Chirongwa cheTrans for Essential Thrombocythemia
Inokosha Thrombocythemia (ET) ndeimwe yeboka rematambudziko eropa anonzi myeloproliferative neoplasms. Mimwe mhando ye myeloproliferative neoplasms inosanganisira primary myelofibrosis uye polycythemia vera .
Kuchinja kwemasvikiro muhutano hunokosha hunoita kuti pave nepamusoro peplatelet zvinyorwa (thrombocytosis). Vanhu vazhinji vasina zviratidzo panguva yekuongorora uye vachava netariro yeupenyu hwemazuva ose.
Zvinetso zvinosanganisira kuvhara mazamu (zvichiita kuti urove, kurwadziwa kwepfungwa, chivharo mumapapu, kana mumvura yakadzika inopisa), kukura kwemyelofibrosis , kana kushanduka kusvika kune myeloid yeukemia (AML) yakaoma. Chimwe chinetso chisingawanzwisisiki chechirwere chinokonzerwa nehutachiona ndechekusimbiswa kwekuwana von Willebrand Disease , chirwere chekubuda ropa.
Ruzhinji rwevanhu ruchada kurapwa pane imwe nguva kunodzivirira matambudziko. Zvisinei, kutarisira hupenyu hwemazuva ose kunowanzoshamisa.
Treatment Options
Kana iwe ukaonekwa kuti une hutachiwana hunokosha mubvunzo wekupedzisira wekuti, "Ndezvipi zvandinoda kurapa?" Pane zvimwe zvingasarudzwa izvo chiremba wako anofanira kukurukura newe. Mushonga wako chiremba anokurudzira unogona kunge wakavakirwa pane zvimwe zvinhu: zviratidzo zvako, zera rako, uye ma laboratory yako. Vanhu vazhinji vachaenda makore akawanda vasingadi kurapwa, izvo zvinowanzotanga kana iwe ukagadzira jira.
- Aspirin: Zuva rezuva rega-dose aspirin inowanzoshandiswa kurapa vanhu vane zviratidzo zvevasomotor. Vasomotor zviratidzo zvinosanganisira misoro, chiziva, kurwadziwa kwepfupa, kupisa kwemaoko uye tsoka, uye kuchinja kunoonekwa. Hazvisi zvisinganzwisisiki kuti aspirin therapy inoshandiswa nemamwe marapirwo. High dose aspirin therapy yakabatanidzwa nehuwandu hwekubuda ropa uye hahukurudzirwi.
- Hydroxyurea: Inonzi chemotherapy agent hydroxyurea inoderedza ngozi yeprombosi mune zvakakosha pakuchengetedzwa kwepasi kuburikidza nekuderedza platelet count. Hydroxyurea inotanga pamwero wakaderera uye yakawedzera kusvikira platelet yakawanda iri pedyo nekusingaperi (100,000 - 400,000 masero per microliter). Hydroxyurea inowanzochengetedzwa zvakanaka pasina zvinokonzerwa nemigumisiro. Zvimwe zvinosanganisa zvinosanganisira hyperpigmentation (darkening) yeganda, kushandiswa kwembambo (misoro yakawanda iri pasi pechipikiri), kuputika kweganda, nemaronda emuromo. Hydroxyurea inoderedzawo kuchena kwemajeri eropa (zvikurukuru neutrophil count) uye hemoglobin inoda kuongororwa zvakakwana nehuwandu hwehuwandu hweropa (CBC).
- Anagrelide: Anagrelide mishonga inonyorera iyo inowedzera pasi peplatelet nekudzivisa kugadzirwa kweplatelet mumapfupa emapfupa. Inowanzoshandiswa apo hydroxyurea isiri iyo inoshanda. Saizvozvowo ne hydroxyurea, anagrelide inotanga pamwero wakaderera uye yakawedzera kusvika pane zvaidiwa. Zvimwe zvinosanganisa zvinosanganisira misoro, palpitations (nokukurumidza mwoyo yakakura), kuchengetedza kwemvura, uye kubuda. Iyi migumisiro yemigumisiro inowanzogadzirisa nekuenderera mberi kwemishonga
- Alpha interferon: Alpha interferon inogona kushandiswa kutarisa platelet inowanikwa muhutano hunokosha, polycythemia vera, uye primary myelofibrosis. Inogonawo kuderedza ukuru hwepleen. Alpha interferon inoshandiswa pasi pechitunha (pasi peganda) zuva rega rega. Alpha interferon inowanikwawo muhutomu hwemazuva akawanda hunonzi pegylated interferon inogona kupiwa kamwe kamwe pavhiki. Interferon therapy inowanzochengeterwa vakadzi vane pamuviri kana vanhu vasingateereri hydroxyurea therapy.
- Plateletpheresis: Plateletpheresis inzira inobvisa maplatelets kubva pakuparadzirwa. Ropa rinobviswa kuburikidza ne-intravenous (IV) catheter uye inounganidzwa mumushandisi uko maplatelet akaparadzaniswa kubva kune dzimwe nzvimbo dzeropa (masero eropa dema, masero matsvuku eropa, uye plasma). Ropa, kuderedza maplatelet, rinodzorerwa kumuviri kuburikidza ne IV kana nesarine solution kana plasma. Plateletpheresis yakachengeterwa vanhu vane marefu eplatelet anoverengeka, maitiro akaoma maziso (mumapapu, brain), kana kubuda ropa. Mhedziso ye plateletheresis ndeyechinguva saka imwe kurapwa inofanira kutanga yakatangwa kwenguva refu yekudzivirira kwe platelet count.
Zvose zvinorapwa zvakataurwa pamusoro apa hazvirape hutachiwana hunokosha. Zvinongopedzisira zvishoma nezvishoma maplatelet count zvichideredza dambudziko rekugadzira jira kana kuti yakawanikwa naW vonbrand disease. Panguva ino, hapana chirwere chinoporesa chinonyanya kukosha. Nenzira yakanaka, pasinei nekushayikwa kwechirwere chekurapa, vazhinji vevanhu vane hutachiwana hunokosha vanogona kurarama hupenyu hwakakwana hune zvibereko.
Sources:
Teferri A. Kurapa uye Kurapa kweEntential Thrombocythemia. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Yakasvika musi waJune 15, 2016)