Primary myelofibrosis (PMF) imwe yezvimwe zvirwere zveropa zvakasarudzwa se myeloproliferative neoplasms. Neoplasm inotsanangurwa sekukura kusingawanzo kwechigunwe chinokonzerwa nekuchinja uye kunogona kusarudzwa sebenign (kwete-yakaipa), isati yanyangadza, kana yakaipa. Myeloproliferative neoplasms inowanzova nehupenzi pakutanga, asi nekufamba kwenguva inogona kuva chirwere chinouraya (kenza).
Kuchinja kwemuviri kweMDC kunokonzera fibrosis (tsvina) yemapfupa emapfupa. Uku kuvhara mumapfupa emapfupa kunovhiringidza kukura kwemasero eropa. Anemia ndiyo inonyanyozivikanwa nebazi rekuwana. Leukocytosis (kukwirira mumasero matsvuku eropa) uye thrombocytosis (yakakwirira platelet count) yakajairika asi sezvo chirwere chinopfuurira, thrombocytopenia (low platelet count) inogona kuitika. Splenomegaly (kuwedzera mu spleen) inokura sezvinonzi spleen inova nzvimbo yechipiri yeropa rekugadzira.
Munhu Wose Anoda Kurapwa Here?
Kunyange zvazvo danho rako rekutanga ringave ririkutsvaga nzira dzingasarudzwa nadzo, yeuka kuti havasi vanhu vose vane PMF vanoda kurapwa. Kurapa kwePMF kunotsanangurwa nehuwandu hwekuwedzera kwezvirwere uye kuponeswa kwepamusoro.
Nzira inonzi Dynamic International Prognostic Scoring System (DIPSS) Plus inoshandisa ruzivo pamusoro pemunhu akafanana nezera, nyeupa yeropa rekuverenga, hemoglobin, kupararira kwemhepo masero, kuvapo kwezviratidzo, genetics, platelet count, uye kuwedzerwa kunoda kuverenga mapeji.
Kushandisa iyi system nevanhu vane PMF inogona kugoverwa muzvikamu zvina zvekufananidzira: pangozi yakadzika, pakati-1 pangozi, pakati-2 panjodzi, uye pangozi yakanyanya. Kupona kwemaMedhia kunowanikwa gore rinopfuura rimwe chete kune varwere vane chirwere chikuru chepachirwere kusvika makore gumi nemashanu kune varwere vane chirwere chisingabatsiri chirwere. PMF kune vanhu vari pasi pezera remakore makumi matanhatu inosangana nehuwandu hwekutsvaga uye kuponeswa kwemuviri kwemakore anoda kusvika makore makumi maviri.
Hematologists vanoshandisa chikwata cheDIPPS Plus pamwe chete nemunhu kuchinja mutemo kuti aone chirongwa chekurapa. Vanhu vane chirwere chisingabatsiri vasina zviratidzo havarapi kurapwa asi vanoongororwa zvakanyanya nezvezviratidzo uye kuipa kwekushaya ropa uye / kana thrombocytopenia. Kana munhu achikura zviratidzo (fever, kurasikirwa, kupfutidza zvakanyanya kana kuwedzera kwepleen) kana kuwedzerwa ropa, chirwere chinofanira kutanga. Ropa rekuisira ropa masero inowanzopiwa apo hemoglobin iri pasi pe8 g / dL. Nokuti zvinowanzoitwa ropa tsvuku rekuisira ropa rinotungamirira kune simbi kuwandisa, kazhinji dzimwe mishonga dzinoedzwa.
Kurapa Kwezviratidzo
- Splenomegaly: Kana chirwere chacho chakanyanya kuwedzerwa uye chiri kukonzera nharo (dzakadai sokusawirirana, multiple splenic infarcts, kuwedzerwa kuwedzerwa ropa), mishonga yemuromo inonzi hydroxyurea inogona kushandiswa. Nekurapa uku kwema40 kubva muzana yevanhu vane PMF vane 50 muzana inoderedza kudarika kwepleen kunenge gore rimwechete. Kana chirwere chacho chisingapindi kune hydroxyurea therapy, splenectomy (kuporesa kubviswa kwepleen) kunogona kudiwa.
- Anemia: Anemia muPMF inogona kurapwa nemhando dzakasiyana dzemishonga yakadai se fluoxymesterone, prednisone, kana danazol. Fluoxymesterone uye danazol vanozivikanwa se androgens (steroid hormone) iyo inoita seyokusimudzira mapfupa ekugadzira marora. Rimwe rematambudziko makuru emishonga iyi ndeyokuti yakabatana nemhomoni yevarume uye inogona kukonzera kukura kwemvere dzemuviri, inzwi rakadzika, kana kuwedzerwa kwemasuru. Thalidomide kana lenalidomide (chimiro che chemotherapy) pamwe ne prednisone inogona kushandiswa zvakare.
Dambudziko Rakakwirira kana Rakapfupi
Vanhu vane chirwere chepakati uye chepamusoro-soro vangave vachida kurapwa kwakasiyana. Zvinonzwisisika zvakaoma kunzwa kuti chirwere chako chiripamusoro-panjodzi-ruzivo rwezvokurapa zvinogona kubatsira kuderedza zvimwe zvekutambudzika uye kutya iwe unogona kunzwa.
- Hematopoietic stem cell transplant (HSCT kana kutema marrow transplant) : Iyi ndiyo chete inorapa kuporesa kweMDC asi ine ngozi yakawanda. Kudyara kunofanira kuitika nguva pfupi mushure mekunyatsoongorora kusati kwatanga kufambiswa kwezvimwe zvinetso kuderedza zvinetso. Nhoroondo yekare, kupindira kwave kuri kune vanhu vasati vava nemakore makumi matanhatu avo vakafananidza vana vavanini vayo (MSD) . Munguva pfupi yapfuura kuchinja kwave kwakaitwa nekuenzaniswa kusina kufanirana kana kusanzwisisiki kwakabatana vanopa.
- Ruxolitinib: Vanhu vane PMF uye zviratidzo zvakakomba zvisina kufanirwa neHSCT vanogona kushandisa ruxolitinib. Ruxolitinib imishonga inozivikanwa se tyrosine kinase inhibitor, kunyanya JAK2 inhibitor. JAK2 inowanzochinja muPMF asi inogonawo kuwanikwa mune dzimwe myleoproliferative neoplasms yakafanana ne polycythemia vera uye inokosha yeprombocythemia. Kurapa neRuxolitinib kunogona kuderedza uswa hwepleen, kuderedza zviratidzo (sekuneta, kurwadziwa kwepfupa), uye kuderedza kucherechedza ropa. Kunyange zvazvo mishonga iyi inotarisa JAK2 mutation, varwere vane dzimwe shanduko vanogona kupindura zvakare.
Sources:
Teferri A. Kurapa kweMutambo Myelofibrosis uye Kutungamira Kwemusoro Myelofibrosis. Mu: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2016.