Zinthu Zowopsya, Kuwonongeka kwa Gene, Kusintha kwa Magulu a Stem ndi JMML
Kodi mwana wa jermieocytic leukemia (JMML) ndi chiyani?
Achinyamata Omwe Amayiococytic Leukemia (JMML) - Tanthauzo
Matenda a khansa ya m'magazi (JMML) ndi mtundu wamba wa khansa ya magazi yomwe imakhudza ana ndi ana; amawerengera osachepera 1% a leukemias aunyamata. Avereji ya zaka za ana omwe ali ndi matendawa ali ndi zaka ziwiri, ndipo si zachilendo kuwona milandu ya ana oposa zaka zisanu ndi chimodzi.
Ndi nthawi 2 ½ zofala kwambiri kuposa anyamata kuposa atsikana.
JMML imatchulidwanso kuti mwana wamwamuna yemwe ali ndi matenda aakulu a khansa ya m'magazi (JCML), ana achikulire a granulocytic khansa ya m'magazi, osachiritsika komanso osagwirizana ndi matenda a khansa ya m'magazi, komanso a mwana wamwamuna.
Zomwe zimachitika
JMML imachitika pamene kusintha (mwachitsanzo, kusinthika kwa thupi) kumaphatikizapo mu DNA ya mtundu wa tsinde lapakhosi mu fupa la fupa lomwe limapereka mtundu wa selo wodziwika monga monocyte . Zosintha zimayambitsa selo yathandizidwa kuti ikhale yochuluka.
Pamene chiwerengero cha maselo osadziwikawa chikuwonjezeka, amayamba kutenga fupa. Pakapita nthawi, iwo adzasokoneza ntchito yaikulu ya msupa, yomwe imatulutsa maselo ofiira a magazi, maselo oyera a magazi ndi mapiritsi.
Zowopsa
Ochita kafukufuku samadziwa chomwe chimayambitsa kusintha kwa DNA komwe kumatsogolera ku JMML, koma maphunziro atsopano akusonyeza kuti akhoza kulandira kuchokera kwa makolo a mwanayo.
Ana omwe ali ndi matenda a neurofibromatosis I ndi Matenda a Noonan ali ndi chiopsezo chowonjezereka chokhala ndi JMML, ndipo ana 14 peresenti amapezeka ndi JMML okhala ndi matenda a Noonan.
Zizindikiro ndi Zizindikiro za JMML
Zizindikiro za JMML zimagwirizana ndi kusungunuka kwa maselo osadziwika mu mafupa ndi ziwalo. Zingaphatikizepo izi:
- Khungu lenileni
- Matenda opatsirana kapena matenda
- Kuchepetsa magazi
- Kutupa mimba yokhudzana ndi ntchentche yowonjezera ( splenomegaly ) ndi chiwindi chofutukuka
- Misozi
- Rash
- Kuchepetsa kudya
- Kukuda
- Kuchedwa kuchedwa
Izi zingakhalenso zizindikiro ndi zizindikiro za zina zomwe sizili ndi khansa. Ngati muli ndi nkhawa za thanzi la mwana wanu, chinthu chabwino kwambiri choti mupange ndi kupita kwa wothandizira zaumoyo wanu.
Kuzindikira JMML
Kuti adziwe JMML, madokotala adzafufuza zotsatira za kuyesa magazi komanso kupuma kwa fupa ndi fupa . Zotsatira zina zidzawonetsa JMML:
- Kuchuluka kwa maselo oyera a magazi, makamaka ma monocytes apamwamba
- Kuchuluka kwa maselo ofiira a magazi (kuchepa kwa magazi)
- Nambala yochepa ya platelet (thrombocytopenia)
- Zosokonekera mu chromosome 7
Kulephera kwa chromosome ya Philadelphia kudzathandizira kudziwa kuti matendawa sali odwala matenda a m'magazi (CML) .
Matenda a Matenda
Pali njira zitatu zosiyanitsira zomwe matendawa amachititsa. Mu mtundu woyamba matendawa akupita patsogolo mofulumira. Mwachiwiri, pali nthawi yochepa pamene mwanayo ali wodekha, akutsatiridwa ndi kupita patsogolo mofulumira. Mwachigawo chachitatu, ana angathe kusintha ndi kukhalabe ndi zozizwitsa zokha kwa zaka 9, panthawi yomwe matendawa amakula mofulumira popanda mankhwala.
Kuchiza kwa JMML
Chithandizo chachikulu cha JMML ndi kusamba kwa maselo, koma nthawi zina mankhwala amagwiritsidwanso ntchito.
Opaleshoni
Udindo wa opaleshoni mukuchiza JMML ndizovuta.
Pulogalamu imodzi yowunikira maphunziro (The Children's Oncology Group-COG) imalimbikitsa splenectomy (kuchotsa ntchentche) kwa ana onse omwe ali ndi matenda omwe afalikira. Njira yowonjezereka ya mankhwala a JMML (European Working Group pa Myelodysplastic Syndrome mu Childhood- EWOG-MDS) imasiya izi kwa wodwala wodwalayo kuti adziwe. Sidziwika ngati phindu la nthawi yaitali la njirayi liposa zoopsa.
Chemotherapy
Ponseponse, JMML nthawi zambiri imakhala ndi vuto la mankhwala a chemotherapy. Purinethol (6- Mercaptopurine) ndi Sotret (isotretinoin) ndi mankhwala amene agwiritsidwa ntchito ndi pang'ono.
Chifukwa cha kupindula kwake kochepa pa chithandizo cha JMML, chemotherapy siyomweyo.
Kusuntha kwa Cell Shem
Kulowetsa maselo a mitsempha yamagetsi ndi mankhwala okhawo omwe angapereke mankhwala a nthawi yaitali kwa JMML. Kafukufuku wapeza zotsatira zofanana zofanana ndi opereka ndalama zogwirizana ndi maselo a banja kapena ogwirizana osagwirizana.
Mlingo wa kubwezeretsedwa kwa maselo osakanikirana kwa JMML ukhoza kukhala pakati pa 30% ndi 50%, omwe ndi okwera kwambiri. Kubwereza kumachitika pafupifupi miyezi itatu ndi theka ndikutsata, ndipo pafupifupi nthawi zonse mkati mwa chaka. Komabe, ngakhale kuti pali ziwerengero zolepheretsa izi, odwala nthawi zambiri amapeza mankhwala ndi kachilombo ka kachiwiri kakang'ono.
Ngakhale kuti akufunikira kuzunza, ana omwe ali ndi JMML akuchita bwino kwambiri ndi kupita patsogolo monga maselo osungunuka. Anapezeka mufukufuku wina omwe anapeza kuti zaka zisanu zapakati pa ana omwe analandira maselo amtundu wa banja ndi 55%, omwe amakhala ndi zaka zisanu ndi zisanu ndi zisanu ndi zisanu (49%) omwe amakhala nawo operewera osagwirizana, ndipo chithandizochi chikukula nthawi zonse .
Mfundo Yofunika Kwambiri
Monga kholo, chimodzi mwa zovuta kwambiri kuganiza ndi mwana wanu kapena mwanayo akudwala. Matenda oterewa angayambitse mwana, makolo komanso abale ake mavuto aakulu. Mwina mukuvutika kuti mufotokozere ana anu zinthu zovuta, osakhoza kukulunga mutu wanu kuzungulira nokha.
Gwiritsani ntchito magulu onse othandizira kapena zopereka zomwe zimaperekedwa ndi malo anu a khansa, komanso okondedwa anu, abwenzi anu, abwenzi anu ndi anansi anu. Pamene inu ndi banja lanu mukhoza kukhala ndi maganizo osiyanasiyana ndikumverera, nkofunika kukumbukira kuti pali chiyembekezo chochiritsidwa ndi kuti ana ena omwe ali ndi JMML apitirize kukhala ndi moyo wathanzi komanso wopindulitsa.
Zotsatira
Chan, R., Cooper, T., Kratz, C., Weiss, B., ndi Loh, M. "Achinyamata a khansa ya khansa ya m'magazi: Lipoti lochokera ku 2 International Mommy JMML Msonkhano" Leukemia Research 2009. 355-362.
Emanuel, P. "Juvenile myelomonocytic leukemia ndi matenda a myelomonocytic khansa ya m'magazi" Leukemia 12 June 2008 1335-1342.
Locatelli, F., Nollke, P., Zecca, M. et al. Mankhwala osokoneza bongo (HSCT) omwe ali ndi ana omwe ali ndi khansa ya m'magazi (JMML): zotsatira za mayesero a EWOG-MDS / EBMT. Magazi . 2005. 105 (1): 410-9
National Cancer Institute. Mfundo zapakati pa khansa ya PDQ. Buku la zaumoyo. Childhood Acute Myeloid Leukemia / Other Myeloid Malignancies Chithandizo (PDQ®). Lofalitsidwa pa intaneti pa 09/29/15. http://www.ncbi.nlm.nih.gov/books/NBK66019/#CDR0000062896__78
Niemeyer, C. ndi Kratz, C. "Achinyamata omwe ali ndi khansa ya m'magazi".
Yoshimi, A., Kojima, S., ndi Hirano, N. "Achinyamata omwe ali ndi khansa ya m'magazi: Epidemiology, etiopathogenesis, matenda, matenda komanso matenda" Mankhwala Ogwiritsa Ntchito Mankhwala 2010 2010 1: 11-21.