Uxilongo kunye nonyango wegazi ahlekise lymphoblastic. iimpawu

leukemia Lymphoblastic, igazi uthathwa ukuba ixhaphakileyo abantwana ngezifo oncological. Oku malunga nama-25% of zomhlaza ibonakele wezifo. Emva koko, thatha igazi leukemia sihlolisise - ntoni na, kutheni na apho noko ungenelelo zonyango kwenziwa kule ngezifo.

Overview

igazi leukemia - ntoni na? Isifo libhekisela kwiqela lomhlaza. BONKE (lymphoblastic etsolo leukemia), iimpawu eziza kuxoxwa ngezantsi, liqela owahlukeneyo of neoplasms zeeseli ukuba babe neempawu ezithile yemfuza immunophenotypic. Ukwandisa imveliso kunye nokwanda ezi iiseli ezingaqhelekanga lomongo wethambo igalelo ukwanda lesibini, iyantlukwano okanye zombini. Ngenxa yoko, nathi afunxwe iiseli amalungu parenchymatous neendawo nkovu. Uninzi (80%) kuzo zonke leukemia (leukemia), kubonakele ebantwaneni, linentsusa lymphoid.

ukwanda

izifo egazini amabi, ehlabathini namhlanje afumaneka kuyo yonke indawo. ZONKE ukwanda kumazwe aphuhlileyo yi amatyala 3-4 ngamnye ikhulu lamawaka abantwana kunyaka ngamnye. Xa amazwe Central Asia kunye Afrika ezisemthethweni zoxilongo yiyona iqhelekileyo. ZONKE kaninzi US States of Japan, China, eYurophu. Le izehlo Incopho ebantwaneni kucingelwa ukuba kwiminyaka engama 2.5-5. Ezingaqhelekanga kakhulu eqhelekileyo lwenzeka kakhulu amakhwenkwe.

predisposing zinto

Okokuqala kufuneka kwaziwe ukuba ivele ngenxa yobudala kukamama care kwangaphambi, ubunzima high ukuzalwa - ngaphezulu kwe-4000 grams, exposure kunye siswini ukuba ionizing kwimitha. Kwezinye iimeko mhlawumbi nokuba kwenzeke ileukemia oyingozi lymphoblastic? Imbali lokugula sikanina inokuquka ulwazi malunga ngokufa ezingekazalwa kwi nokukhulelwa yangaphambili. Oku kwenzeka ngenxa yokuba iimeko engentle yokusingqongileyo, okanye ngenxa yofuzo. Kwimeko izigulane ezinomhlaza kwikhaya izalamane, kwande amathuba ukuba izizukulwana ezizayo ziya kuvela leukemia oyingozi lymphoblastic. Izinto ezibangela isifo ukuze ezinxulumene chromosomal xenga syndromes iziqalelo. Ezi ziquka, ingakumbi, iquka Down syndrome, neurofibromatosis, immunodeficiency bume, Fanconi anaemia, X-ezinxulumene agammaglobulinemia (iziphene) kunye nabanye. Ngenxa yoko, inani lezifundo, iingcali ziye kufika kwisigqibo ukuba wegazi etsolo lymphoblastic, kwakunye nokuphuhliswa kwezinye Oncology, kukho kuguquka yezizwe iiseli eliphuma kuye. Yena kamva okudityanisiweyo okuyinzala izindululi imiba yokusingqongileyo ukuba isebenze ukwanda. Oku kuyeyona nto eqhelekileyo ezinokubangela wegazi. Izizathu vozniknovneniya ngezifo yenye iinkalo ezibalulekileyo xa kukhethwa ayini.

ekuxilongweni

Xa ekuboneni nayiphi Cancer ngezifo ziya kuthathelwa ingqalelo kwiimpawu zeklinikhi iinkalo iiseli le zeendawana ithumba. ZONKE isifo lusekelwe ukuxilongwa ngokwasemzimbeni kunye nembali idatha zaselebhu izifundo paraclinical.

picture clinical

Iimpawu wegazi ezi syndromes ezintsonkothileyo:

• Ukunxila. Xa wegazi etsolo lymphoblastic na oluthile esiphawuliweyo, ukwehla kobunzima, ukudinwa, nomkhuhlane. Akwazi kuxhokonxwa lohlobo, igciwane, protozoal (ezinqabileyo), okanye ezinzwaneni, ingakumbi xa izigulane neutropenia.
• Hyperplastic. Xa leukemia etsolo lymphoblastic na nathi afunxwe udakada wesibindi. Oku kukhokelela hepatosplenomegaly, leyo abonakalise ububele esiswini. Imiqondiso yokuqala wegazi - Ukophuka zophendlo lwezifo amathambo elide okanye umqolo. Bomzimba kunye ekweneni namalungu inokuba ekuqaleni ngokuphosakeleyo iimpawu isifo samathambo okanye nezinye izifo. isifo Ithambo - for osteomyelitis. Xa nokungena leukemic le philisi articular kunye periosteum, intliziyo lomongo wethambo nethumba ukwandisa umthamo kuvela intlungu kakhulu. Ngelo xesha kwi-X-ray ibhaqwe uphawu utshintsho zophendlo lwezifo. Ngokukodwa zibonakala ngokucacileyo kufutshane amalungu ezinkulu amathambo elide. Bomzimba kungenzeka kamva ngenxa necrosis avascular kwamathambo.
• Igazi. Xa leukemia etsolo lymphoblastic, ibhiyozelwa tachycardia, pallor, ukopha of inwebu yangaphakathi emlonyeni, ukopha syndrome apha esikhumbeni. Ngenxa ukunxila kunye anaemia iphuhlisa buthathaka.

zokugula ukubonakaliswa

Ngowe-5-30% amatyala wegazi limfblastnogo amakhwenkwe eziziiprayimari etsolo waphawula ukukhula OKOKUQALA wamatyhalarha. baye lingene ezimbini okanye unilateral yalula ashinyeneyo apho. Ukuziqhelisa, ukwanda kakhulu lamatyala sezintso. Kulo mzekelo, isenokungabi kubonakaliswa lwezonyango zifumaneke ukoyiswa kwazo. ezinobuzaza Rare ziquka pericarditis exudative kunye nokungena engabalulekanga of nkovu isithintelo kwisiqalo phakathi epicardium kunye endocardium. Ngenxa yokwanda kwindawo nkovu kwi mediastinum zingenzeka yokuphazamiseka umsebenzi isixokelelwano sokuphefumla. Ezi mpawu of T-cell leukemia uhlobo kukhokelela syndrome awongamileyo Vena cava. Kwakhona kwenzeka kunye nokungaphumeleli zokuphefumla. Kwezinye iimeko nokungena apho leukaemic ye inwebu kwemiphunga okanye legazi kubo. Ngamanye amaxesha iingcali kunzima umahluko data evela iingxaki ezimbi esosulelayo. Ezinye iimpawu eziqhelekileyo umonakalo ne leukemia etsolo lymphoblastic iliso kufuneka kuqatshelwe ukopha kwiretina, nokudumba ingono kwi luvo optic, nokungena nemithambo. Xa ophthalmoscopy kungabonwa yi-uqweqwe ezantsi lweliso lakho. Mhlawumbi ke ubukho izinto ashinyeneyo bamantshontsho infiltrative yalula apha esikhumbeni. Usulelo kunokwenzeka nakweliphi na ikhava umonakalo. Kule nkalo, le nkqubo diagnostic ingachongwa panaritiums, paronychia, isinambuzane egulayo uluma ezahlukeneyo, ukuhlatywa kunye m. P.

uphando Paraclinical: oki

Athwala unokuncitshiswa okanye oqhelekileyo. Hematocrit, nobomvu cell wegazi kukholisa lifinyele. ukuncipha kwenani reticulocytes. White igazi cell count usenokuba zithotywe, inyusiwe okanye eqhelekileyo. Noko ke, loo nto iiseli amandla kungabonwa kuzo zonke iimeko. Kuba zoxilongo uphawu "ukusilela leukemic." Sithetha ubukho iiseli kuqhuma imvelaphi yokungabikho iifom eziphakathi - metamyelocytes kunye myelocytes - kwifomula egazini. Ngokuqhelekileyo wabona thrombocytopenia.

myelogram

Le nkqubo ithatha indawo okuhlatywa umongo wethambo. izinto Kucingo olubiyele kufuneka yenziwe amanqaku ubuncinane ezimbini. Inkqubo wenziwa kukhethwa phantsi Ukubulawa jikelele. Xa abantwana abadala ngaphezu kweminyaka emibini, omnye okuhlatywa lithatyathwe ngaphambili emikhulu angasemva ilium ukuya kwiminyaka-2 - ukusuka calcaneal tuberosity ziqwempu tibial okanye iziqalelo. iimviwo Cytological morphological ngokuqhelekileyo ibonisa izinto umongo hypercellular kunye ehluma iingqameko inkqubo hematopoietic yesiqhelo kunye nokungena lalahlekelwa izakhiwo cellular.

uphando cytochemical

Le yenye izigaba ezibalulekileyo loxilongo. Ukusebenzisa bala cytochemical luveze ukuba lilungu umgca iseli. Qiniseka ukuba isicelo uphando myeloperoxidase.

tap spinal

Kwakhona inkqubo mandatory ngokuxilonga kuyakrokreleka leukemia etsolo lymphoblastic, ezi zizathu zichazwe ngasentla. zokuhlatywa fluid kufuneka kwenziwe phantsi umthomalalisi kwaye kwimeko phambi egazini peripheral ubuncinane ayi-30 amawaka / ziiplatelets ml. Ukuba kuyimfuneko, phambi kokuba lenquba yenzelwa igazi trombovzvesi. Ukulungiselela tsitopreparat imfuneko ngeemililitha ubuncinane ezimbini zotywala. Xa efumanise izinto ngamandla imiqondiso umonakalo luvo cranial kwafunyaniswa neuroleukemia - leukemic CNS. Kufuneka kuqatshelwe ke, ukuba ukungena kwi-luvo lwe lymphoblasts kunye nathi afunxwe meninges kwenzeka zonke iimeko, nkqu kungekho ukwenza izinto zonyango kunye elabhoratri. Kwakhona kuyanqweneleka, kwaye ekufumanekeni kweempawu luvo, qiniseka ukuba ubambe ikhompyutha okanye Itomography yenyukliya kazibuthe umnqonqo okanye ingqondo.

Okunye uphando

Ngoncedo isithuba ultrasound retroperitoneal kunye kwisisu peritoneal ungakhankanya ubungakanani amalungu parenchymal ngokunyebelezela, iindawo nkovu eyandisiweyo kwimimandla esiswini kunye namanye, ngokwamalungu isinqe, amasende. Njengenxalenye uphethwe isifuba ray e uqikelelo ezimbini. Oku kuyimfuneko ukuze ukubona nokwanda mediastinum. Apho kuboniswe, ubekwa X-reyi amalungu kunye namathambo. Kuhlalutyo zengqo- yenziwa ngaphakathi izifundo elebhu. Oku jikelele ibonisa ukwanda ngaphezu 500 LDH IU, ezazinokuphazamisa kumsebenzi kwesibindi kunye nezintso. Phambi kokuqala chemotherapy ekucetyiswa yi echocardiography kunye ECG. Namhlanje, phakathi kwezinye iindlela, kuphononongo iseli eyosulelwe wasebenzisa iindlela emolekyuli yemfuza cytogenetic yokuqingqa inani zofuzo, kwakunye utshintsho lobume.

Treatment: Ubume

Umgaqo ezingundoqo apho oncohematology ezingabantwana mihla iya kwahlulwa ngokwamaqela lezigulana unyango ngamandla. Kubhaliwe ezikhethiweyo ngokuxhomekeke ubukhali kunye ophuma ileukemia oyingozi lymphoblastic. Ipredikshini karhulumente nayo ibalulekile. Ngoko ke, umzekelo, abo ukuphelisa ngezifo kakhulu ichemotherapy eliphakathi Kucetyiswa ngamandla ukuba ukufumana izixhobo ezinetyhefu ngakumbi enzima. Ukongeza, kuba abo izinga osandla singabanga phezulu ukusinda obuqikelelwayo, kukuqinisa imisebenzi iya kuba ithuba achache. Xa ukwahlulwa ibe iqela lezigulane kwafunyaniswa leukemia, ukuqikelela senziwa ngenxa yamava kwangaphambili, kunye nemigaqo ngamnye zibandakanywe kwinkqubo zokuphawula. Ngamnye kubo ukhandwa elinye okanye elinye icebo izenzo zonyango lwe kwelona ethile. Phantsi ezikulo ngoku yamkela isivumelwano jikelele kwi nolwabiwo iqela umngcipheko izigulane zaye kwafunyaniswa ukuba "leukemia lymphoblastic etsolo". Unyango abantwana yaphuhliswa gqira ngasekupheleni kwenkulungwane yokugqibela eMelika. Ubukhulu becala, imigaqo apho maqhinga zonyango, ukuba ayikhange utshintsho ezizodwa. Xa izigulane abangaphantsi kweminyaka leengxaki esizibonakalisa ngendlela ubundlongondlongo uphawu neuroleukemia, leukocytosis.

iindlela Main therapeutic

Unyango usebenzisa iziyobisi ezifana 'Prednisolone "yaye" Vincristine ". Iyasetyenziswa endolyumbalnoe intshayelelo lithetha "Methotrexate" kunye zokukhanya cranial yokuthintela neuroleukemia. Kwakhona, iye yaphuhliswa ucwangciso olukhethekileyo unyango. Oku kuthetha ukuba zonke izigulane zifumana ezinye idosi ecacileyo izayamaniso arhente chemotherapeutic ikwisakhelo ngexesha elithile, ngokulandelelana protocol. Ekupheleni yoma kwacaca ukuba emva enkalweni inkqubo enjalo wachacha malunga ne-50% abantwana abaye kokufunyaniswa wegazi. Izizathu zenkqubela phambili ukunyanga izifo ezibangelwa kwenkcazo ukungafani ka BONKE waza kamva ukungenisa indlela cytological ngamazwe zokuhlela kunye izinto prognostic, ukusasazwa izigulane bengamaqela, ukuphuhliswa kweenkqubo ngokwahlukeneyo. Ezibaluleke kakhulu yaba ngumbutho uphando multicenter le pharmacokinetics of cytostatics ezahlukeneyo ukuba iirejimeni olusebenzayo chemotherapeutic, kwakunye nophuhliso olukhawulezayo iziganeko ezihamba.

imigaqo esisiseko unyango

Ngenxa imisebenzi echazwe ngasentla, iinkqubo badalwa isizukulwana himiolecheniya esitsha. Izivumelwano ezininzi ezisetyenziswayo namhlanje zisekelwe kwimigaqo kokuqala unyango olunzulu, injongo yazo kukuba bawandise ukutshatyalaliswa ibe lidike iiseli ezingaqhelekanga. Xa intliziyo nokudalwa isinxulumanisi senkqubo unyango yimilo edibeneyo cytostatics, zilandelelana (ukujikeleza), idosi iphezulu-iirejimeni chemotherapeutic, kwakunye nokuthintela izifo olunzulu.

Iimpawu kwesicelo ngamalungiselelo

Amanyathelo aphambili ephilisayo othintelo kunye neuroleukemia intrathecal ziyintoni kuthetha "Prednisolone" "Cytosar", "Methotrexate" (in iidosi ngokobudala) kunye zokukhanya cranial. Le yokugqibela oluqhutywa nisaqala unyango. Xa endolyumbalno iziyobisi elawulwa "MTX" kunomphumela systemic. Kulo mba, ukwenzela ukunciphisa ubunzima ithumba kufuneka ube imiwe yokuqala yokuqala empontshwa therapeutic. zokukhanya Cranial lineziphumo ebezingalindelekanga ngqo ekude. Kulo mba, izifundo oluvela ZONKE unyango, olwenziwe namhlanje, ejolise ekunciphiseni idosi kunye ulwabiwo ecacileyo izigulane kwiqela ingozi ephantsi, apho unyango radiation ingafuneki. Zonke ezi mpumelelo iye yabangela kwi yee- ngasekupheleni kwenkulungwane lokugqibela ulungele ukoyisa i-70 ekhulwini umqobo seminyaka emihlanu-elingama ngenxa ileukemia abantwana ngaphandle likakade.

Iinkqubo Modern zonyango

A unyango esetyenziswa namhlanje, kubandakanya amanqanaba eziliqela. I lokuqala uqhelwaniso kuxolelwe kunye arhente ezintathu okanye ngaphezulu zilawulwa iiveki 4-6. Okwesibini - nokudityaniswa multi-arhente kwesigaba odlulileyo. therapy yesondlo - Wesithathu. Njengoko umthetho, isigaba sokugqibela malunga nesicwangciso-metabolites kusetyenziswa iminyaka 2-3. Ukuxhasa unyango inceda ukuthintela ukuphelisa ezininzi iingxaki ezinxulumene cytopenia kubantu omzimba. Imfuneko yokusebenzisa kwanonyango yesondlo iminyaka 2-3 singqiniwe nkqu phakathi kwinkulungwane yokugqibela. Ngokwesiqhelo ngeli xesha yonke imihla isigulane ithatha ichiza "6-Markaptopuriin". ngeveki Rhoqo wavelisa indlela "Methotrexate". Kuxhomekeka inani leeseli na idosi elimisiweyo. Practice ibonisa ukuba izigulane tolerability eyanelisayo kule rejimeni zonyango. Ukwenziwa imisebenzi kuxhaswa yi-80% ye umthamo efunekayo uthathwa ukuba ibe ngumba ihlwempu prognostic ukuba ukuphinda.

Ukuze ufumane inkcazelo engakumbi,

Kukho kukho iqela leengxaki unyango BONKE. Ngokukodwa, inani lezigulane kunye ithumba mutated iseli clones ukumelana ichemotherapy, ixabiso eliphezulu nasisihlo yeziphumo ebezingalindelekanga emva kwexesha (ukuphazamiseka neuroendocrine, ukukhula yamathumba eziziisekondari). Ukungaphumeleli yonyango yoqheliso izigulane ngenxa ekufeni ngenxa kubunzima eziyityhefu okanye ithumba enganyangekiyo. Kwakhona kulinganiselwe kakhulu Indlela nothomalaliso izigulane abantu bengasamkeli kunyango eqhelekileyo. Njengoko enye indlela isetyenziswa ezifana "Teniposide" "Vepeside" "Cytosar". Namhlanje malunga 70% abantwana babe uyekelelo kunyaka-5. leukemia lymphoblastic Acute abadala Sibuye onke amatyala wesixhenxe. Oku kubonisa ukuba ixesha ithuba kuxolelwe kunokwenzeka ukuphumeza-20% kuphela yamatyala. Njengoko umthetho, ukubuya leengxaki waphawula kwiminyaka yokuqala-2 emva kokuxilongwa. imeko uxolelo kwachazwa ngokuthi nokungabi namandla iiseli egazini, ubukho ngaphantsi kwe-5% esenzeke kwi lomongo wethambo izihlunu, ukuvela iimpawu kwakhona hematopoiesis eqhelekileyo. Ekuhlalutyeni ulwelo fluid kutyhilwa ngaphantsi kwe-5 iiseli mononuclear / microliter. Kuba impumelelo unyango ukuya ekuphumeleleni ngokukhawuleza status kuxolelwa. Ngowe-90% yezigulane basoloko iiseli ezingaqhelekanga avelana ichemotherapy.

Ekuphetheni

Eyona njongo iphambili unyango kukuba lokuyigxotha le ngezifo nomonde ukubuyela ubomi obuqhelekileyo ekuhlaleni impilo entle kunye ubuncinane iingxaki ezibangelwa unyango. Ukufezekisa oku kakhulu kunzima namhlanje. Oku kungenxa yokuba uninzi amayeza antileukemic bengengabo uyamkhetha kunye ityhefu. Noko ke, inkqubela ukuqondwa ukungafani lwezonyango kunye ngokobugcisa esi sifo, baqhubeka liqhuba izifundo ezahlukeneyo ukunika ithemba lokuba Pathogenesis beemolekyuli esi sifo kodwa ziya ziqala kude kube sekupheleni. Kulo mzekelo, ulwazi indlela yeenguqu elinobuhlungu kuya kunceda ukuphuhlisa amayeza kakuhle kwaye ayinabungozi.