I-Myelodysplastic syndromes (MDS) yiqela leesifo sezifo zomnxeba ezinomngcipheko okhulayo wokuba yi -leukemia ye-myelogenous (AML) . Nangona ezi zifo zingaba neempawu ezahlukahlukeneyo kunye nonyango, into efana nayo yonke into yokuba ichaphazela indlela kunye nomnatha wethambo unako ukuvelisa iiseli zezempilo eziphilileyo.
Phantse abantu abayi-10 000 bavelise i-MDS eMelika ngamnye ngonyaka.
Amanye amagama asetyenziswa ukuchaza i-MDS yi-preleukemia, i-hematopoietic dysplasia, i-leukemia ye-myeloid, i-oligoblastic leukemia, okanye i-leukemia.
I-MDS Yakha njani?
I-MDS iqala ngomonakalo we-DNA okanye ukuguqulwa kwegazi elilodwa (i-hematopoietic) . Njengomphumo wale monakalo, umnatha wethambo uqala ukwandisa iiseli zegazi kwaye uqhutywe ngamaseli angaphanga okanye "aqhuma".
Kwi-MDS, kukho ukwanda kweseli yesistim efile (i-apoptosis), ekhokelela ekuphazamiseni umdla. Nangona kunokwandiswa kwemveliso yeeseli kumnkantso, abahlali ixesha elide ukukhutshwa ngaphandle kwegazi. Ngoko ke, abantu abane-MDS baya kuhlanjululwa yi- anemia (inani elibomvu lentsholongwane legazi,) i- thrombocytopenia (inani eliphantsi leplatelet), kunye ne- neutropenia (inani elisezantsi legazi legazi.)
Izinto zobungozi
Ayaziwa yintoni ebangela ukuba utshintsho olwenza ama-syndromes e-myelodysplastic, kunye ne-90% yexesha akukho sizathu esibalulekileyo sesi sifo.
Ezinye izinto ezinobungozi ezinxulumene nokunyuka ziquka:
- Ubudala: Iminyaka yobudala yokuxilongwa yi-70, nangona i-MDS ibonwe nakubantwana abancinane.
- Ukugqithisa imisebe - Abantu abaye bafumana unyango lweemithi zonyango lomhlaza, kunye nokuchasana kwimizila ye-ionizing kwiibhomu ze-athomu kunye neengozi zenyukliya zengozini eyongeziweyo.
- Ukunyuka kweekhemikhali: Ukubonakaliswa kwamakhemikhali e-organic, izitropu ezinzima, izichumisi, i-pesticides kunye ne-herbicide iphakamisa umngcipheko wesifo.
- Umsi wecuba
- Diesel ukukhupha
Ingaba i-Leukemia yangaphambili?
Ukulingana kwenani leemfomfele zomnkantso kubonisa ukuba isifo esibi kangakanani - iiseli ezingapheliyo, ezinzima kakhulu. Emva kokuba umrha wakho ubonisa ukuba abemi bawo benziwa ngamaseli angaphezu kwama-20%, iimeko zibhekwa njenge-AML.
Phantse i-30% yamatyala e-MDS aqhubela phambili kwi-AML. Nangona kunjalo, kubalulekile ukuqaphela ukuba nangona inguqu ingekaze ivele, i-anemia, thrombocytopenia, ne-neutropenia echaphazelekayo ne-MDS isasongela ubomi.
Iincinci
Ukufumana ukuxilongwa kwe-MDS kungekuphela nje ukuquka izifo ezininzi ezahlukeneyo zomnatha weethambo, kukho iimeko ezininzi ngaphakathi kwezi zimilo eziqinisekisa ukuziphatha nokuchazwa kwesifo. Ngenxa yoko, izazinzulu ziye zazabalazela ukuza kunye nenkqubo yokuhlengahlengayo eqwalasela yonke into ehlukeneyo.
Iyokuqala kwezi nkqubo zikwahlula isiFrentshi-American-British (FAB). Iphula i-MDS ukuya kuma-subtypes ama-5 ngokusekelwe kwindlela umongo wethambo ubheka ngayo kunye neziphumo zesibalo segazi esigqibeleleyo (CBC) :
- I-anemia ye-refractory (RA)
- I-anemia ye-Refractory enee-sideroblasts eziphambeneyo (RARS)
- I-anemia ye-refractory kunye nokuqhuma okungaphezulu (RAEB)
- I-anemia ye-Refractory enefuthe elingaphezulu kweenguqu (RAEB-T)
- I-lemonemic i-monomyelocytic leukemia (CMML)
Ukususela ekuphuhlisweni kweemfuno ze-FAB ngo-1982, izazinzulu ziye zafunda okungakumbi malunga nokungaqhelekanga kofuzo olukhokelela kwi-MDS kunye nendima ezithintela ngayo ukuguquka kwezi zifo. Ngenxa yoko, ngo-2001, i-World Health Organization (WHO) yapapasha ezinye iinguqu kwi-FAB system. Banezela ezinye iimeko-5q- syndrome, i-MDS engaxhamlikiyo (MDS-U), kunye ne-cytopenia engabonakaliyo kunye ne-multilineage dysplasia (i-RCMD) - kwaye yabelana nabanye njengama-RAEB kunye ne-CMML ngokusekelwe kwipesenteji zomoya.
Baphinde bacacise ukuba nantoni na ephezulu ngaphezu kwe-20% yokuqhuma komnkantsha yenze i-AML, eyenza i-RAEB-T i-leukemia ngokuchasene ne-MDS.
Indlela yesithathu yokwenza i-MDS isebenzisa i-International Prognostic Scoring System (IPSS). Le nkqubo isebenzisa imigaqo emithathu yokumisela indlela i-MDS eya kuqhuba ngayo: inani leeseli kwisigulane segazi elijikelezayo, inani lezitshixo ezingapheliyo kumnxeba wethambo, kunye ne-cytogenetics (uhlobo lwezinto ezingafaniyo eziphathekayo ezinxulumene ne-MDS).
Ngokusekelwe kwezi zinto, i-IPSS ihlukanisa izigulane zibe ziindidi ezine ezibonisa "ingozi" ye-MDS-low, ephakathi-1, ephakathi-2, nangaphezulu. I-IPSS inikezela indlela ephuculweyo yokuqikelela iziphumo ze-MDS, ukugqiba ukuxela, nokucwangcisa unyango.
IiMDC eziPrayimari nakwiSibini
Kwizigulane ezininzi, i-MDS ibonakala ihlakulela isizathu esingaziwayo, ngaphandle kwebala. Oku kubizwa ngokuba yi-primary okanye de novo MDS. Njengoko kwimeko ye-leukemia kunye nezinye izifo zengxangxongo yamathambo, izazinzulu aziqinisekanga ukuba zeziphi iingxaki ezibalulekileyo ze-MDS.
I-MDS yesibini ibhekisela kwimeko xa ilandela unyango lwangaphambili kunye ne-chemotherapy okanye unyango lwe-radiation.
Ukuxilongwa
I-MDS ifunyaniswa ukuba isebenzisa iindlela ezifanayo ezisetyenziselwa ukuxilonga i-leukemia .
Isinyathelo sokuqala kukuvavanya igazi elijikelezayo legazi ngenxa yegazi elipheleleyo (CBC). Olu vavanyo lujonge inani leempawu zegazi ezibomvu, amangqamuzana egazi ezimhlophe kunye neeplatelets egazini ukufumana ingcamango jikelele yento eyenzekayo emlonyeni. Kwiimeko ezininzi, umntu one-MDS uza kubonisa inani elingaphantsi kweeseli ezibomvu zegazi (i-anemia), kwaye mhlawumbi iiplatelet eziphantsi (thrombocytopenia) kunye neutrophils (neutropenia) kunye.
Ukuba akukho mnye umphumo onokufunyanwa kwisigulane sokuba ne-anemia, oogqirha baya kuthi benze umongo we-aspirate kunye ne-biopsy . Kwisigulane nge-MDS, umongo uya kubonisa ukubonakala okungavumelekanga kunye nenani elandisiweyo lezisele ezifayo okanye "eziqhuma". Xa iiseli zihlolwe kwiqondo lemfuza, ziya kubonisa ukuguquka okanye utshintsho kuma-chromosomes.
Izimpawu kunye neMpawu
Izigulane ezine-MDS zinokufumana iimpawu ze-anemia ezinje:
- Ukuphefumula okuncinci ngokuncinci
- Isikhumba
- Ndiziva ndikhathele
- Iintlungu zesifuba
- Utywala
Izigulane ezimbalwa ziza kuba nemiqondiso ye-neutropenia kunye ne-thrombocytopenia, kuquka iingxaki zokuphuma kwegazi kunye nobunzima bokulwa nezifo.
Kubalulekile ukuba uqaphele ukuba kukho ezinye ezininzi, izimo ezingathí sina ezingabangela le miqondiso kunye neempawu. Ukuba unenkxalabo malunga nayiphi na inkxalabo yezempilo ojongene nayo, kukulungele ukuxoxa nabo nodokotela wakho okanye nabanye ootitshala bezonyango.
Ukuqulunqa
I-MDS ayisinye isifo, kunokuba iqela leemeko ezenza utshintsho kwindlela umnkantso wethambo usebenza ngayo.
Njengoko inzululwazi ifundela ngakumbi malunga ne-genetics kunye nendima abayidlalayo ekuphuhlisweni kwezi ntloba zezifo, sifunda ngakumbi malunga nezinto ezenza ikhosi abaza kuyithatha kunye neziphumo ezinokwenzeka. Kwixesha elizayo, abaphandi baya kukwazi ukusebenzisa olu lwazi ukudala iindlela ezintsha zokwelapha ze-MDS.
Imithombo:
Goldberg, S., Chen, E., Corral, M., et al. "Isiganeko kunye neengxaki zeZliniki zeMyelodysplastic Syndromes Phakathi kweUnited States I-Medicare Beneficiaries" I- Journal ye-Clinical Oncology ngoJuni 2010. 28: 2847-2852.
Bowen, D. "Ukulawulwa kwezigulane ngeMyelodysplastic Syndromes: Iingcamango zokuqala" kuDeeg, H., Bowen, D., Gore, S., Haferlach, T, Beau, M, Niemeyer, C. (eds) (2006) ) I-Hematologic Izibhambathiso: iMyelodysplastic Syndromes. Springer: eNew York. (iphe 89-94).
Haferlach, T., Kern, W. "Ukwahlula kunye nokuHlelwa kweMyelodysplastic Syndromes" kwiDeeg, H., Bowen, D., Gore, S., Haferlach, T, Beau, M, Niemeyer, C. (eds) (2006) I-Hematologic Izibhambathiso: iMyelodysplastic Syndromes. Springer: eNew York. (iphe.40- 51).
National Cancer Institute. I-PDQ yengxelo yeCcercer Information Summaries. Unyango lweMyelodysplastic Syndromes. INgxelo yezobuGcisa. 04/02/15. http://www.ncbi.nlm.nih.gov/books/NBK66015/#CDR0000062929__1
UNimer, S. "iMyelodysplastic Syndromes" Igazi likaMeyi 2008. 111: 4841- 4851.
UScott, B., Deeg, J. "I-Myelodysplastic Syndromes" Ukuhlaziywa koNyaka kaMagqirha ka- 2010. 61: 345-358.