I-myelofibrosis eyinhloko (i-PMF) ingenye yezinkinga eziningi zegazi ezihlukaniswa njengama-neoplasms e-myeloproliferative. I-Neoplasm ichazwa njengokukhula okungavamile kwezicubu ezibangelwa ukuguqulwa komzimba futhi zingahle zihlukaniswe njengezinhle (ezingekho embi), zangaphambi kokulimaza, noma ezimbi. Amaphilisi amancane ase-Myeloproliferative ngokuvamile ayenobungozi ekuqaleni, kodwa ngokuhamba kwesikhathi angaba yisifo esibi (umdlavuza).
Ukuguqulwa kwe-PMF kuholela ku-fibrosis (ukukhwabanisa) komnyofu wethambo. Lokhu kutholakala emnothweni wethambo kuphazamisa ukuthuthukiswa okuvamile kwamangqamuzana egazi. I-anemia iyona yokuthola ibhethri evame kakhulu. I-leukocytosis (ukuphakama kwamangqamuzana egazi amhlophe) kanye ne-thrombocytosis (inani eliphakeme leplatelet) livamile kodwa njengoba lesi sifo siphuthuka, i-thrombocytopenia (isibalo esiphansi se-platelet) singase senzeke. I-splenomegaly (ukukhuliswa ngo-spleen) iqala njengoba i-spleen iba indawo yesibili yokukhiqizwa kwamaseli egazi.
Ingabe Wonke Umuntu Udinga Ukwelashwa?
Ngenkathi isinyathelo sakho sokuqala singase sibe ukuhlola izinketho zokwelapha okungenzeka, khumbula ukuthi akubona bonke abantu abane-PMF abadinga ukwelashwa. Ukwelashwa kwe-PMF kunqunywe ingozi yokuqhubeka kwezifo nokuphila okungaphezu kweningi.
Isistimu ebizwa ngokuthi i-Dynamic International Prognostic Scoring System System (DIPSS) Plus score isebenzisa ulwazi olumayelana nomuntu onjengobudala, isibalo segazi elimhlophe, i-hemoglobin, ukujikeleza kwamaseli amangqamuzana, ukutholakala kwezimpawu, izakhi zofuzo, isibalo seplatelet, kanye nesidingo sokumpontshelwa ukubala amaphuzu.
Ukusebenzisa lesi sistimu abantu abane-PMF bangahlukaniswa ngezigaba ezine zokubikezela: ubungozi obuphansi, ubungozi obuphakathi-1, ubungozi obuphakathi-2, kanye nobungozi obukhulu. Izigaba zokuphila eziphakathi kweMedian ezivela engaphezu konyaka owodwa ezigulini ezinezinkinga eziphakeme eziyiminyaka engu-15 ezigulini ezinesifo esincane. I-PMF kubantu abangaphansi kweminyaka engama-60 ihlotshaniswa nokubikezelwa okungcono nokusinda okuphakathi kwaminyaka emibili kuya eminyakeni engu-20.
Izibalo ze-Hematologists zisebenzisa isaphulelo se-DIPPS Plus kanye nokuguqulwa kwesimo somuntu ukucacisa uhlelo lokulashwa. Abantu abanezifo ezinobungozi abangenayo izimpawu abaphathwayo kodwa abaqashwe ngokucophelela ngezinkomba kanye nokwehla kwesifo se-anemia kanye / noma i-thrombocytopenia. Uma umuntu eqala izimpawu (umkhuhlane, ukulahlekelwa isisindo, ukujuluka ngokweqile noma ukwandisa okukhulu kwe-spleen) noma isidingo sokumpontshelwa, ukwelashwa kufanele kuqaliswe. Ngokuvamile ukumpontshelwa kwegazi okwebomvu kunikezwa lapho i-hemoglobin ingaphansi kuka-8 g / dL. Ngenxa yokuthi ukumpontshelwa kwegazi obomvu okuphindaphindiwe kubangela ukuba insimbi ibe yindlala, ngokuvamile ezinye izindlela zokwelapha ziyazama.
Ukwelashwa Kwezimpawu
- I-Splenomegaly: Uma i-spleen isanda kakhulu futhi ibangela izinkinga (njengokungahambi kahle, ukusabalalisa okuningi kwe-splenic, ukwanda kwezidingo ze-transfusion), imithi yomlomo i-hydroxyurea ingasetshenziswa. Ngalokhu ukwelashwa ngamaphesenti angu-40 abantu abane-PMF banciphisa amaphesenti angu-50 ubukhulu besipeni buhlala cishe unyaka owodwa. Uma i-spleen ingaphenduli ekwelashweni kwe-hydroxyurea, i- splenectomy (ukususwa kokuhlinzwa kwe-spleen) kungadingeka.
- I-anemia: I- anemia ku-PMF ingase iphathwe ngemithi ehlukahlukene njenge-fluoxymesterone, prednisone, noma i-danazol. I-Fluoxymesterone ne-danazol yaziwa ngokuthi i-androgens (i-hormone ye-steroid) ebonakala ikhuthaza ukukhiqizwa komnkantsha we-bone. Enye yezinkinga ezinkulu kule mithi ukuthi ihlobene namahomoni wesilisa futhi angabangela ukuthuthukiswa kwezinwele zomzimba, izwi elijulile, noma ukunyuka kwenqwaba yemisipha. I-Thalidomide noma i-lenalidomide (uhlobo lwe-chemotherapy) kanye ne-prednisone ingasetshenziswa futhi.
Ingozi Ephakeme noma Ephakathi
Abantu abanesifo esiphakathi nesifo esiphezulu bangadinga ukwelashwa okunye. Kuyaqondakala ukuthi kunzima ukuzwa ukuthi isifo sakho singcono kakhulu-ingozi yokukhetha ukwelashwa kungasiza ekunciphiseni ukukhathazeka nokwesaba ongase uzizwe.
- I-Hematopoietic stem cell transplant (i-HSCT noma ukufakelwa komnkantsha wamathambo) : Lena yilapha kuphela yokwelapha okwelapha i-PMF kodwa inengozi enkulu. Ukutshala izihlahla kufanele kwenzeke ngemuva nje kokuxilongwa ngaphambi kokuthuthukiswa kwezinye izinkinga ukunciphisa izinkinga. Ngokomlando, ukuguqulwa kuye kunqunyelwe kubantu abangaphansi kweminyaka engama-60 abaye bafanelana nabanikezeli bezingane abahlala nabo (MSD) . Ukutshantshwa kwamuva nje kwenziwe ngokuhambisana nabaxhasi abathintekayo abangahlobene noma abangathintana nabo.
- I-Ruxolitinib: Abantu abane-PMF nezibonakaliso ezinzima abangabhalwanga i-HSCT bangasebenzisa i-ruxolitinib. I-Ruxolitinib imithi eyaziwa ngokuthi i-tyrosine kinase inhibitor, ikakhulukazi i-JAK2 inhibitor. I-JAK2 inguquko ejwayelekile ku-PMF kodwa ingatholakala nakwezinye izifo ezingezansi ze-myleoproliferative njenge-polycythemia vera kanye ne-thrombocythemia ebalulekile. Ukwelashwa nge-ruxolitinib kunganciphisa usayizi we-spleen, kuncishiswe izimpawu (njengokukhathala, ubuhlungu be-bone), nokunciphisa i-anemia. Nakuba le mithi ihlose ukuguquguquka kwe-JAK2, iziguli ezinezinye izakhi zingaphinde ziphendule.
Imithombo:
I-Teferri A. Ukuguqulwa Kwe-Primary Myelofibrosis Nokuphathwa Kwe-Primary Myelofibrosis. Ku: UpToDate, Post, TW (Ed), UpToDate, Waltham, MA, 2016.