I-myelofibrosis yaseprayimari (PMF) yenye yeengxaki ezininzi zegazi ezichazwe njenge-neoplasms ze-myeloproliferative. I-Neoplasm ichazwa njengokukhula okungavamile kwezicubu ezibangelwa ukuguqulwa kwaye zingachazwa njengezintlu (ezingekho embi), zangaphambili ezibi okanye ezibi. Izidumbu ze-Myeloproliferensi zihlala ziphazamisa ekuqaleni, kodwa ekuhambeni kwexesha zingaba zifo ezibi (umdlavuza).
Ukuguqulwa kwe-PMF kubangela i-fibrosis (inqabile) yomnatha wethambo. Ukutshatyalaliswa komnatha wethambo kukuphazamisa ukuphuhliswa okwejwayelekile kweeseli zegazi. I-anemia yinto ebalulekileyo yokufumana ibhoratri. I-leukocytosis (ukuphakama kweeseli ezimhlophe zegazi) kunye ne-thrombocytosis (inani eliphakamileyo leplatelet) liqhelekile kodwa njengoko isifo sifana, i-thrombocytopenia (inani eliphantsi leplatelet) lingafumaneka. I-Splenomegaly (ukukhulisa kwipeni) ikhula njengoko ipen ibe yindawo yesibili yemveliso yeseli yegazi.
Ngaba Wonke umntu ufuna unyango?
Nangona isinyathelo sakho sokuqala sinokuthi uhlolisise iindlela ezinokunyanga, khumbula ukuba kungekhona bonke abantu abane-PMF abafuna unyango. Unyango lwe-PMF lugqitywe ngumngcipheko wokunyuka kwesifo kunye nokuphila kwangaphezulu.
Inkqubo ebizwa ngokuba yi-Dynamic International Prognostic Scoring System (i-DIPSS) Plus isebenzisa ulwazi malunga nomntu onjengobudala, isibalo segazi elimhlophe, i-hemoglobin, ukujikeleza kweeseli eziqhutyayo, ubukho beempawu, i-genetics, isibalo seplatelet, kunye ne-transfusion kufuneka ukubala amanqaku.
Ukusebenzisa le nkqubo abantu abane-PMF banokuhlulwa ngezigaba ezine zokuxela: umngcipheko ophantsi, ubungozi obuphakathi-1, ubungozi obuphakathi-2, kunye nobungozi obuphezulu. Amanqanaba okusinda eMediya ukusuka kwiminyaka engaphezu kweyodwa kwizigulane ezinezifo eziphezulu zengozi ukuya kwiminyaka eyi-15 kwizigulane ezinezifo ezincinci. I-PMF kubantu abangaphantsi kweminyaka engama-60 idibaniswa nokugqithiswa okungcono kunye nokuphila okuphakathi kwamibini kwiminyaka engama-20.
Abadlali be-Hematologists basebenzisa i-DIPPS Plus amanqaku kunye nomtshintsho wofuzo lomntu ukucacisa isicwangciso sonyango. Abantu abanezifo ezinobungozi abangenayo impawu abaphathwayo kodwa bahlolisiswa ngokugqithiseleyo iimpawu kunye nokunyuka kwe-anemia kunye / okanye i-thrombocytopenia. Ukuba umntu uhlakulela iimpawu (umkhuhlane, ukulahleka kwesisindo, ukujuluka okugqithiseleyo okanye ukwandiswa kwetyeni) okanye iimfuno zokumpontshelwa igazi, unyango kufuneka luqaliswe. Ukuxilongwa kwegazi okomvu ngokuqhelekileyo kunikwa xa i-hemoglobin ingaphantsi kwe-8 g / dL. Ngenxa yokuba igazi eliphindaphindiweyo lokufomfelwa kwegazi likhokelela kwi-iron overload, ngokuqhelekileyo ezinye iindlela zonyango zizama.
Unyango lweMpawu
- I-Splenomegaly: Ukuba ipenki ikhulise kakhulu kwaye ibangela imiba (njengokuba ingonakalisi, i-splenic ephasayo, ukwanda kweemfuno zokumpontshelwa igazi), imyeza yomlomo i-hydroxyurea isenokusetyenziswa. Ngolu unyango malunga neepesenti ezingama-40 zabantu abane-PMF banokunciphisa ipesenti ezingama-50 kwindwangu yeestile ehlala malunga nonyaka omnye. Ukuba ipeni ayiphenduli unyango lwe-hydroxyurea, i- splenectomy (ukususwa kokutyunjwa kweseyile) kunokufuneka.
- I-anemia: I- anemia kwi-PMF inokuphathwa ngeentlobo zamachiza ezinjenge-fluoxymesterone, prednisone, okanye i-danazol. I-Fluoxymesterone kunye ne-danazol zibizwa ngokuba yi-androgens (i-hormone ye-steroid) ebonakala ibangela ukuveliswa kwemveliso yengongo. Enye yezinto ezingalunganga zala mayeza kukuba zihambelana namahomoni angamadoda kwaye zingabangela ukuphuhliswa kweenwele zomzimba, ilizwi elizondayo, okanye ukwanda kwenqwaba yemisipha. I-Thalidomide okanye i-lenalidomide (uhlobo lwe-chemotherapy) kunye ne-prednisone ingasetyenziswa kwakhona.
Umngcipheko ophezulu okanye ophakathi
Abantu abanezifo eziphambili kunye nabasemngciphekweni bangadinga unyango oluthile. Kuyaqondakala ukuba kunzima ukuva ukuba isifo sakho sisona siphezulu-nolwazi lwezonyango lunokukunceda ukunciphisa ezinye iinkxalabo kunye nokwesaba onokuziva.
- I-Hematopoietic stem cell transplant (i-HSCT okanye ithambo lokutshintsha umongo) : Lona kuphela lonyango olukhuselweyo lwe-PMF kodwa lunobungozi obukhulu. Ukutshintshwa kufuneka kuqhutywe emva nje kokuxilongwa ngaphambi kokuphuhliswa kweminye ingxaki yokunciphisa iingxaki. Ngokomlando, ukuguquka kuye kwaphela kubantu abangaphantsi kweminyaka engama-60 abaye bahambelana nabaxhasi be-sibling (MSD) . Ukutshintshana kutshanje kwenziwe ngokuhambelana nabaxhasi abanxulumene nabaxhasiweyo.
- I-Ruxolitinib: Abantu abane-PMF kunye neempawu ezinzima abangabaviwa kwi-HSCT bangasebenzisa i-ruxolitinib. I-Ruxolitinib yipilisi eyaziwa ngokuba yi-tyrosine kinase inhibitor, ngokukodwa i-JAK2 inhibitor. I-JAK2 iyinto eguqukileyo eguquguqukayo kwi-PMF kodwa inokufumaneka nakwezinye i-myleoproliferative neoplasms ezifana ne-polycythemia vera kunye ne-thrombocythemia ebalulekileyo. Ukwelashwa nge-ruxolitinib kunokunciphisa ubukhulu be-spleen, ukunciphisa iimpawu (njengokukhathala, ubuhlungu besifo), nokunciphisa i-anemia. Nangona la mayeza ajoliswe kuguquko lweJAK2, izigulane ezinezinye iinguqu zinokuphendula.
Imithombo:
I-Teferri A. Ukuxhalabisa kwe-Primary Myelofibrosis kunye noLawulo lweMyelofibrosis. Ku: Uhla, Iposi, TW (Ed), UpToDate, Waltham, MA, 2016.