Matenda a Hematopoietic Shemps ndi Myelofibrosis Oyambirira

Ndondomeko ya Kuphatikizidwa Kwadongosolo kwa Cell Myelofibrosis

Kusankha mankhwala abwino a myelofibrosis oyambirira kungakhale kovuta. Kawirikawiri palibe chisankho chimodzi chokha. Pali mankhwala angapo a myelofibrosis oyambirira, koma mankhwala okhaokha omwe amachititsa kuti thupi likhale lopanda mafupa.

Nchifukwa chiyani aliyense samakolola?

Chithandizo chimamveka chodabwitsa, kotero inu mukhoza kudabwa chifukwa chomwe aliyense yemwe ali ndi myelofibrosis oyambirira salandira.

Zonsezi ndizoopsa komanso zopindulitsa.

Chithandizo cha myelofibrosis choyamba chimayambitsa chiopsezo chotengera zizindikiro zomwe mukukumana nazo. Ngati mulibe zizindikiro kapena muli ndi zizindikiro zomwe muli ndi chiopsezo chotsika, matendawa atha kukhala ndi zaka 15.4. Pachifukwa ichi, mavuto omwe angakhalepo (oopsa kapena osaphatikizapo omwe amawagwiritsira ntchito matenda ndi imfa) okhudzana ndi kuika pamtengo amaposa phindu la mankhwala. Kafukufuku wasonyeza kuti anthu asanu omwe ali ndi chiopsezo chotsika kapena chapakatikati-1 amakhala ocheperapo ngati atakhala ndi mankhwala opatsirana pogwiritsa ntchito mankhwala osokoneza bongo. Komabe, m'thupi lachiwiri-komanso lachiopsezo chachikulu, anthu asanu omwe adalandira pulogalamuyi anawoneka bwino kwambiri poyerekeza ndi omwe sanatero.

Ngati muli ndi myelofibrosis, yomwe imayambira pachimake, imakhala yosafunika kwambiri (HSCT). Chifukwa chakuti okalamba amachulukitsa chiopsezo chokumana ndi matenda opatsirana (makamaka kuwirikiza kawiri mpaka kumtengowo ), HSCT yakhala ikusungidwa kwa anthu omwe ali ndi myelofibrosis oyambirira osadutsa zaka 60.

Popeza zaka zapakatikati za anthu omwe ali ndi myelofibrosis oyambirira ali ndi chiwerengero cha 67, izi zimachepetsa chiwerengero cha anthu omwe ali ndi myelofibrosis omwe ali oyenerera kutero. Kuonjezerapo, wopereka wokondedwa wa HSCT ndi wofanana naye. Mchimwene wathunthu (amayi omwe ndi abambo omwe ali olandiridwawo) ali ndi mwayi umodzi wokhala nawo masewera, kuchepetsa chiwerengero cha anthu omwe angathe kulandira mankhwalawa.

Kutsegula Kwadongosolo Kwambiri

Pakalipano, 40 mpaka 60 peresenti ya anthu omwe ali ndi myelofibrosis oyambirira omwe amapitilira kubzala amakhala zaka zitatu kapena zinayi. Pali umboni wina wotsimikizira kuti kupyolera kwa splenectomy (kuchotsa opaleshoni ya ntchentche) isanayambe kusindikizidwa kumagwirizanitsidwa ndi chiwerengero chabwino cha kupulumuka makamaka mwa amuna, koma chifukwa chake sichimvetsetsedwa bwino. Kuwonjezera apo, anthu amene amayamba myelofibrosis pambuyo poti polycythemia vera kapena thrombocythemia yofunikira amawoneka kuti amakhala ndi moyo wabwino kwambiri pambuyo pa kusamalidwa kuposa anthu omwe ali ndi myelofibrosis.

Ndani Sali Wokwanira?

Anthu ena omwe ali ndi chiopsezo chachikulu sali ovomerezeka ku HSCT. Izi makamaka zimakhazikitsidwa ndi mwayi wopulumuka pambuyo pa HSCT.

Zinthu monga mphulupulu zazikulu (masentimita asanu ndi atatu pansi pa nthiti) ndi kuika magazi oposa 20 musanayambe kusamba (makamaka ngati muli ndi zonsezi) zakhala zikugwiritsidwa ntchito ndi zaka zisanu zochepa mutapuma.

Bwanji Ngati Simuli Woyenerera?

Ndiye ndi chiyani chomwe chingalimbikitsidwe ngati muli ndi myelofibrosis yapamwamba yoopsa koma simukugwirizana ndi HSCT kapena mulibe wopereka? Ngati muli ndi zizindikiro monga kupweteka kwa phala, kutopa, kupweteka kwa mafupa, kutuluka usiku, ndi zina zotero, ruxolitinib ikhoza kukhala mankhwala othandiza.

Ruxolitinib yasonyezedwa kuti ichepetse zizindikiro, kuchepetsa kukula kwa spleen, ndi kuchepetsa kuchepa kwa magazi m'thupi mwa anthu omwe ali ndi myelofibrosis oyambirira. Njira ina yopita ku HSCT ikhoza kukhala kulembedwa pa mayesero a zachipatala. Izi zikhoza kukupatsani mwayi wopeza mankhwala omwe akuphunziridwa ngati mankhwala othandiza kuti myelofibosis yoyamba ifike patsogolo.

Sikophweka kuphunzira kuti simukuyenera kuchiritsidwa, koma kumbukirani kufufuza zonse zomwe mungasankhe.

Tsogolo la Kutembenuka Monga Chithandizo

Pomwe pali chithandizo cha mankhwala ozunguliridwa, monga regimen yomwe imagwiritsidwa ntchito poyambitsa mafupa musanayambe kusamba ndi mankhwala omwe amalepheretsa kufalitsa matenda, zimakhala zikugwiritsidwa ntchito kwa anthu oposa zaka makumi asanu ndi limodzi (60) ogwirizana kapena ogwirizana osapereka osagwirizana).

Malo ena amapangitsa anthu kukhala ndi myelofibrosis mpaka zaka 75.

Tikuyembekeza, ndi kupitabe patsogolo, HSCT idzapezeka kwa anthu ambiri omwe ali ndi myelofibrosis. Chifukwa myelofibrosis yoyamba ndi yachilendo, zidzatenga zaka zambiri tisanamvetsetse bwino ntchito ya opereka ndalama ku HSCT chifukwa cha matendawa.

Zotsatira:

Ballen K. Mmene mungayankhire mafunso opatsirana mu myelofibrosis. Magazi a Magazi a Magazi. 2012; 2: e59.

Tefferi A. Utsogoleri wa myelofibrosis oyambirira. Mu: UpToDate, Post TW (Ed), UpToDate, Waltham, MA. (Idapezeka pa June 29, 2016.)