
acute monoosaitik leukemia

acute monoosaitik leukemia (amol, allengil aaml-emm5) oru tharam acute myelooid leukemiayaanu. aaml-emm5ll> 80 sathamaanam raktarbuda koshangalum monoosaitik vamshaavaliyilaan. asthimajjayile monocytukalude aadhipathyamaan eee kaansarinte savisheshatha. sareerathinu paridhiyil aavashyamillaatha monocytukalude amita ulppaadanamundu. amithamaayi uthppaadippikkunna eee monocytukal saadhaarana rogaprathirodha kosha ulppaadanathe thadassappeduthunnu, ithu baadhicha vyakthikk niravadhi aarogya sankeernathakal undaakkunnu.
kaaranagal.
amlinte rogalakshanathil myelooid stem cellukalude janasamkhyayude asaadhaaranamaaya vyaapanavum vyathyasavum ulppedunnu. mikka kesukalilum janithaka vyathiyanangal thirichariyappedunnu. eee kesukalil thirichariyappedunna oru saadhaarana janithaka parivartthanatthe chromosomal translocationukalaayi visheshippikkunnu, avide oru chromosomil ninnulla vivarangal oru noonn-homologous chromosomilake kaimaattam cheyyappedunnu, ithu chromosomukalude asaadhaaranamaaya punakrameekaranam srishtikkunnu. eee translocation palappozhum tii (<aii. dii. 1> oru chromosome ulppedunnu, <aii. dii. 1> mattu chromosome ulppedunnu) ennu churukkipparayunnu. m5, palappozhum chromosome 11 ulppedunna tii (9; 11) polulla savisheshamaaya chromosomal asaadhaaranathakalumaayi bandhappettirikkunnu, ithu 11q23-ll mll (kmta 2) lokkasine badhikkunnu; ennirunnaalum mattu raktarbuda upavibhaagangalilum mll translocationukal kaanapedunnu. amolile tii (8; 16) translocation haemophagocytosisumayi bandhappettirikkunnu. eee translocationukal saadhaarana myelooid mungamiyude vikasanathe thadassappeduthunna chimeric proteinukalude (yathaakramam runks 1-runks 1 tii 1, pml-raara) roopeekaranam nalkunnu.
aaml-emm5 ulppettekkaavunna secondary raktarbudam etoposide polulla epippodophillaaduklas sanbarkkavumaayi bandhappettirikkunnu.
aaml-emm5-nte pala kesukalilum cytokainukalude vardhicha induction kaaranam staatt 3 proteente phosphorylation vardhippichathaayi kaanapedunnu, angane kosha vyaapanavum athijeevanavum vardhikkunnu. avasaanamaayi, epigenetic niyanthranatthil ulppedunna janithaka parivartthanangal eee rakthaarbudavumaayi bandhappettirikkunnu, kaaranam avaa koshangalude vyathyaasathilum vyaapanathilum thaazhekkulla phalangal undaakkunnu. amithamaya saitokin release vakramaaya epigenetic niyanthranatthinte upolppannamaakaam.
roganirnayam.
lokaarogya samghadanayude (aarr) maanadandangal paalikkunnathin, oru rogikk asthimajjayil 20 sathamaanathil kooduthal spodanangal undaayirikkanam, avayil 80 sathamaanathil kooduthal monoosaitik vamshathil pettavarayirikkanam. monoosaitik koshangal pradhaanamaayum monoblaastukalaano (> 80 sathamaanam) (acute monoblastic leukemia) allengil monoblastukaludeyum promonocytukaludeyum (<80 sathamaanam spodanangal) mishrithamaano ennathine aashrayich kooduthal upavibhagam (emm5eiii vezhsas emm5bii) undaakkunnu. ekadesham vrithaakrithiyilulla nucleus, athilolamaaya lassie kromaattin, samriddhamaaya, palappozhum basophilic cytoplasm ennivayiloode monoblastukale verthirichariyaan kazhiyum. eee koshangalkk seudopodukalum undakam. ithinu vipareethamaayi, promonocytukalkku kooduthal churunda nucleus undu, avayude cytoplaasathil metachrometic granuelukal adangiyirikkaam. monoblastukal saadhaaranayaayi emm. pii. oo-negatteevu aanu, promonocyteukal emm. pii. oo variablaanu. monoblastukalum promonocyteukalum noonn-specific estaresinu (enn. ezz. iii) positteev aayi kalayunnu, ennirunnaalum enn. ezz. iii palappozhum negatteevu aakam.
rogaprathirodhaprakrithiyil, emm5-aaml myelooid (sidi13, sidi33), monoosaitik (sidi11bii, sidi11sii) markerukal enniva vyathyasthamayi prakadippikkunnu. koshangal bii-sell marker sidi20 umm enke marker sidi56 umm asaadhaaranamaayi prakadippichekkaam. monoblastukal sidi34 nuu positteev aakam.
chikitsa.
theevramaaya chemotherapy (anthracyclins polullava) allengil asthi majja transplantation vazhiyaan aaml-emm5 chikilsikkunnathu. aaml-5 moolamundaakunna kosha vyaapana proteinukalude amithamaya sajeevamaakkaline cherukkunnathinaayi vikasippichedutha oru pradhana chikilsayaanu tyrosine kinez risapttar inhibittarukal. staatt 3 proteine thadayunnathu mattoru upayogapradamaaya chikilsayaanu.

