Chidule Chachidule cha Chithandizo cha Polycythemia Vera
Polycythemia vera , myeloproliferative neoplasm, imachokera ku kusintha kwa majini komwe kumayambitsa kupanga maselo ofiira ambiri a magazi ( erythrocytosis ). Selo loyera la magazi ndi chiwerengero cha mapaleti chikhoza kukwezedwa. Kuwonjezeka kwa chiwerengero cha maselo a magazi (popanda kuwonjezeka kwa gawo la magazi) kumapangitsa chiopsezo cha kukula kwa clot.
Kuphunzira kuti mwapezeka ndi polycythemia kungakhale kovuta ndipo mwachibadwa mungadabwe, "Kodi angachiritsidwe bwanji?" Mwamwayi, ndi chithandizo, kupulumuka kumawonjezeka kuchoka pa miyezi 6 mpaka 18 kufika 13 kapena kuposerapo.
Imodzi mwa zovuta pochiza polycythemia vera ndikuti anthu oposa 12 peresenti ya anthu adzasintha kukhala myelofibrosis ndipo pafupi 7 peresenti idzakhala ndi vuto lalikulu la leukemia / myelodysplastic syndrome .
Mosiyana ndi anthu ambiri omwe ali ndi vuto la thrombocythmia , anthu omwe ali ndi polycythemia vera amakhala ndi zizindikiro monga mutu, chizungulire, kupweteka kwa matenda. Zizindikirozi zimakhala zopweteka ndipo zimafuna kuyamba kuchipatala.
Phlebotomy kwa PV
Chithandizo chachikulu cha polycythemia vera ndi mankhwala ochizira phlebotomy . Pachiberekero la mankhwala, magazi amachotsedwa ku thupi lofanana ndi zopereka za magazi kuti kuchepetsa chiwerengero cha maselo ofiira a magazi. Mankhwala a phlebotomy amapitilira nthawi zonse kuti asamatengere magazi (maselo ofiira a m'magazi) pansi pa nambala inayake, kawirikawiri 45 peresenti ya amuna ndi 42 peresenti ya akazi. Phlebotomy imapanga polycythemia vera m'njira ziwiri: pochepetsa chiwerengero cha maselo a magazi mwa kuchotsa iwo m'thupi ndi kuchititsa kusowa komwe kumalepheretsa kupanga maselo ofiira atsopano a magazi.
Hydroxyurea kwa PV
Kwa anthu omwe ali ndi chiopsezo chachikulu chokhala ndi magazi (zaka zoposa 60, mbiri ya magazi), mankhwala ena amafunika. Kawirikawiri mankhwala oyamba omwe amaphatikizidwa ndi mankhwala a phlebotomy ndi oral hydroxyurea.
Hydroxyurea ndi mankhwala otchedwa chemotherapeutic agent omwe amachepetsa kupanga maselo ofiira m'magazi.
Zimathandizanso kuchepetsa maselo oyera ndi maselo. Mofanana ndi zinthu zina zomwe zimagwiritsidwa ntchito ndi hydroxyurea, zimayambira pa mlingo wochepa ndipo zinawonjezeka mpaka hematocrit ili mu zolinga zambiri.
Nthawi zambiri amalekerera ndi zotsatira zina zochepa kupatulapo kuchepa kwa maselo a magazi. Zotsatira zina zimaphatikizapo zilonda zakumlomo, hyperpigmentation (kutayika kwa khungu khungu), kuthamanga, ndi kusintha kwa msomali (mzere wandiweyani pansi pa misomali). Mankhwala otsika a aspirin amathandizidwanso ngati mankhwala otsogolera. Aspirin imasokoneza luso la mapuloletti kuti agwirane pothandizira kuchepetsa kukula kwa magazi. Aspirin sayenera kugwiritsidwa ntchito ngati mutakhala ndi mbiri ya nthawi yayikulu yakupha magazi.
Interferon Alpha ya PV
Kuyambira anthu omwe ali ndi kuyabwa kosayembekezereka (kutchedwa pruritus), amayi omwe ali ndi mimba kapena pamene akubereka ana, kapena akutsutsa njira zothandizira kale, angagwiritsidwe ntchito. Interferon alpha ndi matenda osokoneza thupi omwe amachepetsa kuchuluka kwa maselo ofiira a magazi. Ikhoza kuchepetsa kukula kwa nthata ndi kuyabwa. Zotsatirapo zimaphatikizapo kutentha thupi, kufooka, nseru, ndi kusanza komwe kumachepetsa phindu la mankhwalawa.
Busulfan kwa PV
Kwa odwala omwe alephera hydroxyurea ndi / kapena interferon alpha, busulfan, mankhwala a chemotherapeutic, angagwiritsidwe ntchito.
Mlingowo umasinthidwa kuti asunge selo loyera la magazi ndi chiwerengero cha mapiritsi mmalo ovomerezeka.
Ruxolitinib kwa PV
Mankhwala ena ogwiritsidwa ntchito kwa odwala omwe analephera mankhwala ena ndi ruxolitinib . Mankhwalawa amaletsa jekeseni wa Janus Associated Kinase (JAK) yomwe nthawi zambiri imakhudzidwa ndi polycythemia vera ndi myeloproliferaive neoplasms. KaƔirikaƔiri amagwiritsidwa ntchito kwa anthu omwe amapanga post-polycythemia vera myelofibrosis. Zingathandizenso anthu omwe ali ndi zizindikiro zoopsa komanso splenomegaly (mphulupulu) yomwe imapweteka kwambiri kapena zina.
Kumbukirani, ngakhale polycythemia vera ndi matenda aakulu, ndizodziwika bwino zomwe zingatheke kukhala ndi moyo wabwino.
Lankhulani ndi dokotala wanu za njira zosiyanasiyana zochiritsira zomwe zilipo. Iyeyo angakuuzeni zabwino kwambiri kwa inu.
Zotsatira:
Teferri A. Matenda ndi Chithandizo cha Polycythemia Vera. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Idapezeka pa June 22, 2016.)