Ganewar asali da kuma lura da m lymphoblastic cutar sankarar bargo. cututtuka

Lymphoblastic cutar sankarar bargo, jini yana dauke su ya fi na kowa oncological Pathology yara. Shi ne game da 25% na cutar kansa gano a cikin ilimin aikin likita na yara. Next, dauki mukalli cutar sankarar bargo jini - abin da shi ne, me ya sa shi ne, akwai kuma abin da warkewa shisshigi suna da za'ayi a cikin wannan Pathology.

Overview

Cutar sankarar bargo jini - abin da yake da shi? Haka kuma cutar tana nufin wani rukuni na ciwon daji. DUK (m lymphoblastic cutar sankarar bargo), da bayyanar cututtuka na wanda za a tattauna a kasa, shi ne iri-irin rukuni na neoplasms na sel da cewa suna da takamaiman kwayoyin da immunophenotypic halaye. Kara samar da jari na wadannan Kwayoyin a cikin bargo munanan taimaka sakandare yaduwa, bambantawa ko duka biyu. A sakamakon haka, infiltration na sel na parenchymatous gabobin da kuma Lymph nodes. Mafi (game da 80%) na dukkan sankarar bargo (sankarar bargo), gano a cikin yara, yana da lymphoid asalin.

Yaduwar

M jini cututtuka a duniya a yau ake samu a ko'ina. DUK ruwan dare a kasashen da suka ci gaba ne 3-4 lokuta da dubu ɗari yara a kowace shekara. A cikin kasashe na Asiya ta tsakiya da kuma Afirka Pathology ne ƙasa da na kowa. DUK sau da yawa a Amurka jihohin na Japan, China, Turai. A ganiya abin da ya faru a cikin yara yana dauke su da shekaru 2.5-5 shekaru. Mafi na kowa abnormality auku a boys.

predisposing dalilai

Da farko su ya kamata a dangana ga shekaru uwar prenatal kula, high haihuwa nauyi - fiye da 4000 grams, post- da prenatal daukan hotuna zuwa ionizing radiation. A wasu lokuta shi yiwuwa ko faruwa tare da m lymphoblastic cutar sankarar bargo? History of mahaifiyarsa ta rashin lafiya na iya hada bayani game da fetal mutuwa a baya ciki. Wannan zai iya faruwa saboda m muhalli yanayi, ko saboda wani kayyade predisposition. A cikin hali na ciwon daji marasa lafiya a cikin gida na zumunta, kara alama cewa nan gaba za su bayyana m lymphoblastic cutar sankarar bargo. A Sanadin cutar iya dangantawa da chromosomal rashin zaman lafiya syndromes abubuwa. Wadannan sun hada da, musamman, sun hada da Down ciwo, neurofibromatosis, m immunodeficiency, Fanconi anemia, X-nasaba agammaglobulinemia (nakasar) da sauransu. A sakamakon haka, da dama daga karatu, masana sun zo ga ƙarshe cewa a cikin m lymphoblastic cutar sankarar bargo, kazalika da ci gaban sauran Oncology, akwai wani yunkri maye gurbi na progenitor Kwayoyin. Ta baya ninkawa launchers muhalli abubuwan da kunna yaduwa. Wannan shi ne mafi kowa sabon abu da za su iya haifar da cutar sankarar bargo. Dalilan vozniknovneniya Pathology ne daya daga cikin muhimman hakkokin al'amurran lokacin da zabi hanyoyin kwantar da hankali.

bincikowa da

Lokacin da gano wani Pathology Cancer za a ɗauke shi zuwa lissafi na asibiti halaye da kuma siffofin da Kwayoyin cewa yin up da ƙari substrate. DUK ganewar asali dogara ne a kan zahirin jarrabawa da kuma paraclinical data tarihi da dakin gwaje-gwaje da karatu.

asibiti hoto

Ãyõyin cutar sankarar bargo ne mafi hadaddun syndromes:

• Buguwa. A m lymphoblastic cutar sankarar bargo ne alama malaise, nauyi asara, gajiya, da zazzaɓi. A karshen za a iya jawo by kwayar, kwayan, protozoal (m), ko fungal kamuwa da cuta, musamman a marasa lafiya tare da neutropenia.
• Hyperplastic. Lokacin da m lymphoblastic cutar sankarar bargo ne infiltration na baƙin ciki, kuma hanta. Wannan, bi da bi, take kaiwa zuwa hepatosplenomegaly, wanda za a iya bayyana tausayi a ciki. A farko alamun cutar sankarar bargo - pathological samu karaya na dogon ƙasũsuwa ko kashin baya. Soreness da kuma kumburi daga cikin gidajen abinci na iya zama da farko kuskure domin bayyanar cututtuka na rheumatoid amosanin gabbai ko wasu cuta. Kashi cuta - for osteomyelitis. Lokacin da leukemic infiltration na articular kwantena da periosteum, zuciya da kuma bargo da ƙari kara a juz'i na bayyana m zafi. A lokaci guda a kan X-ray gano halayyar pathological canje-canje. Musamman su ne a fili a bayyane a kusa da manyan gidajen abinci da kuma dogon ƙasũsuwa. Soreness iya faruwa daga baya saboda avascular necrosis da kuma osteoporosis.
• M. A m lymphoblastic cutar sankarar bargo, bikin tachycardia, pallor, zub da jini na mucous membranes a cikin bakinsa, hemorrhagic ciwo a kan fata. Saboda abin mãye da anemia tasowa rauni.

Pathology bayyanuwar

A 5-30% na lokuta na farko m da cutar sankarar bargo limfblastnogo boys lura farko testicular kara girma. Akwai sun kasance biyu ko unilateral m m infiltrates. A yi, da wani gwaji karuwa a lokuta ya ruwaito daga koda. A wannan yanayin, ba a samuwa asibiti manifestations na shan kashi. Rare rikitarwa hada da exudative pericarditis da kuma tsokar infiltration na Lymph toshewa a kan bango tsakanin epicardium da endocardium. Tare da karuwa a cikin Lymph nodes a cikin mediastinum iya faruwa disturbances a cikin ayyuka na numfashi tsarin. Wadannan halaye na T-cell cutar sankarar bargo irin gubar da ciwo m vena cava. Yana kuma iya faruwa da kuma na numfashi gazawar. A wasu lokuta can leukaemic infiltration na huhu nama ko hemorrhage a gare su. Wani lokacin masana suna da wuya a bambance data daga rikitarwa na dauke da kwayar cutar pathologies. Wasu kowa bayyanar cututtuka na ido lalacewa da m lymphoblastic cutar sankarar bargo kamata a lura da zub da jini a cikin akan tantanin ido, edema nono a cikin na gani jijiya, jijiyoyin bugun gini infiltration. Lokacin da ophthalmoscopy za a iya gano ta plaque a kasa na da eyeball. Kila ma gaban m bluish infiltrative m abubuwa a kan fata. Kamuwa iya faruwa a kowane lalacewar murfin. A wannan batun, da bincike aiwatar iya gano panaritiums, paronychia, kamu cizon kwari daban-daban, punctures da kuma m. P.

Paraclinical bincike: itacen oak

Haemoglobin za a iya rage ko al'ada. Hematocrit, da kuma ja jini cell count ne yawanci rage. Alama karu a yawan reticulocytes. Farin jini cell count iya rage, ta ƙara ko al'ada. Duk da haka, an ba da ikon Kwayoyin za a iya gano a duk lokuta. Domin Pathology halayyar "leukemic gazawar." Muna magana ne game da kasancewar fashewar Kwayoyin a bango na rashin tsaka-tsakin - metamyelocytes da myelocytes - a cikin jini dabara. Yawancin lokaci lura thrombocytopenia.

myelogram

Wannan hanya da aka dauka bargo huda. Shinge abu da ya kamata a sanya daga akalla biyu da maki. Hanya ne da za'ayi zai fi dacewa a karkashin janar maganin sa barci. A yara girmi shekaru biyu, huda da aka dauka daga gaba da raya ridges ilium har zuwa 2 shekaru - daga calcaneal tuberosity tibial segments ko abubuwa. Cytological morphological jarrabawa yawanci bayyana hypercellular bargo abu da quntata sprouts al'ada hematopoietic tsarin da infiltration na Harin salula Tsarin.

cytochemical bincike

Wannan shi ne daya daga cikin muhimman matakai na ganewar asali. Amfani da cytochemical batawa wahayi zuwa ciki a cell line. Tabbata a yi amfani da bincike kan myeloperoxidase.

kashin baya famfo

Shi ne kuma m bincike hanya a da ake zargin m lymphoblastic cutar sankarar bargo, da dalilai da aka ambata a sama. Cerebrospinal huda ya kamata a yi a karkashin kwantad da hankali da kuma a yanayin saukan gaban a gefe jini na akalla dubu 30 / ml platelets. Idan dole, kafin hanya aka yi transfussion trombovzvesi. Don shirya tsitopreparat bukatar akalla biyu milliliters na sayar da giya. Bayan ganewa na da ikon abubuwa da ãyõyin lalacewar girman kwanyar jijiyoyi kamu neuroleukemia - leukemic CNS. Ya kamata a lura, duk da haka, da cewa shigar azzakari cikin farji cikin juyayi tsarin na lymphoblasts da infiltration na meninges faruwa a dukkan lokuta, ko da a cikin rashi na asibiti da kuma awon manifestations. Shi ne kuma kyawawa, da kuma ganewa na neurological bayyanar cututtuka, a tabbatar rike da kwamfuta ko nukiliya Magnetic tomography na laka ko kwakwalwa.

sauran bincike

Da taimakon duban dan tayi retroperitoneal sarari da kuma peritoneal rami iya saka da size da parenchymal gabobin infiltrated, kara girman Lymph nodes a ciki da kuma sauran yankunan, gabobin a ƙashin ƙugu, golaye. Kamar yadda wani ɓangare na da ganewar asali ne kirji radiography a biyu tsinkaya. Wannan wajibi ne don gane da karuwa a mediastinum. Inda ya nuna, an nada X-ray na gidajen abinci da kuma kasusuwa. A biochemical bincike ne da za'ayi a cikin dakin gwaje-gwaje da karatu. An kullum ya nuna wani karuwa fiye da 500 LDH Iu, zai yiwu disturbances a cikin ayyuka na hanta da kuma kodan. Kafin fara jiyyar cutar sankara shawarar da echocardiography da ECG. Yau, a tsakanin sauran hanyoyin, a cikin kamuwa da kwayar binciken amfani da kwayoyin halitta da cytogenetic hanyoyin domin kayyade yawan chromosomes, kazalika da tsarin canje-canje.

Jiyya: Overview

The asali manufa kan abin da na zamani na yara oncohematology aka raba cikin kungiyoyin na marasa lafiya a kan far tsanani. An zabi dangane da tsananin tare da fitowa da m lymphoblastic cutar sankarar bargo. Hasashen jihar ne kuma muhimmanci. Saboda haka, misali, waɗanda suke kawar da Pathology quite matsakaici jiyyar cutar sankara bada shawara mai karfi ba a yi maka fiye da guba da kuma nauyi kayan aikin. Bugu da kari, ga wadanda kimani rayuwa kudi ne ba don haka high, da intensification na ayyuka zai iya zama wata dama na dawo. Lokacin da rabo a cikin wani rukuni na marasa lafiya bincikar lafiya tare da cutar sankarar bargo, Hasashen aka sanya a kan tushen kafin kwarewa, da kuma mutum ladabi kunshe a cikin lakabtawa tsarin. Kowace daga cikinsu ne kafa ta daya ko wasu dabarun da warkewa ayyuka na wani tsanani. A karkashin halin yanzu rarrabuwa soma wani janar yarjejeniya a kan rarraba da hadarin rukuni na marasa lafiya da suke aka kamu "m lymphoblastic cutar sankarar bargo". Jiyya na yara da aka ci gaba a cikin marigayi sittin karni na karshe a Amurka. By kuma manyan, da akida, da a kan abin da warkewa dabara, ba halartar musamman canje-canje. A marasa lafiya a ƙarƙashin da shekaru Pathology bayyana kanta a wani sosai m hanya da kuma halin neuroleukemia, leukocytosis.

Main warkewa hanyoyin

A lura yana amfani da kwayoyi kamar "Prednisolone" da "Vincristine". Haka kuma an yi amfani da endolyumbalnoe gabatarwar nufin "Methotrexate" da kuma girman kwanyar sakawa a iska mai guba domin hana neuroleukemia. Har ila yau, musamman magani shirin da aka ɓullo. Wannan yana nufin cewa dukan marasa lafiya sama mabayyani sashi da kuma haduwa da tsoka jamiái a wani takamaiman lokaci, bi da bi yarjejeniya. By ƙarshen seventies ya bayyana cewa, bayan da nassi na da irin wannan shirin gano game da 50% na yara wanda aka kamu da cutar sankarar bargo. A dalilai domin kara ci gaba da magani daga cututtuka sa da definition na heterogeneity na DUK kuma baya gabatar da tsarin kasa da kasa da cytological rarrabuwa da kuma prognostic dalilai, da rarraba marasa lafiya a kungiyoyin, ci gaban bambancin shirye-shirye. Na bayar da muhimmanci shi ne shiri na multicenter bincike binciken na pharmacokinetics na daban-daban cytostatics ga mafi tasiri da tsoka regimens, kazalika da m ci gaba da rakiyar events.

Ka'idodi na far

Godiya ga ayyuka aka bayyana a sama, da shirye-shirye da aka halitta himiolecheniya sãbuwa. Mai ladabi amfani a yau su ne bisa ka'idojin farko m far, da manufar wanda shi ne ya kara da halakar a tafkin mahaukaci Kwayoyin. A zuciya na halittar warkewa aikace-aikace shirin ne a cikin nau'i na haduwa da cytostatics, m (juyawa), high-kashi da tsoka regimens, kazalika da wani m cutar rigakafin.

Features na aikace-aikace na shirye-shirye

Babban curative da kuma m matakan da intrathecal neuroleukemia ne nufin "Prednisolone" "Cytosar", "Methotrexate" (a cikin dosages bisa ga shekaru) kuma girman kwanyar sakawa a iska mai guba. A karshen ne da za'ayi a farkon matakai na far. Lokacin da endolyumbalno gudanar miyagun ƙwayoyi "MTX" yana da wani tsari sakamako. A wannan batun, domin ya rage da ƙari taro dole ne ya zama farkon gudanar da farko warkewa lumbar huda. Girman kwanyar sakawa a iska mai guba yana m da kuma kai tsaye illa. A wannan batun, karatu a ingantawa DUK far, gudanar a yau, da nufin rage da kashi da kuma a sarari kasafi na marasa lafiya a cikin low hadarin kungiyar, ga abin da radiation far ba bukata. Duk wadannan nasarori sun sanya shi yiwuwa a cikin marigayi eighties na karshe karni shirya don shawo kan kashi 70 cikin dari shãmaki biyar-shekara rayuwa kudi domin cutar sankarar bargo a cikin yara ba tare da komowan cutar.

Modern warkewa shirye-shirye

A lura da cewa an yi amfani da yau, ya shafi dama bulan. Na farko shi ne shigar da na gafarta musu tare da uku ko fiye da jamiái suna gudanar for 4-6 makonni. Na biyu - da bunqasar na Multi-wakili na baya zamani. Na uku - tabbatarwa far. Matsayin mai mulkin, da karshe lokaci na anti-metabolites amfani ga 2-3 shekaru. Tallafa far taimaka wajen hana da kuma kawar da yawa daga cikin matsalolin da dangantaka da jawo cytopenia da immunosuppression. Da bukatar amfani da wani goyon baya far ga 2-3 shekaru da aka tabbatar har ma a tsakiyar karni na karshe. Kullum a wannan lokaci kowace rana da haƙuri daukan da miyagun ƙwayoyi "6-Markaptopuriin". Kowane mako ya gabatar a wajen "Methotrexate". Dangane da yawan Kwayoyin ne sa sashi. Yi ya nuna cewa a cikin marasa lafiya m tolerability wannan warkewa regimen. Kisa da ayyuka da goyan bayan 80% na bukata girma da aka dauke su a matalauta prognostic factor for komawa.

Don ƙarin bayani,

Akwai matsaloli a cikin magungunan DU. Musamman, adadin marasa lafiya da ƙwayoyin ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar ƙwayar cuta waɗanda suke da tsayayya ga chemotherapy, farashin kima da bayyanar cututtuka na ƙarshen (cututtukan neuroendocrin, ciwon haɗari, ƙwayoyin tumuttuka) suna karuwa. Rashin rashin karɓar magani shine saboda mutuwar marasa lafiya da farko saboda matsalolin haɗari ko tsari mai tsari. Har ila yau, hanyoyin zuwa ga likitocin marasa lafiya da basu amsa ga tsarin ka'ida ba suna iyakancewa. A madadin wannan, ana amfani da irin wadannan jami'in "Teniposide", "Vepesid", "Cytosar". A yau, kimanin kashi 70 cikin 100 na yara suna da gyaran shekaru biyar. Magungunan cutar sankarar lymphoblastic mai tsanani a cikin tsofaffi sukan dawo a kowane bakwai. Wannan yana nuna cewa tsawon lokaci na tsagaitawa zai iya samuwa ne kawai a cikin kashi 20 cikin dari. A matsayinka na mai mulki, ana iya dawo da ilmin lissafi a farkon shekaru 2 bayan ganewar asali. Jihar remission yana nuna rashin ragowar wutar lantarki a cikin jini, kasancewarsa da kasa da kashi 5 cikin hauka a cikin kasusuwan kasusuwa, bayyanar alamun sabuntawa na ka'idar hematopoiesis. A cikin bincike na cerebrospinal ruwa saukar kasa da 5 mononuclear Kwayoyin / microliter. Don nasarar nasarar jiyya, yana da mahimmanci don cimma nasarar gafara. A cikin kashi 90 cikin 100 na marasa lafiya, a matsayin mai mulkin, malaman pathological suna nuna lafiyar su zuwa chemotherapy.

A ƙarshe

Babban manufar farfadowa shi ne ya ceci marasa lafiya daga ilimin lissafi kuma ya mayar da shi zuwa rayuwa ta zamantakewar al'ada da kuma kyakkyawar yanayin lafiya tare da mafi yawan rikitarwa da cutar ta haifar. Don cimma wannan a yau yana da wuyar gaske. Wannan shi ne saboda gaskiyar cewa mafi yawan maganin antileukemic ba su da zabi kuma mai guba. Duk da haka, nasarorin da ke tattare da fahimtar ilimin lissafi da nazarin kwayoyin halitta, da ci gaba da nazarin binciken daban-daban sunyi fatan cewa cutar kwayar cutar za ta kasance ta ƙarshe har zuwa karshen. A wannan yanayin, ilmantarwa game da hanyoyin da zazzafar ƙwayar tumatir zai ba da izinin inganta tasirin magungunan magunguna.