Inkcazo emfutshane malunga nokunyangwa kwePolycythemia Vera
I-Polycythemia vera , i-myeloproliferative neoplasm, ibangelwa ukuguquka kwemfuyo ekhokelela kwimveliso yamanzi amaninzi obomvu (i- erythrocytosis ). Isalathisi segazi esimhlophe kunye nokubala kweplatelet nako kunokuphakanyiswa. Olu konyuka kwinani lamaseli egazi (ngaphandle kokunyuka kwincenye yamanzi egazini) landisa ingozi yokuphuhlisa i-clot.
Ukufunda ukuba ufunyenwe ukuba i-polycythemia inokubandezeleka kwaye ngokwemvelo ungase uzibuze, "unokunyangwa njani?" Ngethamsanqa, kunyango, ukunyuka kwenyuka ukusuka ezintandathu ukuya kwezi-18 ukuya kwi-13 iminyaka okanye ngaphezulu.
Enye yemingeni ekwelapheni i-polycythemia vera kukuba abantu abangaphezu kwe-12 abantu baya kutshintshwa babe yi- myelofibrosis kunye neepesenti ezingama-7 ziya kuba ne-acute leukemia / myelodysplastic syndrome .
Ngokuchasene nabantu abaninzi abane- thrombocythemia ebalulekileyo , abantu abane-polycythemia vera bavame ukuba neempawu ezinjengeentloko, isisu, ukungcola ukuxilongwa. Ezi zimpawu zixakeka kwaye zifuna ukuqaliswa unyango.
Phlebotomy yePV
Unyango oluphambili lwe-polycythemia vera luyi- phlebotomy yonyango . Kwi-phlebotomy yokwelapha, igazi lisuswe emzimbeni ofana nomnikelo wegazi ukunciphisa inani leeseli ezibomvu zegazi. I-phlebotomy yonyango iyaqhubeka rhoqo ukuba igcine i-hematocrit (ingxube yeeseli ezibomvu zegazi) ngaphantsi kwenani elithile, ngokuqhelekileyo ipesenti ezingama-45 kumadoda kunye nama-42 ekhulwini kwabafazi. I-Phlebotomy ithatha i-polycythemia vera ngeendlela ezimbini: ngokunciphisa inani lamaseli egazi ngokuzikhupha ngokusemzimbeni kwaye ngokubangela ukusilela okumisela ukuveliswa kweefom entsha yegazi.
Hydroxyurea yePV
Kubantu abanomngcipheko ophezulu wokuphuhlisa i-clot yegazi (ngaphezu kweminyaka engama-60 ubudala, imbali yegazi le-clot), kufuneka unyango olongezelelweyo. Ngokuqhelekileyo iyeza lokuqala elongezelelweyo kwi-phlebotomy yonyango ngumlomo we-hydroxyurea.
I-Hydroxyurea yi-agent engumlomo we-chemotherapeutic eyanciphisa ukuveliswa kweeseli ezibomvu zegazi kwindawo yomongo.
Kwakhona kunciphisa ukuveliswa kweeseli ezimhlophe zegazi kunye neeplatelet. Kuyafana nezinye iimeko eziphathwe nge-hydroxyurea, iqaliswa kwiqondo eliphantsi kwaye linyuka de i-hematocrit iluhlu lweenjongo.
Ngokuqhelekileyo ukunyamezela kakuhle kunye nemiphumo emibini ngaphandle kokunciphisa ukuveliswa kwamaseli egazi. Ezinye iziphumo ezichaphazelekayo zibandakanya izilonda zomlomo, ukuchithwa kwe-hyperpigmentation (ukuguqulwa kwamnyama kwesikhumba), ukugqithisa, kunye nokutshintsha kwe-nail (imigca emnyama phantsi kwepiki). I-dose ephantsi ye-aspirin ikwaqwalaselwa kwakhona njengonyango lokuqala. I-Aspirin iyonakalisa amandla okwenziwa kweeplatelet ukuba zihlangane kunye nokunciphisa ukuphuhliswa kwamacandelo egazi. I-Aspirin ayifanele isetyenziswe ukuba unayo imbali yecala elikhupha igazi.
I-Interferon Alpha yePV
Kubantu abanokubethwa okungenakulibala (okubizwa ngokuba yi-pruritus), abafazi abakhulelweyo okanye kwiminyaka yokuzala abantwana, okanye ukukhanyela kwiipilisi zangaphambili, i-intelon alpha ingasetyenziswa. I-intelon ye-alpha yintsholongwane engaphantsi komzimba eyanciphisa inani le-cell red. Inokunciphisa ubukhulu bepen kunye nokubamba. Imiphumo emibi ifaka i-fever, ubuthathaka, isisongela, kunye nokuhlanza okubangela ukuba luncedo lonyango.
Busulfan kwiPV
Izigulane eziye zahluleka i-hydroxyurea kunye / okanye i-interferon alpha, busulfan, imithi ye-chemotherapeutic, ingasetyenziswa.
I dose ishintshiweyo ukugcina inani lamaseli ezimhlophe kunye ne-platelet count kwibala elimkelekileyo.
Ruxolitinib yePV
Olunye unyango olwenzelwe izigulane olwehlulekile ezinye iindlela zokwelapha yi- ruxolitinib . Eli nyanga livimbela i-enzyme yeJanus Associated Kinase (JAK) edlalwa rhoqo kwi polycythemia vera kunye nezinye i-myeloproliferaive neoplasms. Ngokuqhelekileyo kusetyenziswa abantu abaphuhlisa i-post-polycythemia vera myelofibrosis. Kungaba luncedo kubantu abaneempawu ezinzima kunye ne-splenomegaly (ipenti ekhulisiweyo) ebangela intlungu ebalulekileyo okanye neminye imiba.
Khumbula, nangona i-polycythemia vera yimeko engapheliyo, yimeko eyaziwayo ekwazi ukukwazi ukuqhuba umgangatho wobomi kunye nayo.
Thetha nogqirha wakho malunga neendlela ezahlukeneyo zonyango ezikhoyo. Uya kuphakamisa okulungileyo kuwe.
Imithombo:
I-Teferri A. Iprognosis kunye noPyrothemia Vera. Ku: U-UpToDate, i-Post TW (Ed), UpToDate, i-Waltham, MA. (Kufumaneka ngoJuni 22, 2016.)