Tātaritanga me te maimoatanga o te kanisā lymphoblastic whakapeka. tohu

kanisā Lymphoblastic, toto whakaaro te ki hei te tino noa oncological māuiuitanga tamariki. Ko reira e pā ana ki te 25% o pukupuku kitea i roto i te Fale-. Next, tango i te ofi titiro toto kanisā - he aha te mea te reira, he aha te mea i reira, me te aha wawaotanga haumanu e kawea atu i roto i tenei māuiuitanga.

Overview

toto kanisā - he aha te mea te reira? E pā ana te mate ki te rōpū o te mate pukupuku. KATOA (lymphoblastic whakapeka kanisā), te tohu o e ka e kōrero i raro, ko te rōpū heterogeneous o neoplasms o ngā pūtau e whai āhuatanga ira, me te immunophenotypic motuhake. Whakanuia te hanga, me te haaputuputuraa o enei pūtau i roto i te abnormalities wheua wheua whai wāhi tini tuarua, pārōnaki ranei e rua. Ka rite ki te hua, kookuhutanga o ngā pūtau o whekau parenchymatous me taikiri. Te nuinga (e pā ana ki 80%) o te kanisā (kanisā) katoa, kitea i roto i ngā tamariki, he takenga mai lymphoid.

nuinga

E kitea mate toto mārere i roto i te ao nei i teie mahana i nga wahi katoa. nuinga KATOA i roto i nga whenua whakawhanakehia he 3-4 take ia tamariki rau mano i ia tau. I roto i nga whenua o Central Asia ko Africa he iti noa māuiuitanga. maha KATOA i roto i te āhua US o Hapani, China, Europe. whakaaro te pā tihi i roto i ngā tamariki te ki hei te tau o 2.5-5 tau. puta te pīari tino noa i roto i te mau tamaroa.

predisposing āhuatanga

Tuatahi o te katoa kia pēnei ratou ki te tau o te whaea tiaki prenatal, taimaha whanau tiketike - neke atu i te 4000 karamu, tūpāpaku me te prenatal te rongo ki ionizing radiation. I roto i ētahi wā kia ara puta ai ki te kanisā lymphoblastic whakapeka? History i te mate a whaea kia ngā mōhiohio e pā ana ki te mate fetal i roto i te hapū o mua. Tenei i taea e puta tika ki ngā āhuatanga taiao kino, e tika ana ranei ki te predisposition ira. I roto i te take o te tūroro mate pukupuku i te whare o whanaunga, te whakanui ake i te tūponotanga e ka whakatika whakatupuranga heke mai kanisā lymphoblastic whakapeka. kia whai pānga te take o te mate ki te syndromes pūira tapepe huānga. Ko ētahi o ēnei, i roto i ngā, ngā raro syndrome, neurofibromatosis, ārai tāupe, Fanconi anemia, X-hono agammaglobulinemia (congenital) me ētahi atu. Ka rite ki te hua, he maha o ngā akoranga, kua tae mai tohunga ki te mutunga e i roto i te kanisā lymphoblastic whakapeka, me te te whanaketanga o ētahi atu matepukupuku, i reira te mea he irakētanga māhorahora o te pūtau tupuna. Ia muri hanga Launchers āhuatanga taiao e whakahohe tini. Ko te āhuatanga tino noa e taea meinga kanisā tenei. māuiuitanga take vozniknovneniya ko tetahi o nga āhuatanga taketake ina tīpako whakaora.

tātaringa

A, no te tāutu i tetahi Cancer māuiuitanga ka tangohia ki pūkete nga āhuatanga haumanu me ngā āhuatanga o te pūtau e hanga ake te tïpako puku. hāngai ana tātaritanga KATOA kei i runga i te whakamātautau tinana me te hītori raraunga me te taiwhanga rangahau paraclinical.

pikitia haumanu

Tohu o te kanisā he syndromes matatini ake:

• Haurangi. I roto i te kanisā lymphoblastic whakapeka he ahuatanga i tohua, mate taimaha, te rohirohi, kirika. Ko te whakamutunga taea te pōreareatanga e te viral, kitakita, protozoal (onge), ranei mate fungal, ngā i roto i tūroro ki neutropenia.
• Hyperplastic. A, no te whakapeka kanisā lymphoblastic ko kookuhutanga o te kōateate me ate. Tenei, i roto i te tahuri, arata'i ki hepatosplenomegaly, e taea whakaatu aroha i roto i te puku. Ko te tohu tuatahi o te kanisā - piere pathological o wheua roa tuaiwi ranei. tuatahi kia tetahi raru me te ngawhatanga o te hononga iho kia hala mo tohumate o kaikōiwi atu disorders ranei. mate wheua - mō te tapû-. A, no te puta kookuhutanga leukemic o te capsule articular me te puku kiriuhi wheua, ngakau me te wheua wheua te whakanui ake i roto i te rōrahi whānui mamae. I te wa ano i runga i te X-ray kitea āhuatanga o huringa pathological. Rawa e mārama kitea tata nga hononga nui, me wheua roa ratou. kia puta i muri tetahi raru e tika ana ki te necrosis avascular me te osteoporosis.
• Anemic. I roto i te kanisā lymphoblastic whakapeka, whakanuia tachycardia, ataahua, toto o kiriuhi mucous i roto i te mangai, hemorrhagic syndrome i runga i te kiri. Nā ki te haurangi me te anemia whakawhanake ngoikore.

faaiteraa māuiuitanga

I roto i te 5-30% o ngā take o tamariki kanisā limfblastnogo whakapeka tuatahi kitea whakarahi testicular MATAMUA. He kua rua unilateral ranei infiltrates mātotoru ongonoa. I roto i te mahi, he hua nui i roto i te wā korerotia o te tākihi. I roto i tenei take, e kore ai e wātea faaiteraa haumanu o ratou mate. pōauautanga Rare ngā pericarditis exudative me te kookuhutanga myocardial o te waitinana arai i runga i te whakamārama i waenganui i te epicardium me endocardium. Ki te hua i roto i te taikiri i roto i te mediastinum kia puta raruraru i roto i te mahi o te pūnaha romahā. Enei āhuatanga o momo kanisā T-pūtau mata ki te syndrome iahoki vena cava pai. Ka taea hoki e puta te reira me kore romahā. I roto i ētahi wā kookuhutanga reira leukaemic o te kiko pūkahukahu toto ranei i roto ia ratou. I te tahi taime e uaua ki te rerekëtanga raraunga i pōauautanga o pathologies hopuhopu tohunga. Me kï ētahi tohu noa o te kino kanohi ki kanisā lymphoblastic whakapeka toto i roto i te etine, edema kōmata i roto i te karere whatu, kookuhutanga iaia. A, no te taea te kitea ophthalmoscopy i te tohu i te raro o te 'Afio. Pea hoki te aroaro o mātotoru huānga mamae kore infiltrative kikorangi i runga i te kiri. Ka taea e mate puta i tetahi uhi kino. I roto i tenei whakaaro, kia tāutuhia ai te tukanga tātaritanga panaritiums, paronychia, ngau pepeke huaketo rerekē, punctures me m. P.

tūhuratanga Paraclinical: oki

Ka taea te iti noa ranei pūtoto. Hematocrit, me tatau pūtau toto whero te nuinga o te wā iti. heke i tohua i roto i te maha o reticulocytes. kia heke ai White tatau pūtau toto, nui haere noa ranei. Heoi, kei te kore e taea te kitea i reira te pūtau mana i roto i te wā katoa. Hoki āhuatanga māuiuitanga "kore leukemic." Kei te korero tatou e pā ana ki te aroaro o ngā pūtau hau i roto i te papamuri o te kore o ngā puka takawaenga - metamyelocytes me myelocytes - i roto i te tātai toto. Ko te tikanga kitea thrombocytopenia.

myelogram

Kei te tango i tēnei tukanga te poka wheua wheua. kia rauemi taiepa kia hanga i te iti rawa kia rua ngā. kawea Hātepe i roto i pai i raro i rehunga whānui. I roto i ngā tamariki pakeke ake i te rua tau, tangohia te poka i te mua me tupuke muri Illium ake ki te 2 tau - i te wāhanga tibial calcaneal tuberosity huānga ranei. te tikanga heheu whakamātautau morphological Cytological rauemi wheua hypercellular ki tupu whāiti pūnaha hematopoietic noa, me te kookuhutanga o blasts hanganga pūtau.

rangahau cytochemical

Tenei Ko tetahi o nga wāhanga faufaa o te tātaritanga. Mā te whakamahi i te poke cytochemical whakakitea ki no ki te raina pūtau. Kia tino ki te tono rangahau i runga i myeloperoxidase.

tap tuaiwi

Ko reira ano hoki whakahauanga tukanga tātaritanga i roto i whakapae kanisā lymphoblastic whakapeka, te take whakahuatia i runga ake. kia poka Cerebrospinal kia whakamana i raro i whakarokiroki me roto i te take o te aroaro i roto i te toto piringa o te iti rawa 30 mano / platelets ml. Ki te tika, i mua i whakamana i te tikanga e huhu trombovzvesi. No te faaineine i tsitopreparat hiahia i te iti rawa e rua milliliters o waipiro. Ki runga ki te rapunga o nga mea timatanga mana me nga tohu o te kino ki te raru cranial pāngia neuroleukemia - leukemic CNS. Me kï hoki, Heoi, e te kătahi ki te pūnaha io o lymphoblasts me kookuhutanga o meninges puta i roto i te wā katoa, ara i roto i te ngaro o mau faaiteraa haumanu me te taiwhanga. Ko reira ano hoki e minaminatia, me te rapunga o tohu neurological, kia mohio ki te mau i te rorohiko karihi tomography autō o te ua roro ranei ranei.

Ētahi atu rangahau

Ki te awhina o wāhi ultrasound retroperitoneal me kōhao peritoneal taea whakapūtā te rahi o te okana parenchymal infiltrated, taikiri rahi i roto i te wāhi puku, me te tahi atu, whēkau i roto i te papatoiake, komurumurua. Hei wāhanga o te tātaritanga he pouaka Radiography i rua haurangi. Ko te tika tenei ki te kitea te hua i roto i te mediastinum. Hea tohua, i whakaritea te X-ray o hononga me wheua. I roto i te tātari matüora kawea i roto i roto i te akoranga taiwhanga. Kei te te tikanga whakaatu te reira i te hua nui ake i 500 LDH Iu, raruraru taea i roto i te mahi o te ate, me nga whatukuhu. I mua i tīmata hahau tūtohutia e echocardiography me ECG. I teie mahana, i roto i era atu tikanga, i roto i te ako pūtau pangia whakamahia he tikanga iranga, me te cytogenetic rāpoi mō te whakatau i te maha o ngā pūira, me te huringa hanganga.

Treatment: Overview

Te parau tumu taketake ki runga ki nei te wahia hou oncohematology pediatric ki ngā rōpū o tūroro i runga i therapy kaha. Kei te tīpakohia te reira i runga i te pakeke ki nei puta ki te kanisā lymphoblastic whakapeka rānei. He nui ano hoki kāwanatanga matapae. Na, mo te tauira, te hunga e e ki te whakakore i te māuiuitanga e kaha tūtohutia rawa hahau āhua kore ki te riro taputapu atu paitini, me te taimaha. I tua atu, hoki te hunga nei pea, oranga auau ko kore pera tiketike, te whakakahanga o ngā mahi i taea e te tupono o te ora. A, no te te wehenga ki te rōpū o tūroro pāngia ki kanisā, hanga matapae i runga i te pūtake o te wheako mua, a ngā tikanga takitahi i roto i te pūnaha tapanga. Ia o ratou i hanga e tetahi te atu rautaki o mahi whakaora o te tetahi kaha ranei. I raro i te whakarōpū nāianei tangohia te whakaaetanga whānui i runga i te tohatoha i te rōpū mōrea o tūroro i pāngia "kanisā lymphoblastic whakapeka". I whakawhanakehia maimoatanga o tamariki i roto i te mutunga o nga Heoi o te rau tau whakamutunga i roto i Amerika. Na roto i te a nui, te mau parau tumu i nei te tātai haumanu, kua kore oti huringa motuhake. I roto i te tūroro i raro i te tau o te māuiuitanga faaite ake i roto i te ara tino pukuriri, me te te āhuatanga neuroleukemia, leukocytosis.

tikanga whakaora Main

Ka whakamahi te maimoatanga taero pērā i "Prednisolone" me "Vincristine". Kei te whakamahia hoki te reira i endolyumbalnoe introduction tikanga "Methotrexate" me te irradiation cranial mō te ārai neuroleukemia. Ano, i whakawhanakehia tētahi hōtaka maimoatanga motuhake. Te tikanga o tēnei e farii i tūroro katoa etahi inenga mārama me ngā o āpiha chemotherapeutic i roto i te wā motuhake, aua kawa. Na roto i te mutunga o nga hitu ahuru ka reira mārama e muri i te wahi o te hōtaka taua whakahokia mai e pā ana ki te 50% o ngā tamariki e kua pāngia ki te kanisā. Ko te take mō te atu te ahunga whakamua i roto i te maimoatanga o mate meinga e te whakamāramatanga o te heterogeneity o KATOA me muri whakauru i te pūnaha o te cytological ao whakarōpū me ngā āhuatanga prognostic, te tohatoha o tūroro i roto i ngā rōpū, te whanaketanga o ngā hōtaka rerekëtanga. O te faufaa nui ko te whakahaere o te ako rangahau multicenter o nga pharmacokinetics o cytostatics rerekē mō te regimens chemotherapeutic whai hua ake, me te te whanaketanga tere o ngā tahi.

te mau parau tumu Basic o therapy

Ngā mihi ki te mahi whakaahuatia ana i runga, i hanga te hōtaka himiolecheniya whakatupuranga hou. hāngai ana te nuinga o ngā tikanga e whakamahia ana i tenei ra e i runga i te mau parau tumu o tuatahi therapy kaha, te whakaaro o te mea ki te whakanui i te whakangaromanga o te poka wai o ngā pūtau rerekē. I te ngakau o te hanganga o te hōtaka tono whakaora kei roto i te puka o ngā o cytostatics, ngā (hurihanga), tiketike-horopeta regimens chemotherapeutic, me te he ārai mate kaha.

Āhuatanga o te tono o teuteu

Ko te mehua curative me ārai matua ki neuroleukemia intrathecal he tikanga "Prednisolone" "Cytosar", "Methotrexate" (i roto i te toketaá rite ki tau) me te irradiation cranial. kawea te whakamutunga i roto i roto i te tīmatanga o te therapy. A, no te endolyumbalno raau whakahaere "MTX" kua he pānga pūnahanaha. I roto i tenei whakaaro, i roto i te tikanga ki te whakaiti i te papatipu puku me waiho hei kainga wawe o te tuatahi poka lumbar whakaora. He pānga taha mamao, me te hāngai irradiation cranial. I roto i tenei whakaaro, rangahau i runga i arotautanga o therapy KATOA, whakahaere i tenei ra, e hāngai ana ki te whakaiti i te horopeta me te mārama tohatoha o tūroro i roto i te rōpū mōrearea iti, no te mea e kore te te therapy radiation hiahiatia. kua hanga enei whakatutukitanga katoa taea reira i roto i te mutunga o nga eighties o te rau tau whakamutunga he rite ki te hinga i te 70 ōrau arai-tau e rima auau ora mō kanisā i roto i ngā tamariki, kahore relapses.

hōtaka whakaora Modern

He maimoatanga whakamahia e te tenei ra, whai wāhi te maha o ngā wāhanga. Ko te tuatahi kei te e faaterehia te whakaurutanga mai o te murunga ki toru, neke atu rānei māngai mō te 4-6 wiki. Ko te tuarua - te whakatōpū o maha-māngai o te wāhanga o mua. therapy tiaki - Tuatoru. Ka rite ki te tikanga, te wāhanga whakamutunga o te anti-metabolites whakamahia mō te 2-3 tau. Te tautoko i therapy āwhina ki te ārai me te tokomaha o nga pōauautanga e pā ana ki cytopenia piri me immunosuppression faaore. Ko te hiahia ki te tono i te therapy oranga ano hoki mo 2-3 tau i kï kua ara i roto i te waenganui o te rau tau whakamutunga. Tikanga i roto i tenei wā katoa ra te manawanui e te tarukino "6-Markaptopuriin". Katoa wiki whakaurua e ia he tikanga o "Methotrexate". Tei runga i te maha o ngā pūtau he huinga inenga. E whakaatu ana Practice e i roto i tūroro tolerability pai o tenei talena whakaora. whakaaro Execution o nga mahi e tautokona ana e 80% o te rōrahi hiahiatia te ki te waiho i te take prognostic rawakore mō te hohokitanga.

Mō ētahi atu pārongo,

He he maha o ngā raruraru i roto i te maimoatanga o KATOA. I roto i te ngā, he maha piki haere o tūroro ki iramate pūtau puku mutated ātete ki te hahau, utu nui, me te takanga o ngā pānga taha mutunga (disorders neuroendocrine, tupu o te pukupuku tuarua). Ko te kore o te maimoatanga whakaurutanga o tūroro e tika ana ki te mate wawe e tika ana ki pōauautanga paitini puku ātete ranei. Hoki rawa iti huarahi ki te therapy o tūroro e kore e urupare ki te maimoatanga paerewa. Ka rite ki te kē tikanga whakamahia te pērā i "Teniposide" "Vepeside" "Cytosar". I teie mahana e pā ana ki 70% o ngā tamariki i te murunga-tau 5. Whakapeka kanisā lymphoblastic i roto i ngā pakeke mai anō ko i te whitu o nga take. Tenei kōrero e te roanga o te wā o te murunga he taea ki te whakatutuki% 20 anake o te wā. Ka rite ki te tikanga, te hoki o te māuiuitanga kitea i roto i te 2 nga tau tuatahi i muri i te tātaritanga. Murunga huru āhuatanga rite te kore o te mana i roto i nga pūtau toto, te aroaro o iti iho i te 5% blasts i roto i te wheua hinu wheua kikokiko, te ahua o nga tohu o te ora o te hematopoiesis täkupu. I roto i te tātari o te inu cerebrospinal whakakitea iti iho i te 5 ngā pūtau mononuclear / microliter. Hoki te angitu o te maimoatanga ki te paetae tere o te mana murunga. I roto i te 90% o tūroro e te tikanga e tairongo ki te hahau pūtau rerekē.

I roto i te mutunga

Ko te whāinga matua o te therapy ko ki te pehea e o te māuiuitanga manawanui me tona hoki ki te ora tonu pāpori, me te ki te hauora pai me te iti o pōauautanga meinga e te maimoatanga. Hei whakatutuki i te reira rawa uaua tenei ra. Ko e tika ana ki te meka e kore e te nuinga o rongoā antileukemic rawa whiriwhiri, me te paitini tenei. Heoi, Kōkiritanga i te māramatanga o te heterogeneity haumanu, me te koiora o te mate, tonu whakahaere ngā rangahau hoatu tumanako e tonu ka kia deciphered te pathogenesis rāpoi o te mate tae noa ki te mutunga. I roto i tenei take, ka āwhina i te matauranga o nga tikanga o te huringa mārere ki te whakawhanake i whakaora ake whai hua, me te iti iho paitini.