Myelofibrosis yapakati (PMF) ndi imodzi mwa mavuto ambiri a magazi omwe amachitidwa ngati myeloproliferative neoplasms. Neoplasm imatchulidwa ngati kukula kwachibadwa kwa minofu yomwe imayambitsa kusintha kwa thupi ndipo ikhoza kusankhidwa kukhala yowonongeka (yosakhala yoipa), isanafike poipa, kapena yoipa. Matenda a myeloproliferative poyamba amachititsa manyazi, koma m'kupita kwanthaŵi angasanduke matenda oopsa (khansa).
Kusintha kwa PMF kumachititsa fibrosis (scarring) ya mafupa. Izi zomwe zimapezeka m'mafupa zimasokoneza kukula kwa maselo a magazi. Matenda a m'magazi ndi omwe amapeza kafukufuku wambiri. Leukocytosis (kukwera mu maselo oyera a magazi) ndi thrombocytosis (kukwera kwa mapepala apamwamba) ndizofala koma ngati matendawa akufalikira, thrombocytopenia (chiwerengero chochepa cha platelet) chikhoza kuchitika. Splenomegaly (kutambasula mu mphala) imakula pamene nthendayo imakhala malo achiwiri opanga maselo a magazi.
Kodi Aliyense Amafunikira Chithandizo?
Ngakhale njira yanu yoyamba ingakhale yowunika njira zomwe mungathe kuchita, kumbukirani kuti sikuti anthu onse omwe ali ndi PMF amafuna chithandizo. Kuchiza kwa PMF kumatsimikiziridwa ndi chiopsezo cha matenda opitirira komanso kupulumuka kwapadera.
Chipangizo chotchedwa Dynamic International Prognostic Scoring System (DIPSS) Plus chiwerengero chimagwiritsa ntchito chidziwitso chokhudza munthu ngati zaka, chiwerengero cha maselo oyera a magazi, hemoglobin, kuthamanga kwa maselo, kukhalapo kwa zizindikiro, majini, chiwerengero cha platelet, ndi kuikidwa magazi kuti awerenge mapepala.
Kugwiritsira ntchito dongosolo lino la anthu ndi PMF kungagawidwe m'magulu anayi owonetsera: chiwopsezo chochepa, chiwopsezo-choyamba, chiwopsezo chachiwiri, komanso chiopsezo chachikulu. Kupulumuka kwapakati pa chaka chimodzi kwa odwala omwe ali ndi chiopsezo chachikulu kwa zaka 15 kwa odwala omwe ali ndi chiopsezo chachikulu. PMF mwa anthu osapitirira zaka makumi asanu ndi limodzi (60) akugwirizana ndi kupuma kwabwino komanso kupulumuka kwapakati pa zaka ziwiri mpaka 20.
Mankhwala a Hematologists amagwiritsa ntchito chiwerengero cha DIPPS Plus pamodzi ndi kusintha kwa chibadwa cha munthu kuti apeze dongosolo la mankhwala. Anthu omwe ali ndi chiopsezo chachikulu omwe alibe zizindikiro samachiritsidwa koma amayang'anitsitsa mosamala za zizindikiro ndi kuwonongeka kwa magazi m'thupi komanso / kapena thrombocytopenia. Ngati munthu akukulitsa zizindikiro (malungo, kutaya thupi, kupweteka kwambiri kapena kutambasula kwa phala) kapena kufunika koika magazi, mankhwala ayenera kuyamba. Amagazi opatsirana ofiira magazi amaperekedwa nthawi zambiri pamene hemoglobini ili osakwana 8 g / dL. Chifukwa chakuti magazi ofiira m'magazi ambiri amachititsa kuti chitsulo chiwonjezere, nthawi zambiri mankhwala ena amayesedwa.
Kuchiza kwa Zizindikiro
- Splenomegaly: Ngati nthendayo ikukula kwambiri ndipo ikuyambitsa mavuto (monga kuvulaza, kuchuluka kwa splenic, kuwonjezera kuika magazi), mankhwala ovomerezeka a hydroxyurea angagwiritsidwe ntchito. Ndi mankhwalawa okhudza 40 peresenti ya anthu omwe ali ndi PMF ali ndi 50 peresenti yochepetsetsa kukula kwa nthata kwa pafupifupi chaka chimodzi. Ngati nthendayo isayankhe mankhwala a hydroxyurea, splenectomy (kuchotsa opaleshoni ya mphala) ingafunike.
- Kuchepa kwa magazi m'thupi: Kutaya magazi m'thupi mwa PMF kungapangidwe ndi mankhwala osiyanasiyana monga fluoxymesterone, prednisone, kapena danazol. Fluoxymesterone ndi danazol amadziwika kuti androgens (hormone ya steroid) yomwe imawoneka kuti imayambitsa kupanga mafuta a mafupa. Imodzi mwazikuluzikulu za mankhwalawa ndikuti zimagwirizana ndi mahomoni amphongo ndipo zingayambitse ubweya wa thupi, mau ozama, kapena kuchuluka kwa minofu. Thalidomide kapena lenalidomide (mtundu wa chemotherapy) komanso prednisone angagwiritsenso ntchito.
Pangozi Yaikuru Kapena Yapakati
Anthu omwe ali ndi matenda apakati komanso omwe amawopsa kwambiri angafune thandizo linalake. Ndizomveka kumvetsa kuti matenda anu ndi apamwamba kwambiri-chidziwitso cha chithandizo cha mankhwala chingathandize kuchepetsa nkhawa ndi mantha omwe mungamve.
- Kachilombo ka maselo a Hematopoietic (HSCT kapena kupaka mafupa a mafupa) : Awa ndiwo njira yokhayo yothandizira PMF koma ili ndi chiopsezo chachikulu. Kuwedzeretsa kuyenera kuchitika posachedwa atagonjetsedwa musanamangidwe ndi mavuto ena kuti athe kuchepetsa mavuto. M'mbuyomu, anthu omwe ali ndi zaka zosachepera 60 omwe sali ndi zaka makumi asanu ndi awiri ( 60) omwe amafanana ndi omwe amapereka chithandizo (MSD) . Zosintha zatsopano zakhala zikuchitidwa pofananirana ndi othandizira othandizana kapena osagwirizana.
- Ruxolitinib: Anthu omwe ali ndi PMF ndi zizindikiro zazikulu omwe sali ovomerezeka ku HSCT akhoza kugwiritsa ntchito ruxolitinib. Ruxolitinib ndi mankhwala otchedwa tyrosine kinase inhibitor, makamaka JAK2 inhibitor. JAK2 ndi chizoloŵezi chofala pakati pa PMF koma amapezekanso m'mayendedwe ena a myleoproliferative monga polycythemia vera ndi vuto lofunika kwambiri la thrombocythemia. Chithandizo ndi ruxolitinib chikhoza kuchepetsa kukula kwa nthata, kuchepetsa zizindikiro (monga kutopa, kupweteka kwa mafupa), ndi kuchepetsa kuchepa kwa magazi. Ngakhale mankhwalawa akuwongolera kusintha kwa JAK2, odwala omwe ali ndi kusintha kwina angayankhe.
Zotsatira:
Teferri A. Matenda a Myelofibrosis Oyambirira ndi Oyang'anira Myelofibrosis Oyambirira. Mu: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2016.