Ukufingqiwe Okufushane Kokuphathwa KwePolycythemia Vera
I-Polycythemia vera , i-neoplasm, i-myeloproliferative neoplasm, ephumela ekuguquleni kwezakhi zofuzo okuholela ekukhiqizweni kwamaseli amaningi abomvu amaningi (i- erythrocytosis ). I-white white cell ne-platelet izibalo zingase ziphakanyiswe. Lokhu kwanda inani lamangqamuzana egazi (ngaphandle kokwanda kwesabelo segazi) kwandisa ingozi yokuthuthukiswa kwe-clot.
Ukufunda ukuthi utholakale ukuthi i-polycythemia ingase ihlupheke futhi ngokwemvelo ungase uzibuze, "ingabe ingaphathwa kanjani?" Ngenhlanhla, ngokuphulukiswa, ukusinda kuyanda kusuka ezinyangeni eziyisithupha kuya kweziyi-18 kuya eminyakeni engu-13 noma ngaphezulu.
Enye yezinselelo zokwelapha i-polycythemia vera ukuthi abantu abangaphezu kuka-12 abantu abazoguqulwa babe yi- myelofibrosis futhi azungeze amaphesenti angu-7 bazoba ne-acute leukemia / myelodysplastic syndrome .
Ngokuphambene nabantu abaningi abane- thrombocythemia ebalulekile , abantu abane-polycythemia vera bavame ukuba nezibonakaliso ezinjengezinhloko zekhanda, isizungu, ukuchithwa ekuxilongweni. Lezi zimpawu ngokuvamile zicindezela futhi zidinga ukuqala ukwelashwa.
I-Phlebotomy ye-PV
Ukwelashwa okuyinhloko kwe-polycythemia vera yi- phlebotomy yokwelashwa . Ku-phlebotomy yokwelapha, igazi lisuswa emzimbeni ofana nomnikelo wegazi ukunciphisa inani lamaseli abomvu egazi. I-phlebotomy yezokwelapha iqhutshwa njalo ukuze igcine i-hematocrit (ukuhlushwa kwamaseli abomvu abomvu) ngaphansi kwenombolo ethile, ngokuvamile ngamaphesenti angu-45 amadoda namaphesenti angu-42 abesifazane. I-Phlebotomy ithatha i-polycythemia vera ngezindlela ezimbili: ngokunciphisa inani lamangqamuzana egazi ngokususa ngokomzimba emzimbeni futhi ngokubangela ukuntuleka okuvimbela ukukhiqizwa kwe-cell cell ebomvu.
I-Hydroxyurea ye-PV
Kubantu abanengozi enkulu yokuthuthukisa i-clot yegazi (engaphezu kweminyaka engama-60 yobudala, umlando we-clot blood), ukwelashwa okwengeziwe kuyadingeka. Ngokuvamile imithi yokuqala eyongezwa ekwenzeni i-phlebotomy yokwelashwa i-oral hydroxyurea.
I-Hydroxyurea yikomidi le-chemotherapeutic yomlomo elinciphisa ukukhiqizwa kwamaseli abomvu ebomvu emathambo.
Futhi kunciphisa ukukhiqizwa kwamangqamuzana amhlophe wegazi namaplatelet. Ngokufanayo nezinye izimo eziphathwe nge-hydroxyurea, iqala ngomthamo ophansi futhi yanda kuze kube yi-hematocrit ebangeni lomgomo.
Ngokuvamile kuvunyelwe kahle ngemiphumela emibi ngaphandle kokunciphisa ukukhiqizwa kwamaseli egazi. Ezinye imiphumela emibi zibandakanya izilonda zomlomo, ukuguqulwa komzimba (ukungcola okumnyama kwesikhumba), ukuqhuma, nezinguquko ze-nail (imigqa emnyama ngaphansi kwezipikili). I-aspirin ye-dose ephansi nayo ibhekwa njengendlela yokwelapha yangaphambili. I-aspirin isonakalisa ikhono lamaplatelet ukunamathela ndawonye ngaleyo ndlela ukunciphisa ukuthuthukiswa kwezindwangu zegazi. I-aspirin akufanele isetshenziswe uma uye waba nemlando yenkathi enkulu yokugaya.
I-Interferon Alpha ye-PV
Kubantu abanesisindo esingenakuqhathaniswa (okuthiwa i-pruritus), abesifazane abakhulelwe noma eminyakeni yokubeletha kwengane, noma ukuphikisana nezinqubo zangaphambili zokwelashwa, i-interferon alpha ingasetshenziswa. I-Interferon alpha yisifo esithinta isisu esinciphisa inani le-cell red. Kungabuye kunciphise usayizi we-spleen nokuluma. Imiphumela emibi ihlanganisa umkhuhlane, ubuthakathaka, isicashu, kanye nokuhlanza okuvimbela ukusizwa kwalo muthi.
Busulfan for PV
Iziguli eziye zahluleka i-hydroxyurea kanye / noma i-interferon alpha, busulfan, imithi ye-chemotherapeutic, ingasetshenziswa.
Umthamo ushintshwe ukugcina isibalo segazi elimhlophe nesibalo seplatelet ebangeni elamukelekayo.
I-Ruxolitinib ye-PV
Enye imithi esetshenziselwa iziguli ezahluleka ezinye izindlela zokwelapha i- ruxolitinib . Le mithi ivimbela i-enzyme yeJanus Associated Kinase (JAK) evame ukuthinteka kuma-polycythemia vera nakwezinye izimo ze-myeloproliferaive. Kuvame ukusetshenziswa kubantu abathuthukisa i-post-polycythemia vera myelofibrosis. Kungasiza futhi kubantu abanezimpawu ezinzima kanye ne-splenomegaly (i-pleen ekhulisiwe) ebangela ubuhlungu obukhulu noma ezinye izinkinga.
Khumbula, nakuba i-polycythemia vera yisimo esingapheli, isimo esaziwayo esilawulekayo futhi kungenzeka ukuhola izinga elihle lokuphila nalo.
Khuluma nodokotela wakho mayelana nezinketho ezahlukene zokwelashwa ezitholakalayo. Uzosikisela okuhle kakhulu kuwe.
Imithombo:
I-Teferri A. Ukuguqulwa nokuPhathwa kwePolycythemia Vera. Ku: Phezulu, I-post TW (Ed), UpToDate, Waltham, MA. (Kufinyelelwe ngoJuni 22, 2016.)