Njodzi Zviitiko, Gene Kubvisa Zvinhu, Semu Cell Cell Transpants uye JMML
Chii chinonzi vana vechidiki myelomonocytic leukemia (JMML) uye inoshandiswa sei?
Juvenile Myelomonocytic Leukemia (JMML) - Tsanangudzo
Juvenile myelomonocytic leukemia (JMML) rudzi rushoma rwekenza yeropa ine utsinye inobata vana nevana vaduku; inoshandisa zvishoma kudarika 1% yevana leukemias. Ivhareji yezera revana vanorwara ndeyemakore maviri, uye hazvishamisi kuona zviitiko zvevana vaduku kupfuura makore matanhatu.
Iko 2 ½ kazhinji inowanikwa kune vakomana kupfuura vasikana.
JMML yave ichinziwo sevana vechidiki vasingazivikanwi neyeukemia (JCML), vana vechidiki vasingaverengeki granulocytic leukemia, vasingagumi uye vanopikisa myelomonocytic leukemia, uye vana vanokonzerwa neukemia 7.
Zvinoitika sei
JMML inowanikwa apo kuchinja (somuenzaniso, kuchinja-chinja) kunowedzera muDNA yemhando ye stem cell mu bone marrow iyo inowanzopa kune rudzi rwechikero chinonzi monocyte . Iko kuchinja kunokonzera kuti seni inobatwa iwedzere kunze kwekutonga.
Sezvo nhamba dzemasero aya asina kukwana ari kuwedzera, anotanga kutora marora emapfupa. Nokufamba kwenguva, ivo vachipindira nebasa guru remuropa, iro rinozobudisa masero matsvuku eropa, masero masero eropa uye maplatelet ane utano.
Risk Factors
Vatsvakurudzi havazivi chinokonzera kuchinja kweDNA kunotungamirira kuJMML, asi zvidzidzo zvitsva zvinoratidza kuti inogona kupiwa nhaka kubva kuvabereki vemwana.
Vana vane neurofibromatosis type I uye Noonan syndrome vane ngozi yakawanda yekuwedzera JMML, uye kusvika 14% yevana vanorwara neJMML vane Noonan syndrome.
Zviratidzo nezviratidzo zveJMML
Zviratidzo nezviratidzo zveJMML zvakabatana nekuunganidza kwemasero asina kukwana mumapfupa emafuta uye nhengo. Dzinogona kusanganisira:
- Pale pepa
- Kakawanda kurwara kana kurwara
- Kusachena kwechiropa
- Kudzora mimba yakabatana nekuwedzerwa chirwere ( splenomegaly ) uye chiropa chakawedzerwa
- Fever
- Mapundu
- Kuderedza kudya mwoyo
- Kukosora
- Kukura kunonoka
Izvi zvinogonawo kuva zviratidzo uye zviratidzo zvevamwe vasina maronda. Kana iwe uine hanya nehutano hwemwana wako, chinhu chakanakisisa chaunofanira kuita ndoshanyira mutengi wako wehutano.
Kuongorora JMML
Kuti uongorore JMML, vanachiremba vachaongorora migumisiro yeropa rekuongorora pamwe chete nemafuta emapfupa okuda uye biopsy . Zvimwe zviwanikwa zvicharatidza JMML:
- Chimwe chichena chemasero masero eropa, zvikurukuru monocytes
- Low red cell count count (anemia)
- Low platelet count (thrombocytopenia)
- Kuipa kwemukromosome 7
Kushayikwa kweiFirladelphia chromosome kunobatsira kuziva kuti chirwere chacho hachisi chirwere chisingaperi chine myelogenous leukemia (CML) .
Dendaase Paths
Kune zvikamu zvitatu zvakasiyana-siyana umo rudzi urwu rwehutachiona hunoita. Mune rudzi rwekutanga chirwere chinokurumidza kufambira mberi. Muchipiri rwechipiri, pane nguva inopera kana mwana akasimba, achiteverwa nekukurumidza kufambira mberi. Muchikamu chechitatu, vana vanogona kuvandudza uye kuramba vachiratidza zvinyoro zvinyoro zvishoma kusvika makore 9, panguva iyo chirwere chinokurumidza kufambira mberi pasina kurapwa.
Kurapa kweJMML
Iko kunyanya kurapwa kweJMML is stem cell transplant, asi pane dzimwe nguva kurapwa kunoshandiswawo zvakare.
Surgery
Basa rekuvhiyiwa mukurapa kweJMML rinokakavara.
Chimwe chidzidzo chinowanzobatwa (Children's Oncology Group- COG) inokurudzira splenectomy (spleen kubviswa) kune vose vana vane chirwere ichi chakawedzera. Icho chikuru chinonzi JMML chirongwa protocol (European Working Group paMyelodysplastic Syndrome muChidiki-EWOG-MDS) inoisiya kuchiremba wechiremba kuti asarudze. Hazvizivikanwe kana ruzivo rwekugara kwenguva ino rwekuita izvi huri nani kune ngozi.
Chemotherapy
Pamusoro pezvose, JMML inowanzova nehurombo husingapindirani kune chemotherapy. Purinethol (6- Mercaptopurine) uye Sotret (isotretinoin) mishonga yakave yakashandiswa nechechi duku chekubudirira.
Nemhaka yekubatsira kwayo zvishoma mukurapa kweJMML, chemotherapy haisi iyo yakaenzana.
Cell Cell Stp Transplant
Allogeneic stem cell transplant ndiyo chete chirwere chinogona kupa chirwere chenguva refu cheJMML. Kutsvakurudza kwakawana yakafanana kubudirira kwehutano pamwe chete nemhuri yakabatanidzwa yemasero emasero kana vakafananidza vatsigiri vasingabatanidzi.
Muyero wekudzokerazve shure kwe stem cell kushandiswa kweJMML inogona kuva pakati pe30% uye 50%, iyo yakanyanya kwazvo. Kudzoka kunowanikwa musvondo wemwedzi 3 ½ mushure mekutengesa, uye nguva dzose mukati megore. Zvisinei, pasinei nhamba idzi dzinoodza mwoyo, varwere vanowanzopora mushonga neechipiri stem cell transplant.
Pasinei nekudikanwa kwekurwadza, vana vane JMML vari kuita zviri nani nekufambira mberi kwakadai se stem cell transplants. Yakawanikwa mune imwe tsvakurudzo yemakore mashanu ekupona kwevana vakagamuchira zvakaenzana nemasero emakadzi emhuri vaiva 55%, vane makore mashanu ekupona kwema 49% kune avo vaiva nevatsigiri vasingabatanidzi, uye kurapwa uku kwavandudzika nguva yose .
The Bottom Line
Somubereki, chimwe chezvinhu zvakaoma zvikuru kufungidzira mwana wako kana mwana ari kurwara. Urwu urwere hunogona kuisa dambudziko guru kumwana, vabereki uye vanin'ina. Iwe unogona kunge uchinetseka kutsanangura mamiriro ezvinhu akaoma kuvana vako, usingakwanisi kuputira musoro wako uchipoteredza iwe pachako.
Shandisa zvese mapoka ekutsigira kana zvipo zvinopiwa nekenza yako, pamwe chete nevanodiwa, shamwari, mhuri uye vavakidzani. Nepo iwe nevemhuri yako mungave mune hutano hwakasiyana-siyana uye manzwiro, zvinokosha kuyeuka kuti pane tariro yekurapa uye kuti vamwe vana vane JMML vanoenderera mberi kutungamirira hupenyu hwakanaka uye hunogutsa.
Sources
Chan, R., Cooper, T., Kratz, C., Weiss, B., naLoh, M. "Vana vechidiki myelomonocytic leukemia: Mushumo unobva ku2 International JMML Symposium" Leukemia Research 2009. 355-362.
Emanuel, P. "Juvenile myelomonocytic leukemia uye isingaperi myelomonocytic leukemia" Leukemia 12 June 2008 1335-1342.
Locatelli, F., Nollke, P., Zecca, M. et al. Hamtopoietic stem cell transplantation (HSCT) mune vana vane vana vangu myelomonocytic leukemia (JMML): migumisiro yeEWOG-MDS / EBMT muedzo. Ropa . 2005. 105 (1): 410-9
National Cancer Institute. PDQ Cancer Information Summaries. Health expert version. Utano Hwakanaka Myeloid Leukemia / Zvimwe Myeloid Malignancies Treatment (PDQ®). Yakabudiswa paIndaneti 09/29/15. http://www.ncbi.nlm.nih.gov/books/NBK66019/#CDR0000062896__78
Niemeyer, C. uye Kratz, C. "Vana vechidiki myelomonocytic leukemia" Current Treatment Options muOncology 2003 4: 203-210.
Yoshimi, A., Kojima, S., uye Hirano, N. "Vana vechidiki myelomonocytic leukemia: Epidemiology, etiopathogenesis, kuongorora, uye kutarisirwa" Vanachiremba Mishonga 2010 2010 1: 11-21.