Ukuxilongwa kanye nokwelashwa of acute wegazi lymphoblastic. izimpawu

wegazi lymphoblastic, igazi ubhekwa ezivamile oncological zokugula izingane. Kumayelana 25% zomdlavuza ezitholakele Pediatrics. Okulandelayo, thatha eduze ukubukeka wegazi igazi - kuyini, kungani kuba khona futhi yini ezelaphayo abazenzi kule egunjini lokuhlolwa kwezifo.

Uhlolojikelele

Umdlavuza wegazi igazi - kuyini? Lesi sifo libhekisela eqenjini umdlavuza. KONKE (acute lymphoblastic wegazi), izimpawu zazo kuzoxoxwa ngezansi, iqembu heterogeneous ka neoplasms lamaseli anezici ezithile zofuzo immunophenotypic. Khulisa ukukhiqiza futhi ukuqongelela lezi lamaseli kwezinso umnkantsha neqhaza ekwandeni yesibili, kwamangqamuzana noma kokubili. Ngenxa yalokho, kokuziphatha amaseli kwezitho parenchymatous futhi zamanzi. Iningi (80%) wabo bonke wegazi (wegazi), kubonakele ku children, imvelaphi lymph.

nokudlanga

izifo ebulalayo igazi emhlabeni namuhla zitholakala yonke indawo. BONKE nokudlanga ababhekene nazo emazweni asethuthukile kuyinto 3-4 amakesi izingane ayizinkulungwane ezingamakhulu unyaka ngamunye. Emazweni Ase-Asia Ephakathi futhi Afrika zokugula lingaphansi ezivamile. BONKE ngokuvamile e-US uthi of Japan, China, I-Europe. Kuvamisile isiqongo izingane ubhekwa kweminyaka eyi 2.5-5. I angahlangani ezivamile kwenzeka kubafana.

ungalokothi uphuze izinto

bona kuqala kunazo zonke okufanele kwenziwe isibaluli engu-unina ukunakekelwa okufanele kwangaphambi kokubeletha, okusezingeni eliphezulu ezizalwa zinesisindo esiphansi - kuka 4000 amagremu, ukuchayeka kwe- senzakalo futhi kokubeletha ukuze ionizing emisebeni. Kwezinye izimo kungase kwenzeke ngisho ne-acute wegazi lymphoblastic? Umlando lokugula kamama ingase ihlanganise ulwazi olumayelana ukufa fetus ukukhulelwa odlule. Lokhu kungavela ngenxa kwezimo zendawo ezimbi, noma ngenxa yofuzo. Endabeni umdlavuza iziguli ekhaya izihlobo, kwande amathuba okuba kuyovela izizukulwane ezizayo acute lymphoblastic leukemia. Izimbangela zesifo lingaphathelaniswa ne chromosomal ukuntengantenga syndromes izakhi. Lezi zihlanganisa, ngokukhethekile, zihlanganisa i-Down Syndrome, neurofibromatosis, immunodeficiency variable, Fanconi kwegazi, X-exhumene agammaglobulinemia (azalwa naso) nabanye. Ngenxa yalokho, eziningi izifundo, ochwepheshe baye bafinyelela esiphethweni ukuthi sibi wegazi lymphoblastic, kanye nokuthuthukiswa yezinye Oncology, kukhona izinto ngokuguqula izakhi, okuzenzakalelayo amaseli sikakhokho. Yena kamuva wawuhlanganisa Iziqalisi yizici zemvelo ukuthi kusebenze ayanda. Lona Kuyaziwa kakhulu kungabangela nomdlavuza wegazi. Izizathu vozniknovneniya zokugula ingenye izici eziyisisekelo zokuthi uma kukhethwa zokwelapha.

ukuhlola

Lapho eveza iyiphi Cancer zokugula kufanele kucatshangelwe izici emitholampilo izici amangqamuzana akha substrate lwesimila. BONKE uphethwe kusekelwe behlolwa futhi paraclinical umlando idatha futhi laboratory izifundo.

emitholampilo isithombe

Izimpawu wegazi yilezi syndromes eziyinkimbinkimbi:

• Ukudakwa. Ngo-acute wegazi lymphoblastic kuyinto umkhuhlane ngokuphawulekayo, kwesisindo, ukukhathala, imfiva. Lesi sakamuva singabangela ukuba senziwa viral, igciwane, protozoal (ezingavamile), noma izifo fungal, ikakhulukazi in iziguli neutropenia.
• Hyperplastic. Lapho acute lymphoblastic leukemia kuyinto kokuziphatha ubende kanye esibindini. Lokhu, kuholela hepatosplenomegaly, okuyinto engaveza ngesisa esiswini. Izimpawu zokuqala wegazi - waphuka sokugembula lamathambo eside noma emgogodleni. Soreness kanye ukuvuvukala emalungeni kungaqalisa sizididanise izimpawu zesifo sokuqaqamba kwamalunga omzimba noma nezinye izifo. isifo Bone - I-osteomyelitis. Lapho kokuziphatha leukemic we capsule articular kanye periosteum, inhliziyo futhi umnkantsha isimila lwanda ivolumu avele ubuhlungu ebanzi. Ngesikhathi esifanayo ku-X-ray Kutholwe isici izinguquko sokugembula. Ikakhulukazi bona kuyabonakala eseduze namajoyinti ezinkulu namathambo eside. Soreness kungenzeka kamuva ngenxa necrosis avascular kwamathambo.
• Anemiya. Ngo-acute wegazi lymphoblastic, kugujwa tachycardia, pallor, ukopha ka nolwelwesi lwamafinyila emlonyeni, oluhambisana syndrome esikhumbeni. Ngenxa adakwe anaemia eba buthaka.

zokugula ukubonakaliswa

Ngo 5-30% we amacala eyinhloko acute wegazi limfblastnogo abafana waphawula ZOKUQALA ukukhulisa wamasende. Sekube kobuhlungu lingene ezimbili noma sohlangothi olulodwa obukhulu. Ekusebenteni, ukukhuphuka kwezinga lamacala abikiwe izinso. Kulokhu, kungase kungabi ukubonakaliswa etholakalayo basemtholampilo kunqotshwa kwazo. yezinkinga ezingavamile zihlanganisa pericarditis exudative futhi kokuziphatha esivimba amanzi ka-lymph isimiso ku ngemuva phakathi epicardium futhi endocardium. Nokwanda e-lymph ku mediastinum kungenzeka abahluphayo umsebenzi umgudu wokuphefumula. Lezi izici T-cell wegazi uhlobo ziholela sifo aphakeme vena cava. Iphinde singenzeka nokuvaleka kwemigudu yokuphefumula. Kwezinye izimo kokuziphatha khona leukaemic ijwabu wamaphaphu noma ukopha kuzo. Ngezinye izikhathi izazi kunzima ukuhlukanisa idatha kusuka yezinkinga pathologies ezithathelwanayo. Ezinye izimpawu ezivamile umonakalo iso nge acute wegazi lymphoblastic kufanele ukopha kwe-retina, edema ingono e emsipheni optic, kokuziphatha semithambo kuphawulwe. Lapho ophthalmoscopy kungatholakala yi-plaque ezansi yeso. Mhlawumbe futhi khona bluish izakhi obukhulu infiltrative kobuhlungu esikhumbeni. Ukutheleleka zingaphinde zivele noma ikhava umonakalo. Kule ndaba, inqubo zokuxilonga kungenzeka panaritiums ezikhonjwe, paronychia, isinambuzane itheleleke eluma ezahlukene, punctures kanye m. P.

nophenyo Paraclinical: oki

I-hemoglobin zingancishiswa noma evamile. Hematocrit, futhi abomvu egazi count ngokuvamile encishisiwe. ukwehla emakiwe kunombolo reticulocytes. igazi White cell count kungancipha, anda noma evamile. Nokho, it is hhayi amaseli amandla kungatholakala kuzo zonke izimo. Ukuze zokugula isici "ukuhluleka leukemic." Sikhuluma phambi kuqhuma amaseli ngemuva yokuntuleka amafomu Lesisemkhatsini - metamyelocytes futhi myelocytes - ku-formula igazi. Ngokuvamile waphawula thrombocytopenia.

myelogram

Le nqubo ithatha wokubhoboza umnkantsha. Fence impahla kufanele yenziwe amaphuzu okungenani ezimbili. Inqubo wenziwa mhlawumbe ngaphansi izinzwa jikelele. Ezinganeni abaneminyaka engama amabili, wokubhoboza ethathwa phambi emikhulu ezingemuva ilium kuze kube iminyaka engu-2 - kusukela calcaneal tuberosity tibial izingxenye noma izakhi. Cytological luhlolo morphological ngokuvamile kwembula hypercellular umnkantsha impahla kanye amahlumela ayisesemikhulu hematopoietic evamile uhlelo nokungena kokuziphatha ukuqhuma izakhiwo amaselula.

ucwaningo cytochemical

Lona omunye izigaba ezibalulekile ukuxilongwa. Ukusebenzisa staining cytochemical wembula kwazo ukuba nibe ngabakaJesu umugqa cell. Qiniseka ukuthi ukusebenzisa ucwaningo ku myeloperoxidase.

Thepha umgogodla

Libuye saziso nenqubo ethile yokuthola isifo e esolwa acute wegazi lymphoblastic, izizathu ezibalulwe ngenhla. wokubhoboza cerebrospinal kufanele kwenziwe ngaphansi ukupholisa futhi uma khona egazini neuropathy okungenani 30 ayizinkulungwane / platelet ml. Uma kudingekile, ngaphambi inqubo wenziwa ukumpontshelwa trombovzvesi. Ukuze silungiselele tsitopreparat isidingo okungenani ezimbili namamililitha yotshwala. Phezu ukuthola izakhi amandla izibonakaliso ukulimala nezinzwa cranial kutholakala ukuthi uphethwe yini neuroleukemia - leukemic CNS. Kufanele kuphawulwe nokho, ukuthi Ukungena ku isimiso sezinzwa lymphoblasts futhi kokuziphatha meninges zenzeka kuzo zonke izimo, ngisho nalapho engekho ukubonakaliswa emitholampilo laboratory. Libuye efiselekayo, futhi ukuthola izimpawu zokungasebenzi kahle kwemizwa, qiniseka ubambe kukhompyutha noma zenuzi tomography magnetic emgogodleni noma ubuchopho.

Okunye ucwaningo

Ngosizo ultrasound retroperitoneal Isikhala ingaphakathi peritoneal ungacacisa ubukhulu kwezitho parenchymal ngesinyenyela, lakhulisiwe izindlala zamanzi esiswini nakwezinye izindawo, izitho ukhalo, amasende. Njengengxenye uphethwe esifubeni esimweni ezenziwa ezimbili. Lokhu kuyadingeka ukuze ithole ukwanda mediastinum. Lapho kuboniswe, umiswa-X-ray amalunga namathambo. Ekuhlaziyeni kwamakhemikhali wenziwa ngaphakathi izifundo laboratory. Kuvamise ebonisa ukwenyuka okukhulu kuka-500 LDH IU, iziphazamiso ezazingase zibe khona ku umsebenzi kwesibindi nezinso. Ngaphambi kokuqala ngamakhemikhali okwamenza ezinconywe echocardiography futhi ECG. Namuhla, phakathi kwezinye izindlela, kuseli cwaningo elinegciwane wasebenzisa izindlela yamangqamuzana zofuzo cytogenetic sokunquma inani lezakhi zofuzo, kanye izinguquko ezihlelekile.

Ukwelashwa: Uhlolojikelele

Isimiso esiyisisekelo phezu kwayo oncohematology yesimanje ezingane is baba ngamaqembu iziguli yokwelapha umfutho. It is akhethiwe kuye ngobucayi ngawo, uqhubeka nenqubo acute wegazi lymphoblastic. isimo Ukubikezela nalo libalulekile. Ngakho, ngokwesibonelo, labo abangababiziweyo ukuqeda zokugula ngempela nokwelashwa ngamakhemikhali okusesilinganisweni sinconywa ukungatholi amathuluzi enobuthi kakhulu futhi ezisindayo. Ngaphezu kwalokho, labo sokusinda kabani ezilinganisiwe izinga akuyona okusezingeni eliphezulu kunjalo, ukwanda imisebenzi kungaba ithuba yokutakula. Lapho division zibe iqembu iziguli ukuthi unomdlavuza wegazi, ukuqagela senziwa ngesisekelo nesipiliyoni, futhi yezifiso ngabanye kufakwe ohlelweni kophawu. Ngamunye wabo Kumiswa elilodwa noma ezinye isu yezenzo oqabulayo a umfutho ezithile. Ngaphansi ngezigaba zamanje yokutholwa isivumelwano jikelele ukusatshalaliswa by ingozi iqembu ekubulaleni iziguli ezigulela saxilongwa kwatholakala "acute wegazi lymphoblastic". Ukwelashwa kwezingane yasungulwa sixties ngasekupheleni kwekhulu elidlule eMelika. Ngokwengxenye enkulu, izimiso eyayisekelwe kuzo leyo amacebo zokwelapha, baye akukho zinguquko ekhethekile. In iziguli ezingaphansi kweminyaka engu-pathology kuzibonakalisa ngendlela nolaka futhi lubonakala neuroleukemia, leukocytosis.

izindlela Main zokwelapha

Le nqubo yokwelapha isebenzisa izidakamizwa ezifana "Prednisolone" futhi "Vincristine". Iphinde isetshenziselwe endolyumbalnoe isingeniso kusho "Methotrexate" futhi ngemisebe cranial yokuvimbela neuroleukemia. Futhi, isimiso esikhethekile ukwelashwa yasungulwa. Lokhu kusho ukuthi zonke iziguli zithola ezinye umthamo ecacile futhi inhlanganisela ejenti chemotherapeutic ngesikhathi Uhlaka ethize, ngokulandelana olandelwayo. Lwathi luphela ngeminyaka yoma kwaba sobala ukuthi ngemva ukudlula isimiso esinjalo walulama mayelana 50% nabantwana abaye ukuthi unomdlavuza wegazi. Izizathu intuthuko eyengeziwe ekwelapheni izifo ezibangwa kwencazelo heterogeneity BONKE futhi kamuva Kwetfula luhlelo cytological ngamazwe ngezigaba kanye izici prognostic, ukusatshalaliswa iziguli amaqembu, ukuthuthukiswa kwezinhlelo umahluko. Lokubaluleke kakhulu kwaba inhlangano yocwaningo cwaningo multicenter we pharmacokinetics ka cytostatics ezahlukene ukuze zemithi ngempumelelo ngaphezulu chemotherapeutic, kanye ukuthuthukiswa ngokushesha izenzakalo eyayihambisana nalo.

izimiso eziyisisekelo yokwelapha

Ngenxa imisebenzi echazwe ngenhla, izinhlelo zadalwa isizukulwane himiolecheniya entsha. yezifiso Iningi esetshenziswa namuhla zisekelwe izimiso ka kokuqala ukwelashwa olunzulu, injongo okuyinto ukukhulisa ukubhujiswa libe yichibi amaseli angavamile. At inhliziyo ukudalwa zokwelapha hlelo hlelo lokusebenza ngesimo nenhlanganisela cytostatics, ngokulandelana (ukuzungeza), umthamo ephezulu zemithi chemotherapeutic, kanye isifo zokuvimbela olunzulu.

Izici kwesicelo amalungiselelo

The main izinyathelo ekwelapheni kanye zokuvimbela ne neuroleukemia intrathecal kuyizindlela "Prednisolone" "Cytosar", "Methotrexate" (e imithamo ngokobudala) kanye irradiation cranial. Lesi sakamuva Kwenziwa ku eqala ukwelashwa. Lapho endolyumbalno izidakamizwa iqondiswa "MTX" unethonya letindzala. Kule ndaba, ukuze kuncishiswe mass isimila kumelwe kube eliyifa yokuqala zokwelapha lumbar wokubhoboza. ngemisebe Cranial has akude futhi oqondile engemihle. Kule ndaba, izifundo ku nokwenza BONKE yokwelapha, iqhutshwe ngayo namuhla, okuhloswe ngazo ukunciphisa umthamo kanye nokwabiwa ecacile iziguli ongaphakeme iqembu ingozi, okuyiwona ukwelashwa ngemisebe ayidingekile. Zonke lezi impumelelo iye yasiza in the Eighties sekwephuzile kwekhulu elidlule ilungele unqobe amaphesenti 70 isithiyo oneminyaka emihlanu izinga ukusinda wegazi ezinganeni ngaphandle ukwehluleka kwesikhashana.

izinhlelo Modern zokwelapha

Indlela yokwelapha esebenza esetshenziswa namuhla, kuhilela izigaba eziningana. Esokuqala le lokungeniswa ka ukuthethelelwa nabenzeli ezintathu noma ngaphezulu iphathwa amasonto 4-6. Esesibili - ukuhlanganiswa multi-ejenti esigabeni odlule. ukwelashwa yesondlo - Okwesithathu. Njengomthetho, esigabeni lokugcina anti-metabolites wasebenzisa iminyaka 2-3. Ukusekela ukwelashwa kusiza ukuvimbela futhi uqede abaningi ngenxa yezinkinga ezihambisana ne cytopenia eyenga futhi kwamasosha omzimba. I nesidingo sokufaka isicelo yokwelapha yesondlo iminyaka 2-3 okuye kungase kutholakale ngisho phakathi nekhulu leminyaka eledlule. Ngokuvamile ngalesi sikhathi nsuku zonke isiguli kuthatha lesi sidakamizwa "6-Markaptopuriin". Njalo ngeviki wethula yokukhalaza "Methotrexate". Kuye inani lamaseli kuyinto Isikali isethi. Prakthiza ebonisa ukuthi iziguli tolerability egculisayo yaleli lwemithi zokwelapha. Ukubulawa imisebenzi kusekelwa 80% umthamo edingekayo ubhekwa ompofu isici prognostic ngoba Ukuphinda.

Ukuze uthole ukwaziswa okwengeziwe,

Kunezindlela eziningana izinkinga ekwelapheni BONKE. Ikakhulukazi, inani elandayo leziguli ne isimila soguquko lwegciwane singalawuleka cell ukufaniswa amelana nokwelashwa ngamakhemikhali, zibiza nesenzakalo zamuva side effects (neuroendocrine neziyaluyalu, ukukhula izimila zesibili). Ukuhluleka ukwelashwa lokungeniswa iziguli ngenxa ukufa ekuseni ngenxa yezinkinga enobuthi noma isimila ukumelana. Futhi kulinganiselwe kakhulu indlela yokwelapha iziguli ezigulela ungaphenduli yokwelapha esezingeni. Njengoba izindlela okunye isetshenziswa ezifana "Teniposide" "Vepeside" "Cytosar". Namuhla-70% izingane abe ukuthethelelwa engu-5. Acute wegazi lymphoblastic e abadala ivela kuzo zonke izimo lwesikhombisa. Lokhu kusikisela ukuthi isikhathi kwenkathi ukuthethelelwa kungenzeka ukufeza% 20 kuphela amacala. Njengomthetho, ukubuya zokugula ebonwe yokuqala engu 2 isifo satholakala. isimo ukuthethelelwa kwachazwa ngokuthi ukungabi kwamandla e amaseli weengazi, khona ngaphansi kwama-5% ukuqhuma ngo umnkantsha izicubu, ukubonakala izimpawu zokusimama ka hematopoiesis kuyinsakavukela. Esikhathini ukuhlaziya cerebrospinal fluid wembula esingaphansi 5 mononuclear amaseli / microliter. Ukuze impumelelo ukwelashwa impumelelo okusheshayo isimo ukuthethelelwa. Ngo-90% eziguli ngokuvamile amaseli angavamile nozwelo nokwelashwa ngamakhemikhali ukuphi.

Ekuphetheni

Inhloso ngqangi yalo ayikho indlela yokuyelapha ukuqeda lo zokugula nesineke futhi ukubuya layo ukuphila okuvamile kwezenhlalo kanye nempilo enhle nge okungenani yobunzima obabangelwa ekwelapheni. Ukuze sifinyelele kunzima ngempela namuhla. Lokhu kungenxa yokuthi iningi imithi antileukemic akuzona abakhethayo kakhulu futhi ezinobuthi. Nokho, ukuthuthuka ukuqonda heterogeneity emitholampilo begazi sifo, baqhubeka baqhuba izifundo ezihlukahlukene sikhanyisa amakhandlela ukuletha ithemba ukuthi pathogenesis yamangqamuzana lesi sifo isazoqhubeka ziqala kuze kube sekupheleni. Kulokhu, ulwazi etindlela ezinguquko abulalayo kuzosiza ukuthuthukisa ukwelashwa okuphumelelayo nokuncane anobuthi.