Chirwere uye vabatwe chakasimba lymphoblastic neleukemia. zviratidzo

Lymphoblastic neleukemia, ropa riri kuonekwa rakajairika oncological zvokurwara vana. Kunenge 25% kenza dzakaonekwa mu Pediatrics. Next, kunyatsotarisisa neleukemia ropa - chii, nei ikoko uye kupindira chii kuchirapa zviri kuitwa zvokurwara ichi.

tsananguro

Neleukemia ropa - chii? The chirwere anoreva boka kenza. ALL (chenjera lymphoblastic neleukemia), zviratidzo izvo dzichakurukurwa pazasi, ndizvo heterogeneous boka neoplasms masero kuti vane chaiyo kuvabereki uye immunophenotypic maitiro. Kuwedzera kugadzirwa uye kuunganidza izvi masero mumwongo abnormalities kubatsira yechipiri kuwanda Kusiyanisa kana vaviri. Somugumisiro, infiltration masero ose parenchymatous nadzo uye node nodes. Most (80%) kubva neleukemia zvose (neleukemia), dzakaonekwa kuvana, ane lymphoid mavambo.

Kupararira

Nyanyisa zvirwere munyika nhasi ropa zvinowanikwa kwose kwose. Kupararira ALL munyika dzakasimukira ndiyo 3-4 nyaya por zviuru zana vana pagore. Munyika Central Asia uye Africa zvokurwara iri ishoma. ALL kakawanda US inoti kweJapan, China, Europe. Yepamusoro kuitika mune vana kuonekwa makore 2.5-5. The inonyanya abnormality kunoitika muna vakomana.

predisposing zvinhu

Kutanga vanofanira anonzi makore amai havana hanya, mukuru kubereka uremu - anopfuura 4000 magiramu, mushure mokumuka uye pamuviri kuona ionizing nemwaranzi. Mune dzimwe nguva kunyange nokuitika chakasimba lymphoblastic neleukemia? History of raamai chirwere zvingasanganisira mashoko pamusoro fetus rufu mune yapfuura pamuviri. Izvi zvinogona kuitika nokuda ezvinhu yaivhuvhuta zvakatipoteredza, kana kuti nokuda majini kubatwa. Kana kenza varwere mumba nehama, kuwedzera rinofanira zvizvarwa ramangwana pachamuka chakasimba lymphoblastic neleukemia. Zvinokonzera chirwere vangava chokuita chromosomal kusagadzikana syndromes zvinhu. Izvi zvinosanganisira, zvikurukuru, zvinosanganisira chirwere cheDown syndrome, neurofibromatosis, shanduka immunodeficiency, Fanconi kupererwa neropa, X-chokuita agammaglobulinemia (vachiberekwa) uye vamwe. Somugumisiro, nhamba yezvidzidzo, nyanzvi vakasvika pakugumisa kuti rikwanise lymphoblastic neleukemia, uyewo kugadzirwa kwepurogiramu mamwe oncology, pane kwatangwa mutation pamusoro muvambi masero. Iye dzakwidzwa chakawedzerwa launchers zvezvakatipoteredza zvinhu Activate kuwanda. Ichi ndicho chakajairika anogona kukonzera neleukemia. Zvikonzero vozniknovneniya zvokurwara mumwe chinokosha zvinhu pakusarudza kurapa.

Diagnostics

Kana kuziva chero zvokurwara Cancer anofanira kufungwa nhoroondo zvekuchipatara unhu uye zvaidiwa masero anoumba bundu substrate. chirwere ALL hunobva mumuviri kuongororwa uye paraclinical date nhoroondo uye mumarabhoritari zvidzidzo.

zvekuchipatara mufananidzo

Zviratidzo neleukemia ndivo syndromes zvikuru kunzwisisa:

• Kudhaka. In chenjera lymphoblastic neleukemia segwapa zvakawanda zvive, uremu, kurukutika, fivhiri. Ekupedzisira inogona kukonzerwa akabatwa, kwemabhakitiriya, protozoal (zvisingawanzoitiki), kana fungal noutachiona, kunyanya varwere neutropenia.
• Hyperplastic. Kana chenjera lymphoblastic neleukemia ndiyo infiltration pamusoro chirwatata uye chiropa. Izviwo, kunotungamirira hepatosplenomegaly, zvinogona kuratidza unyoro mudumbu. Zviratidzo zvokutanga neleukemia - pathological mitswe mapfupa refu kana nomuzongoza. Soreness uye mumatenhere mapfundo anogona Pakutanga kururama kuti zviratidzo dzinonzi rheumatoid arthritis kana zvimwe zvirwere. Bone chirwere - nokuti osteomyelitis. Kana leukemic infiltration pamusoro articular Kamudzira uye periosteum, mwoyo uye mwongo bundu achiwedzera vhoriyamu kuonekwa zvakawanda marwadzo. Panguva iyoyo pasi X-ray padzakacherechedza hunhu Kumwe kuchinja kunotanga. Kunyanya vari pachena zvinoonekwa pedyo huru mapfundo mapfupa refu. Soreness zvingaitika pashure nokuda avascular necrosis uye osteoporosis.
• Neta neta. In chenjera lymphoblastic neleukemia, vakapemberera tachycardia, pallor, ropa pamusoro mucous membranes mumuromo, Hemorrhagic chirwere paganda. Nokuda kudhaka uye kupererwa neropa hwapedyo neutera.

zvokurwara kuratidzwa

Muna 5-30% nyaya chinonyanya rikwanise neleukemia limfblastnogo vakomana vakachengeta OKUTANGA testicular nedumbu. Pane vaviri kana yaitsigira divi nyarara batana infiltrates. In tsika, wandei kuwedzera yakashumwa vanenge itsvo. Muchiitiko chino, kungasava rinowanikwa zvekuchipatara kuratidzirwa kukundwa kwavo. Rare yematambudziko zvinosanganisira exudative pericarditis uye myocardial infiltration kuti node mhingaidzo pamusoro pakanyorwa pakati epicardium uye endocardium. With nokuwedzera node nodes vari mediastinum zvingaitika kuvhiringidzika basa kufema hurongwa. maitiro aya T-sero neleukemia mhando achitungamirira chirwere mukuru vena cava. Rinogonawo kuitika uye kufema kukundikana. Mune zvimwe zviitiko ikoko leukaemic infiltration pamusoro zvemapapu rinenge kana ropa mavari. Dzimwe nguva nyanzvi zvakaoma kusiyanisa mashoko kubva yematambudziko nezvirwere pathologies. Zvimwe zviratidzo zvinowanzovapo kukuvara ziso chete chenjera lymphoblastic neleukemia zvinofanira kucherechedzwa ropa iri the retina, edema munyatso ari optic etsinga, vascular infiltration. Kana ophthalmoscopy anogona kuonekwa ne bheji racho pazasi yeziso. Zvichida kuvapo gobvu rinokwanisa kucheneruka infiltrative nyarara zvinhu pamusoro ganda. Chirwere chinogona kuitika chero kukuvara viomak. Panyaya iyi, chirwere muitiro anogona ringazivikanwa panaritiums, paronychia, utachiona Tupukanana inoruma zvakasiyana, punctures uye m. P.

Paraclinical kuferefeta: OAK

Chizenga zvingaderedzwa kana chakaipa. Hematocrit, uye matsvuku eropa count inowanzopiwa ipfupi. Nemuumbi kuderera kwenhamba reticulocytes. White eropa count anogona kuderedzwa, hwakawedzera kana zvakajairika. Zvisinei, hazvina simba masero dzinogona kuonekwa nguva dzose. Nokuti zvokurwara mamirire "leukemic kukundikana." Tiri kutaura pamberi Nokufema masero pakanyorwa kushaya zvenguva siyana - metamyelocytes uye myelocytes - muropa fomura. Zvinowanzochengetwa thrombocytopenia.

myelogram

nzira iri kutora mwongo ponji. Fence zvinhu zvinofanira kuitwa kubvira dzinenge mbiri pfungwa. Procedure rinoitwa kudiwa pasi zvavo yokutindivadza. In vana vakuru nemakore maviri, mumwe ponji hunobviswa mberi uye neshure anooneka zvikuru nemichinjiziri ilium kusvika 2 years - kubva calcaneal tuberosity tibial zvikamu kana zvinhu. Kazhinji Cytological morphological kuongororwa inoratidza hypercellular mwongo zvinhu pamwe kujekesa inomera kwomuzvarirwo hematopoietic maitiro uye infiltration pamusoro kwokurira kwose kwemabhosvo cellular hurongwa.

cytochemical tsvakurudzo

Uyu ndiwo mumwe zvinokosha nzendo nechirwere. Uchishandisa cytochemical staining chakaziviswa kuva sero mutsetse. Iva nechokwadi kuti kushandisa kutsvakurudza myeloperoxidase.

musana pombi

Uyewo, mutemo unoti chirwere yokuisa vanofungidzirwa chakasimba lymphoblastic neleukemia, zvikonzero ataurwa. Cerebrospinal ponji zvinofanira kuitwa pasi nyaradzo uye kana chokuvapo ari peripheral ropa zvinenge 30 zviuru / Rine mamiririta maplatelet. Kana zvakakodzera, pamberi nzira rinoitwa ropa trombovzvesi. Kuti agadzirire tsitopreparat kudiwa milliliters dzinenge mbiri nezvinobata. Pamusoro kuonekwa simba zvinhu uye zviratidzo kukuvara cranial tsinga chirwere neuroleukemia - leukemic CNS. Zvinofanira kucherechedzwa Zvisinei, kuti kupinda mumba tsinga dzose lymphoblasts uye infiltration kuti meninges kuitika pose pose, kunyange kusavapo kunotoda kurapwa uye mumarabhoritari kuratidzwa. Uyewo chinodiwa, uye kuziva miedzo chetsinga zviratidzo, iva nechokwadi kuti abate kombiyuta kana renyukireya rinobva tomography kuti musana kana uropi.

Other tsvakurudzo

Achibatsirwa Ultrasound retroperitoneal nzvimbo uye peritoneal mhango anogona kutaridzwa kukura parenchymal nhengo infiltrated, kuwedzere node nodes mudumbu uye dzimwe nharaunda, nhengo iri chiuno, manhu. Sechikamu nechirwere iri mubhokisi radiography mu pamaberere maviri. Izvi zvakakodzera kuona kuwedzera mediastinum. Apo akaratidza, anogadzwa X-ray mapfundo mapfupa. Mugore nokukanganisika ongororo iri kuitwa mukati murabhoritari zvidzidzo. Vazhinji zvinoratidza kuwedzera kupfuura 500 LDH Iu, zvinobvira nenyonganiso mubasa chiropa uye neitsvo. Asati atanga mishonga ndakarumbidzwa echocardiography uye ECG. Nhasi, nezvimwe nzira, ari utachiona muchitokisi kudzidza akashandisa samolecular kuvabereki uye cytogenetic nzira nokuda kuziva nhamba okutodza, uyewo STRUCTURAL kuchinja.

Treatment: Overview

Inokosha pamusoro izvo ano chiremba oncohematology iri akatsemura mapoka varwere pamusoro kurapa zvikuru. It anosarudzwa zvichienderana kwokuomerwa nako zvinobuda chete chenjera lymphoblastic neleukemia. Kufembera mamiriro kunokoshawo. Saka, Somuenzaniso, vaya kubvisa zvokurwara chaizvo mwero mishonga iri zvakasimba varumbidzwa varege kugamuchira zvakawanda chepfu uye inorema zvokushandisa. Uyezve, vaya vane vanofungidzirwa wokurarama haisi yakakwirira, ari intensification zvinhu aigona kuva mukana kupora. Kana kukamurwa kuva boka varwere nechirwere neleukemia, achifanotaura inoitwa pachishandiswa risati ruzivo, uye munhu protocols dziri Kunyora mugadziriro. Mumwe nomumwe wavo achiumbwa nomumwe kana vamwe nzira yose kuchirapa zviito imwe zvikuru. Under kuitika kupatsanura kushandisa vakawanda vanobvumirana pamusoro kuparadzira kubudikidza boka mukana varwere vakanga chirwere "chakasimba lymphoblastic neleukemia". Treatment vana yakazokudziridzwa mukupera sixties remakore rapfuura iri America. Kazhinji, nhungamiro pamusoro izvo kuchirapa mazano, havana ndavhiyiwa chaiwo kuchinja. In varwere pasi pemakore zvokurwara kunooneka pacharo nenzira nechisimba kwazvo nzira uye inozivikanwa neuroleukemia, leukocytosis.

Main kuchirapa nzira

Marapirwo anoshandisa zvinodhaka zvakadai se "Prednisolone" uye "Vincristine". Rinoshandiswawo endolyumbalnoe sumo rinoreva "Methotrexate" uye cranial irradiation yokudzivisa neuroleukemia. Uyewo, purogiramu kurapwa chaiyo yakazokudziridzwa. Izvi zvinoreva kuti varwere vose vanogamuchira yakajeka chipimo chemushonga uye mubatanidzwa chemotherapeutic vamiririri iri nguva puranga, ukuwo Protocol. By kuguma manonwe zvakava pachena kuti pashure kwemutemo chirongwa chakadai akapora vanenge 50% vana vave chirwere neleukemia. Zvikonzero kufambira mberi mukurapa zvirwere zvinokonzerwa tsanangudzo heterogeneity zvose uye zvadaro kudzidzisa hurongwa dzakawanda cytological kupatsanura uye prognostic zvikonzero, kuparadzira varwere mumapoka, pave kusiyaniswa zvirongwa. Zvikuru yaiva sangano multicenter tsvakurudzo kudzidza pharmacokinetics ose cytostatics siyana kuti hubudirire chemotherapeutic regimens, uyewo kukurumidza kukura zvakatevera kuitika.

Basic misimboti yokurapa

Nemhaka mabasa pamusoro apa, mapurogiramu vakasikwa chizvarwa himiolecheniya chitsva. protocols Most kushandiswa nhasi kwakavakirwa nenhungamiro kwokutanga ndichibvunza kurapa, chinangwa chiri kuti maximize kuparadzwa dziva masero mwero. At moyo kwezvisikwa chikumbiro chirongwa kuchirapa iri muchimiro mubatanidzwa cytostatics, anotevedzana (rotation), mukuru-dose chemotherapeutic regimens, uyewo kudzivirira chirwere nyanyisa.

Zvinoumba kushanda gadziriro

Huru hunorapa uye matanho pamwe intrathecal neuroleukemia vari rinoreva "Prednisolone" "Cytosar", "Methotrexate" (mu dosages maererano nezera) uye cranial irradiation. The yokupedzisira rinoitwa mukutanga zvokukura mushonga. Kana endolyumbalno raitongwa zvinodhaka "MTX" ane systemic nomugumisiro. Panyaya iyi, kuti kuderedza bundu gobvu anofanira kuva mangwanani kunhaka yokutanga kuchirapa lumbar ponji. Cranial irradiation ane kure uye yakananga nemishonga. Panyaya iyi, zveBhaibheri Optimization yokurapa ALL, kuitwa nhasi, zvainanga achidzikisa dose uye yavaive zvakajeka varwere vari pasi boka ngozi, nokuti idzo nemwaranzi ARV haamanikidzwi. kubudirira zvose izvi vakaita kuti zvikwanisike mukupera eighties remakore rapfuura akagadzirira kukunda 70 muzana chipingamupinyi-gore shanu wokurarama kuti neleukemia muvana pasina zvazvo.

Modern kuchirapa zvirongwa

A kurapwa rinoshandiswa nhasi, kunosanganisira maitikiro akawanda. Chokutanga ndiye induction chekanganwiro vatatu kana kupfuura nevasori vari kutarisirwa kwevhiki 4-6. Wechipiri - the nenzvimbo multi-nomumiririri yapfuura chokutambudzika. Third - rechengetedzo mishonga. Kazhinji, wokupedzisira chokutambudzika Anti-metabolites kushandiswa kwemakore 2-3. Kutsigira kurapa kunobatsira kudzivirira uye kubvisa vazhinji yematambudziko chokuita anonyengetedzwa cytopenia uye immunosuppression. Aifanira kushandisa Nokuraramisa kurapa kwemakore 2-3 rave vaudzewo kunyange pakati rokupedzisira remakore. Kazhinji panguva nguva iyi ose zuva murwere anotora zvinodhaka "6-Markaptopuriin". Ose vhiki akatanga senzira "Methotrexate". Zvichienderana nhamba masero ari yakatarwa chipimo chemushonga. Practice inoratidza kuti Varwere inogutsa tolerability iyi kuchirapa zvaiitwa. Achipa mabasa richitsigirwa 80% of inodiwa vhoriyamu anoonekwa kuva murombo prognostic raivapo dzokororo.

Kuti uwane mamwe mashoko,

Kune vakawanda matambudziko pakurapa ALL. Zvikurukuru, kuwedzera nhamba varwere mutated bundu masero miitiro inoburitsa zvinhu zvakanyatsofanana husingakwanisi mishonga, kudhura uye panowanikwa mukupera nemishonga (neuroendocrine zvinetso, ikure yechipiri anomera). Kukundikana induction pakurapa varwere nokuda mangwanani rufu nokuda ine chepfu yematambudziko kana bundu nemishonga. Uyewo chaizvo zvishoma kusvika kune mishonga wevarwere vasingadi kupindura mutemo kurapwa. Sezvo imwe nzira nzira inoshandiswa akadai "Teniposide" "Vepeside" "Cytosar". Nhasi anenge 70% vana vane makore 5 kanganwiro. Chenjera lymphoblastic neleukemia nedzevakuru kuripo kana ose wechinomwe. Izvi zvinoratidza kuti ya nguva kuregererwa zvinokwanisika kubudirira 20 chete% nyaya. Kazhinji, kudzoka zvokurwara kwakacherechedzwa ari okutanga 2 years pashure kuongorora chirwere. Kuregererwa ezvinhu inozivikanwa se kushaya simba iri mumasero ropa, kuvapo asingasviki 5% rinoparadza mu mwongo rinenge chimiro zviratidzo kuzoita hematopoiesis vasingambooni chakaipa nazvo. Ari kuongorora cerebrospinal mvura yakaratidza asingasviki 5 mononuclear masero / microliter. Nokuti kubudirira kurapwa nokukurumidza budiriro chekanganwiro mamiriro. Muna 90% wevarwere kazhinji masero kanganisika vane hanya mishonga.

In mhedziso

Chinangwa chikuru chemusha yokurapa ndiko kubvisa murwere zvokurwara wayo adzokera zvisingatomboiti nemagariro upenyu uye kuti utano hwakanaka ane zvishoma yematambudziko akakonzerwa kurapwa. Kuita izvozvo zvakaoma nhasi. Izvi imhaka yokuti vazhinji antileukemic mishonga hazvisi vanosarudza chaizvo uye chepfu. Zvisinei, kufambira mberi mune kunzwisisa kurapwa uye zvipenyu heterogeneity kwechirwere chacho, vakaramba kuitisa zvidzidzo siyana kupa tariro kuti samolecular pathogenesis chirwere vachange deciphered kusvikira pakuguma. Muchiitiko chino, zivo zvigadziriswe pakati akaipa kushanduka kuchabatsira kukudziridza hubudirire uye zvishoma chepfu kurapa.