Ama-Syndromes aseMyelodysplastic (MDS)

Izifo ze-Myelodysplastic (MDS) ziyizifo zezifo zomnumongo ezineengozi eyengeziwe yokwenza i-myelogenous leukemia (AML) . Ngenkathi lezi zifo zingase zibe nezimpawu ezihlukene kanye nokwelapha, into eyodwa abafana nayo yilezi zithinta ukuthi umthambo wethambo unamandla kangakanani ekukhiqizeni amaseli egazi enempilo.

Cishe abantu abayi-10 000 bakhulisa i-MDS e-United States unyaka ngamunye.

Amanye amagama asetshenziselwa ukuchaza i-MDS yi-preleukemia, i-dysplasia ye-hematopoietic, i-suyocute leememiaid leukemia, i-legoemia ye-oligoblastic, noma i-leukemia ephosa.

I-MDS Ithuthukisa kanjani?

I-MDS iqala ukulimala kwe-DNA noma ukuguqulwa kwesinye segazi esisodwa (hematopoietic) stem cell . Ngenxa yalomonakalo, umnkantsha wamathambo uqala ukwandisa amangqamuzana egazi futhi ugcwala ngamaseli angashadile noma "aqhuma".

Ku-MDS, kukhona nokwanda kokufa kwe-cell program (i-apoptosis), okuholela ekuphazamiseni okuthakazelisayo. Ngenkathi kungase kube nokwanda kokukhiqizwa kwamaseli emnkantso, abahlali isikhathi eside ngokwanele ukuze bakhululwe ngaphandle kwegazi. Ngakho-ke, abantu abane-MDS bavame ukuhlushwa yi- anemia (isibalo esincane esibomvu segazi,) i- thrombocytopenia (isibalo esiphansi seplatelet), ne- neutropenia (isibalo esincane segazi elimhlophe.)

Izingozi Zezingozi

Ayaziwa ukuthi yini ebangelwa ukuguqulwa kwezinto ezidala izidakamizwa ze-myelodysplastic, kanti i-90% yesikhathi asikho isizathu esizwakalayo sesifo.

Ezinye izinto ezikhona ezingengozini ezihambisana nokukhula zifaka:

Ingabe I-Pre Leukemia?

Ukulinganisa kwenani lokuqhuma kwamangqamuzana emnkantsheni kubonisa indlela lesi sifo esibi ngayo-amangqamuzana angakhula, kakhulu kakhulu. Uma umnkantsha wakho ubonisa ukuthi inani lalo lenziwe ngamangqamuzana angaba ngu-20% amangqamuzana, isimo sibhekwa njenge-AML.

Amacala angu-30% we-MDS athuthuka ku-AML. Noma kunjalo, kubalulekile ukuqaphela ukuthi noma ngabe lolu shintsho lungaze lwenzeke, i-anemia, thrombocytopenia, ne-neutropenia ehambisana ne-MDS isengcupheni yokuphila.

Ama-subtypes

Ukuthola ukuxilongwa kwe-MDS akuhlanganisi nje kuphela ukuphazamiseka okwehlukene kwamathambo eminwe, kunezici eziningana ngaphakathi kwalowo nalowo mimoya onquma ukuziphatha nokubikezelwa kwesifo. Ngenxa yalokho, ososayensi baye balwela ukuthola uhlelo lokuhlukanisa olucabangela zonke lezi ziguquko ezihlukene.

Okokuqala kulezi zinhlelo yizigaba zaseFrance- American-British (FAB). Iphula i-MDS phansi kuma-subtypes ama-5 ngokususelwa ekutheni umthambo wethambo ubheka kanjani nemiphumela yesibalo segazi esigcwele (CBC) :

Kusukela ekuthuthukiseni izinga le-FAB ngo-1982, ososayensi baye bafunda kabanzi mayelana nokungahleleki kwezakhi zofuzo eziholela ku-MDS kanye nendima lapho lezi zinguquko zidlala phakathi nesifo. Ngenxa yalokho, ngo-2001, i-World Health Organization (WHO) yanyathelisa izinguquko ezithile ohlelweni lwe-FAB. Banezela izimo ezithile - i-5q- syndrome, i-MDS engahlanhlisi (MDS-U), ne-cytopenia ekhanyelayo nge-multilineage dysplasia (i-RCMD) - futhi yabelana nabanye njengo-RAEB no-CMML ngokususelwa kumaphesenti okuqhuma emnothweni wethambo.

Baphinde bacacise ukuthi noma yikuphi okukhulu kunamaphesenti angama-20 okuphefumula emlonyeni okwakhiwa i-AML, okwenza i-RAEB-T i-leukemia iphikisana ne-MDS.

Indlela yesithathu yokuhlukanisa i-MDS isebenzisa uhlelo lwe-International Prognostic Scoring System (IPSS). Le nqubo isebenzisa izindlela ezintathu zokunquma ukuthi i-MDS izoqhubeka kanjani: inani lamaseli egazini elijikelezayo legazi, inani lama-immature aqhuma amangqamuzana emnothweni we-bone, kanye ne-cytogenetics (uhlobo lwezinto ezingezansi zofuzo ezihambisana ne-MDS).

Ngokusekelwe kulezi zici, i-IPSS ihlukanisa iziguli zibe yizigaba ezine ezibonisa "ingozi" ye-MDS-low, ephakathi-1, ephakathi-2, nangaphezulu. I-IPSS inikeza indlela engcono yokubikezela imiphumela ye-MDS, unqume ukubikezela, futhi uhlele ukwelashwa.

I-MDS eyinhloko kanye neyesibili

Eziguli eziningi, i-MDS ibonakala ihlakulela isizathu esingaziwayo, ngaphandle kwe-blue. Lokhu kubizwa ngokuthi MDS eyinhloko noma ye-novovo. Njengokwengqondo ye-leukemia kanye nezinye izifo zethambo lomnkantsha, ososayensi abaqiniseki kahle ukuthi yiziphi izimbangela ze MDS.

I-MDS yesibili ibhekisela esimweni lapho ilandela ukwelashwa kwangaphambilini nge-chemotherapy noma ukwelashwa kwamafutha.

Ukuxilongwa

I-MDS itholakale isebenzisa amasu afanayo asetshenziselwa ukuxilonga i-leukemia .

Isinyathelo sokuqala ukuhlola igazi legazi lesiguli ngokubala okuphelele kwegazi (i-CBC). Lokhu kuhlolwa kubheka inani lamaseli egazi abomvu aphile, amangqamuzana egazi amhlophe, namaplatelet egazini ukuze uthole umqondo jikelele walokho okwenzeka emorheni. Ezimweni eziningi, umuntu one-MDS uzobonisa izinombolo eziphansi zamaseli abomvu obomvu (i-anemia), mhlawumbe namaplatelet aphansi (thrombocytopenia) kanye neutrophils (neutropenia) kanye.

Uma kungekho esinye isizathu esitholakala ukuthi isiguli sibe ne-anemia, odokotela bazobe benza i- aspirate yamathambo kanye ne-biopsy . Esigulini se-MDS, umnkantsha uzobonisa ukubukeka okungavamile kanye nenani elikhulayo lamaseli amancane noma "aqhuma". Lapho amangqamuzana ehlolwa ezingeni lezakhi zofuzo, azobonisa ukuguqulwa komzimba noma izinguquko kuma-chromosomes.

Izimpawu Nezibonakaliso

Iziguli ezine-MDS zingase zithole izimpawu ze-anemia ezifana nalezi:

Iziguli ezimbalwa zizobe nezibonakaliso ze-neutropenia ne-thrombocytopenia, kuhlanganise nezinkinga zokuphuma kwegazi kanye nobunzima ukulwa nezifo.

Kubalulekile ukuqaphela ukuthi kunezinye izimo eziningi ezingathí sina ezingabangela lezi zimpawu nezimpawu. Uma ukhathazekile nganoma yikuphi ukukhathazeka kwezempilo ozizwayo, kuhle ngaso sonke isikhathi ukuxoxa nabo nodokotela wakho noma ochwepheshe bezokwelapha.

Ukuyifinyelela

I-MDS ayiyona isifo esisodwa, kunalokho iqoqo lezimiso ezibangela ushintsho endleleni umthambo wethambo usebenza ngayo.

Njengoba isayensi ifunda kabanzi mayelana nezakhi zofuzo kanye nendima abayidlala ekuthuthukiseni lezi zinhlobo zezifo, sifunda nokunye okwengeziwe ngezici ezithatha inkambo azoyithatha kanye nemiphumela engenzeka. Esikhathini esizayo, abacwaningi bazokwazi ukusebenzisa lolu lwazi ukudala ukwelashwa okusha nokusebenza okunamandla kwe-MDS.

Imithombo:

Goldberg, S., Chen, E., Corral, M., et al. "Ukukhubazeka Nezifo Zomtholampilo Zama-Myelodysplastic Syndromes Phakathi kwe-United States I-Medicare Beneficiaries" Journal of Clinical Oncology June 2010. 28: 2847-2852.

Bowen, D. "Ukuphathwa kweziguli ezineMyelodysplastic Syndromes: Imiqondo Eyisingeniso" kuDeeg, H., Bowen, D., Gore, S., Haferlach, T, Beau, M, Niemeyer, C. (eds) (2006) ) I-Hematologic Izidakamizwa: I-Myelodysplastic Syndromes. Springer: eNew York. (k. 89-94).

U-Haferlach, T., Kern, W. "Ukwahlukaniswa nokufakwa kwesiteji se-Myelodysplastic Syndromes" kuDeeg, H., Bowen, D., Gore, S., Haferlach, T, Beau, M, Niemeyer, C. (eds) (2006) I-Hematologic I-Malignancies: I-Myelodysplastic Syndromes. Springer: eNew York. (pp. 40- 51).

I-National Cancer Institute. I-PDQ ye-Cancer Summaries Information. Ukwelashwa kwe-Myelodysplastic Syndromes. I-Health Professional Version. 04/02/15. http://www.ncbi.nlm.nih.gov/books/NBK66015/#CDR0000062929__1

I-Nimer, S. "I-Myelodysplastic Syndromes" Igazi ngoMeyi 2008. 111: 4841- 4851.

UScott, B., Deeg, J. "I-Myelodysplastic Syndromes" Ukubuyekezwa Kwaminyaka Yonke Yezokwelapha 2010. 61: 345-358.