Izinto ezinobungozi, ukukhubazeka kweGenes, ukuTshintshiselwa kweStem Transpants kunye neJMML
Yintoni i-juvenile ye-myelomonocytic leukemia (JMML) kunye nendlela yokuphathwa ngayo?
I-Youth Myelomonocytic Leukemia (JMML) - Inkcazo
I-juvenile ye-myelomonocytic leukemia (JMML) yinto engabonakaliyo yomhlaza wegazi ochaphazelayo ochaphazela abantwana kunye nabantwana abancinci; ii-akhawunti ezingaphantsi kwe-1% yama-leukemias ebuntwaneni. Umyinge weminyaka yobudala ekuxilongwa ngabafundi abaneminyaka emi-2 ubudala, kwaye akuqhelekanga ukubona iimeko kwizingane ezineminyaka engaphezu kweminyaka emithandathu ubudala.
Ngama-2 ½ amaxesha aqhelekileyo ngaphezu kwamakhwenkwe kunamantombazana.
I-JMML iphinde ikhankanywe njenge-levemia engapheliyo ye-leememiagenous leukemia (JCML), isifo esingumhlala-mntwana esingasigxina se-granulocytic leukemia, esingapheliyo kunye nesifo sengqondo se-myelomonocytic leukemia.
Kwenzeka njani
I-JMML iyenzeka xa utshintsho (umzekelo, utshintsho) luqokelela kwi-DNA yohlobo lwe-stem cell kwi-bone marrow evulela ngokubanzi uhlobo lweseli eyaziwayo njenge- monocyte . Utshintsho lubangela ukuba iseli elichaphazelekayo liphindwe likhulu.
Njengoko iinombolo zala maseli angavamile, ziqala ukuthatha umongo wethambo. Emva kwexesha, baya kuphazamisana nomsebenzi osisiseko somongo, ozovelisa iiseli ezibomvu zegazi, iiseli zegazi ezimhlophe kunye neeplatelets.
Izinto zobungozi
Abaphandi abazi into ebangela ukuba utshintsho lwe-DNA olukhokelela kwi-JMML, kodwa izifundo ezintsha zibonisa ukuba izuze kubazali bomntwana.
Abantwana abanesifo se-neurofibromatosis I kunye ne-Noonan syndrome banomngcipheko okhulayo wokuphuhlisa i-JMML, kunye ne-14% yabantwana abafumana i-JMML ene-Noonan syndrome.
Iimpawu kunye neempawu zeJMML
Iimpawu neempawu ze-JMML zihlobene nokuqokelela kweeseli ezingaqhelekanga kwi-bone kunye namatyala. Zingaquka:
- Isikhumba
- Ukusulela rhoqo okanye ukugula
- Ukuphuma ngegazi
- Isisu esiswini esithintekayo kwipenti ekhulisiweyo ( splenomegaly ) kunye nesibindi esandisiweyo
- Iifutha
- Rash
- Ukunciphisa ukutya
- Isiqhamo
- Ukulibaziseka okuphuhlisayo
Ezi ziyakwazi ukubonakalisa iimpawu kunye neempawu zezinye iimeko ezingekho komhlaza. Ukuba unenkxalabo ngempilo yengane yakho, into enokuyenza yintyelela umboneleli wakho wezempilo.
Ukuchonga iJMML
Ukufumanisa i-JMML, oogqirha baya kuhlola iiphumo zeemvavanyo zegazi kwakunye nomnatha weethambo kunye ne-biopsy . Ezinye iziphumo ziza kubonisa iJMML:
- Inani eliphezulu lamhlophe yegazi, ikakhulukazi i-monocytes ephezulu
- Inani elibomvu lamaseli egazi (i-anemia)
- Inani eliphantsi leplatelet (thrombocytopenia)
- Ukungavumelekanga kwi-chromosome 7
Ukungabikho kwe- chromosome yaseFiladelphia kuya kunceda ukucacisa ukuba lesi sifo asiyiyo isifo esingapheliyo sesifo se-leukemia (CML) .
Izifo Iindlela
Kukho iindlela ezintathu ezahlukileyo apho olu hlobo lwe-leukemia lwenziwa. Kulo hlobo lokuqala uhlobo lwesifo luqhubekela phambili ngokukhawuleza. Ngoluhlobo lwesibini, kukho ixesha elide xa umntwana ezinzile, elandelwa yinkqubela phambili ngokukhawuleza. Ngoluhlobo lwesithathu, abantwana banokuphucula baze bahlale bebonakalisa impawu ezinobuncinane kangangeminyaka engama-9, ngexesha apho isifo siphuthuka ngokukhawuleza ngaphandle kwonyango.
Unyango lweJMML
Uphuhliso oluphambili lweJMML luyi-stem cell transplant, kodwa ngamanye amaxesha unyango lusetyenziswa kwakhona.
Ukuhlinzwa
Indima yonyango ekwenzeni i-JMML inokuphikisana.
Esinye isifundo somsebenzi esivamile (I-Children's Oncology Group- COG) sincoma u-splenectomy (ukususwa kwe-spleen) kubo bonke abantwana abanesifo esibandezelekile. Elinye iqela eliphambili le-JMML yonyango (i-European Working Group kwi-Myelodysplastic Syndrome ebuntwaneni-i-EWOG-MDS) ishiya isigqirha isiguli ukuba sigqibe. Ayaziwa ukuba inzuzo yangexesha elide le nkqubo iphakamisa ingozi.
Chemotherapy
Kukho konke, i-JMML ihlala isabela kakuhle kwi-chemotherapy. I-Purinethol (6- i-Mercaptopurine) kunye ne-Sotret (isotretinoin) yizidakamizwa ezisetyenzisiweyo ngomlinganiselo omncinci wokuphumelela.
Ngenxa yenzuzo eyincinci kwonyango lwe-JMML, i-chemotherapy ayiyona imilinganiselo.
Utshintsho lweStem Cell
I-Allogeneic stem cell transplant yodwa yonyango enokunika unyango olude lweJMML. Uphando lufumene amazinga aphumelelayo afanayo kunye nabaxhasi bee-cell stem okanye abaxhasi abangahambelaniyo.
Isantya sokubuyela emva kwe-stem cell transplant for JMML ingaba phakathi kwe-30% ne-50%, ephezulu kakhulu. Ukuphindaphinda kubakho umlinganiselo weenyanga ezi-3 ½ emva kokutsalwa, kwaye phantse njalo unyaka. Nangona kunjalo, nangona manani adikibalayo, izigulane zivame ukufezekisa unyango nge-second stem cell transplant.
Nangona kunesidingo sokonyango, abantwana abaneJMML benza ngcono ngakumbi ngokuqhubela phambili njengengxube ye-stem cell. Kufunyenwe kwiphando elithile lokuba izinga lokusinda iminyaka emi-5 yabantwana abafumana ama-stem cells asetshatshelweyo alingama-55%, ngokulinganiselwa kwiminyaka emihlanu engama-49% kulabo babenabanikeli abangabandakanyekanga, kwaye olu unyango luphucula lonke ixesha .
Ngaphantsi
Njengomzali, enye yezinto ezinzima kakhulu ukucinga ukuba umntwana wakho okanye umntwana uyagula. Olu hlobo lokugula lunokubeka ubunzima obukhulu kumntwana, kubazali kunye nakwabowabo. Unokuba nzima ukuchaza imeko enzima kubantwana bakho, ngaphandle kokukwazi ukufaka intloko yakho malunga nawe.
Zisebenzise nayiphi na amaqela enkxaso okanye izibonelelo ezibonelelwa ngumbindi wakho wesiko, kunye nabathandekayo bakho, abahlobo, osapho kunye nabamelwane. Nangona wena kunye nosapho lwakho unokuba nobuninzi beemvakalelo kunye neemvakalelo, kubalulekile ukukhumbula ukuba kukho ithemba lokunyanga nokuba abanye abantwana abaneJMML baya kuqhuba ubomi obunempilo novelisayo.
Imithombo
Chan, R., Cooper, T., Kratz, C., Weiss, B., noLoh, M. "I-juvenile ye-myelomonocytic leukemia: Ingxelo evela kwi-2 ye-International JMML Symposium" I- Leukemia Research 2009. 355-362.
Emanuel, P. "I-levemia ye-myelomonocytic leukemia kunye ne-myelomonocytic leukemia engapheliyo" I- leukemia 12 Juni 2008 1335-1342.
Locatelli, F., Nollke, P., Zecca, M. et al. I-Hamtopoietic stem cell transplantation (HSCT) kubantwana abanentsholongwane ye-myelomonocytic leukemia (JMML): iziphumo zetyala le-EWOG-MDS / EBMT. Igazi . 2005. 105 (1): 410-9
National Cancer Institute. I-PDQ yengxelo yeCcercer Information Summaries. Inguqu yezobugcisa. I-Childhood Enomdlavuza ye-Myeloid Leukemia / nezinye i-Myeloid Malignancies Treatment (PDQ®). Ishicilelwe kwi-intanethi 09/29/15. http://www.ncbi.nlm.nih.gov/books/NBK66019/#CDR0000062896__78
Niemeyer, C. kunye neKratz, C. "I-juvenile ye-myelomonocytic leukemia" Iindlela zokwelashwa zamanje kwi-Oncology 2003 4: 203-210.
Yoshimi, A., Kojima, S., noHirano, N. "I-juvenile ye-myelomonocytic leukemia: i-Epidemiology, i-etiopathogenesis, i-diagnostic, kunye nokuphathwa kweengxaki" Izidakamizwa zezilwanyana 2010 2010 1: 11-21.