugqirha akrokrela ukuba kusenokuba kukho iindima utshintsho yentshabalalo. Xa iimpawu kubonisa zesibindi kwekhono, kuya kufuneka iimvavanyo zegazi. Ukuba kukho iimpawu umonakalo lung, okanye kwiimeko apho ingcali ngokwesiseko uvavanyo lonyango lokuqala akrokrela ukuba Langerhans seli histiocytosis ukuze ayibonise emiphungeni, amisele-X-reyi okanye ngekhompyutha Itomography kwesifuba. CT scan intloko wenza ukuvavanya imeko ingqondo. Iteknoloji entsha ngeso, PET (i ukukhutshwa Itomography) nazo zinokuba luncedo.

Ukuba urhanela Langerhans iseli histiocytosis ebantwaneni (bona iifoto. Ekuqaleni kweli nqaku) biopsy kusenokuba yimfuneko, njengoko oku yeyona ndlela kuphela ukuqinisekisa uxilongo presumptive. Kule nkqubo, ugqirha lisusa izicuku kwithishyu okanye ithambo iza kufundwa elebhu. Kwiimeko ezininzi, isampuli izinto eziphilayo licatshulwa kwiindawo apho ngqo localized Langerhans cell histiocytosis: amathambo, ulusu kunye iindawo nkovu. iimvavanyo zegazi kunceda ukuqinisekisa ukuba umongo linayo.

ubude

Kunzima ukuqikelela ukuba abonakalalise ngayo ngokukhawuleza isifo nendlela ngokwaso. Kwezinye iimeko, le ngezifo liyalahleka ngokwayo kwaye ayifuni ungenelelo lonyango. Oku kwenzeka xa ukuhlukunyezwa yenzelwe kwindawo enye (in ithambo okanye eluswini), okanye xa ixhwele kunye nomonakalo lung liyayeka ukuba kudla midiza. Noko ke, oko kuyenzeka ukuba esi sifo kuba siyabulala; ukufa linakho ukuqikelelwa kwezo meko xa beqokelela iiseli ziyanda engaqhelekanga bahlasele ngexesha elinye iqela amalungu ahlukeneyo okanye amalungu omzimba. Imo zempilo otshayayo uqalisa ukuphucula kuphela xa ukuyeka.

ukuvimbela

Ekubeni unobangela zokuphula kuhlala imfihlelo, ngoko kude akukho manyathelo kuthintelo. Kambe ke, njengoko emiphungeni ayachaphazeleka kuphela abalandeli ukuba ukutshaya, ukuyeka nomkhwa kunokubonwa njenge histiocytosis othintelo eyendayo emiphungeni.

unyango

Ukuba isigulana kukho uxilongo be "histiocytosis leeseli Langerhans", unyango iya kuxhomekeka ebukhulwini kunye nobungakanani iintanda.

Ukuba nemvelo labonwa kwinkqubo enye kuphela umzimba, ugqirha uyakukumisela corticosteroids, ngo "Prednisolone" ethile. Ukongezelela, kusenokufuneka ukuba bangenele izifundo nechemotherapy. Ukongeza, kummandla ochaphazelekayo ngethambo asuswe curettage - sisusa. Ukuba iiseli pathologically accrescent bebekelwa kwenye indawo yolusu, esi sifo sinokunyangeka xa ukuyigubungela ochaphazelekayo ndawo nitrogen lwemostade - arhente kusetyenziswe amachiza. Iyasetyenziswa "Methotrexate". Radiotherapy ibhalwa kwiimeko apho izifo ithambo (umz, emlenzeni eliphezulu okanye emqolo) yakhokelela buthathaka lonke amathambo ukuqhekeka. uqhaqho iintungu eplastikini emqolo - fusion - kucetyiswa xa ukungazinzi ithambo kummandla wesibeleko.

Ngokubanzi (systemic) Langerhans cell histiocytosis - imeko emibi kakhulu. Kuba unyango nechemotherapy singasebenza, Noko ke, ukusebenza wayo kakhulu kuyehla xa kuthelekiswa kuthomalaliso kunye sisilonda esidumbileyoesingaphakathi elinye. Nangona iingcaphephe bahlola ukusebenza kwamachiza ezininzi, unyango eliphezulu okwangoku kufunyenwe. Nakuba kunjalo, ukwenzela ukuba kubekwe esweni uphuhliso sifo ugqirha wakho ukumisela enye yezi ndlela zilandelayo:

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

Kwezinye iimeko, oogqirha bacebisa isibindi abohlukeneyo, lomongo wethambo, okanye umphunga, kodwa phakathi abaphandi ukuvumelana eyiyo abohlukeneyo kwizigulane kunye histiocytosis okwangoku kufikelelwe.

Xa ukubona ugqirha?

Qiniseka ukuba uqhagamshelane wabantwana zokhathelelo ezingundoqo ukuba ukrokrela Langerhans cell histiocytosis ebantwaneni. Iimpawu ocular, iintlungu okanye ukudumba kwi ithambo, irhashalala ziqatha iimpawu zesi sifo ezinqabileyo. Ukuba nayiphi na kwezi mpawu okanye leengxaki abadala kufuneka adibane nogqirha.