I-Myelofibrosis iyisifo somongo wethambo esibangela ukuba umnkantsha ube yintambo, okanye inqabileyo. Iifom ezinqabileyo ngaphakathi kweengxube zomnyozi wegazi, kwiindawo ezingcwele zamathambo athile. Nangona kunjalo, "utywala" kwi-myelofibrosis, nangona kunjalo, akufani nokuqhekeza okubangelwa kwilonda lokuphulukisa. Umnkantso we-Bone ngumhlaba wokuvelisa kuwo onke amangqamuzana egazi lomzimba, kwaye ngoko inkqubo yokuqhawulayo eqalayo ayikho into enobungozi, kwaye ekugqibeleni ingakhokelela kwiingxaki ngokubala kweeseli, iingxaki ezinzulu kunye nokunciphisa ixesha lokuphila.
Xa i-fibrosis ithatha, oku kuqhubela phambili ekuveliseni iiselingi zegazi, ngokungaqhelekanga kwimizimba yeseli kunye ngamanye amaxesha kwiseli yegazi ngokwabo. Ekugqibeleni, kwi-myelofibrosis ephambili, oku kungenza umongo ungaphumeleli ukwenza umsebenzi wayo ngokupheleleyo. Xa i-myelofibrosis iqhubela phambili okanye iguquka, iingxaki ezisongela ubomi ezifana ne-leukemia kunye negazi eliphambili kunye nokukhupha iimeko ezinokukhupha. Kwakhona kunokwenzeka ukuba i-myelofibrosis iholele kwi -leukemia ephawulekayo , umdlavuza wegazi obungozi.
I-Primary ye-Myelofibrosis
I-Myelofibrosis ingaba yiprayimari okanye yesibini. Xa ivela ngokutsha, okanye ifunyanwa yodwa ngokwayo okokuqala, ibizwa ngokuba yi- primary myelofibrosis. Xa ikhula ngokubambisana nesinye isifo, ukulimala okanye imeko yegazi kuthiwa yi- secondary myelofibrosis. Ngokomzekelo, i-myelofibrosis emva kwesifo segazi esibizwa ngokuba yi- polycythemia vera yindlela enye ye-myelofibrosis yesibini.
Ngaba Kufana Njalo Njengomdlavuza?
Izinto ezininzi zifana nomhlaza, kodwa iingcali zibhekisela kuyo "njenge- neoplasm" ye-myeloproliferative neoplasm . Ukugcina izinto zilula, igama le- neoplasm linokucingelwa njengegama eliqingqiweyo elithi "ucuba okanye ukukhula" kunye nezicubu okanye ukukhula kungabonakalisa okanye ezimbi. I-Myelofibrosis ayiqinisekanga, kodwa nayo ayikho impawu abantu abaye bayilindela xa becinga ngomhlaza, okanye i-neoplasms ezimbi.
Oko kwathiwa, nokuba ubheka i-myelofibrosis njengomhlaza okanye ukukhula okulimazayo kweethambo zomncuba, okwangoku akukho nonyango lonyango olukhuselayo (kodwa ukutshintsha umongo we-bone kungenza unyango), kwaye yinkqubo enokuthi iqhubele phambili ingozi-kungakhathaliseki ngezantya ezahlukeneyo kubantu abahlukeneyo. Iifomu eziphambili ze-myelofibrosis zinciphisa ixesha lokuphila kwaye zidala umthwalo wempilo obalulekileyo kwizigulane.
Uza kufumana iindawo ezininzi ezinokugula ezibhekisela kwi-myelofibrosis njenge "mdlavuza ongabonakaliyo wegazi." Oku kunokuba yindlela efanelekileyo yokunxibelelana nombono jikelele, kodwa kukho enye ibali. I-Myelofibrosis ingakhokelela kumdlavuza wegazi, kodwa kwezinye iimeko, i-myelofibrosis ingabangela umdlavuza wegazi.
Iintlobo
Ngaphandle kweprayimari kunye nesibini, kukho ezinye iindlela zokwenza i-myelofibrosis. Enye indlela kukuba iimeko zecandelo lesi sifo zibe yintlupheko eyahlukeneyo kuxhomekeke kwiziphumo zomntu xa isifo sifumaneka okokuqala. Izixhobo eziliqela ezahlukeneyo ziyafumaneka ukunceda oogqirha banqume izinga lakho lomngcipheko, ukukunceda ukukhokela unyango kunye nokulolonga.
Ukukhula
Ngokwe -Leukemia & Lymphoma Society , i-myelofibrosis iyenzeka malunga nama-1.5 kubantu abayi-100,000 eMelika ngamnye ngonyaka.
Ichaphazela amadoda namabhinqa kwaye ngokuqhelekileyo ifunyanwe kubantu abangaphezu kweminyaka engama-60, kodwa inokwenzeka nanini na. Kulinganiselwa ukuba malunga nama-16,000 ukuya kuma-18 500 abantu base-United States bane-myelofibrosis.
Izizathu
Iindidi ezininzi zeerrow neesejethi ziyaziwa ukuba zibandakanyeka, nangona kunjalo, isizathu esicacileyo sokuqhekeza ngokweqile kwi-myelofibrosis akucaci ngokupheleleyo. Uninzi lweemfuyo kunye ne-chromosomal eziye zafunyanwa, kubandakanya ukuguquka okubizwa ngokuthi "JAK2 V617F ukuguqulwa kwamashishini," nangona ukuguqulwa kwamanzi akuthethi ukuba uya kuba yi-primary myelofibrosis. Isizathu sokuguquka okunjalo asiyazi, kwaye akukho zichaso ezithile okanye izinto ezinobungozi ezinokudityaniswa kwi-myelofibrosis ephambili kwiimeko ezininzi.
Ukongeza kwii-blood cancer, ezinye iingxaki zegazi ezingenayo umdlavuza ezibizwa ngokuba yi-"syeloproliferative neoplasms" ezifana ne-polycythemia vera kunye ne-thrombocythemia ebalulekileyo nayo inokukhokelela kwimizi-myelofibrosis yesibini. I-myelofibrosis yesiSekondari okanye esebenzayo inokuthi iyenzeka ekuphenduleni imichiza okanye ukulimala emzimbeni, ukusuleleka okanye ukulahleka kwegazi kwi-bone lomnatha.
I-myelofibrosis yamabanga aphezulu, i-myelofibrosis ehlobene ne-polycythemia vera, kunye ne-myelofibrosis enxulumene ne-thrombocythemia ebaluleke kakhulu, maxa wambi ihlanganiswa kunye "njenge-myelofibrosis," kodwa izazinzulu zithi kukho kunokufunda nokuqonda malunga nokungafani phakathi kohlobo ngalunye.
Iimpawu
Izigulane ezininzi azikho iimpawu ngexesha lokuxilongwa, kodwa iimpawu eziqhelekileyo ziquka ezi zilandelayo:
- Ukukhathala
- Ukuhla ukusinda
- Ukukhupha ebusuku
- Fever
- Uvakalelwa kukuba kunzima ukuphefumla
- Ukuphazamiseka esiswini (ngenxa yengubo ekhulisiweyo)
Ukungaphumeleli komnxeba wethambo kungakhokelela kwiimpawu ezivela kwiindawo eziphantsi kwegazi, ezifana nokukhathala ngenxa yokuba neeseli zegazi ezibomvu kakhulu. Impembelelo kwiiplatelet zegazi zingakhokelela ekuphepheni kwegazi kunye neengxaki zokuqhawula.
Ezinye iimpawu, ezifana nokuzaliswa kwesisu okanye uxinzelelo, kungenzeka ngenxa yento yonke eyenzeka ngaphandle komnxeba wethambo obunobumba, ukwenzela ukwenza iiseli ezintsha zegazi:
- Ngokuqhelekileyo, ukuhamba kwemveliso yeeseli yegazi kwintshontsho yamathambo kwiintsana okanye malunga nexesha lokuzalwa. Ngaphambi kokuzalwa, nangona kunjalo, iintsana zinokuvelisa iiseli zegazi ezintsha kwiindawo ezinjengepen, isibindi, kunye ne-lymph nodes-ezi ndawo ezingaphandle komnatha wethambo zibizwa ngokuthi yi-extramedullary.
- Ngokuqhelekileyo kubantu abadala, isayithi kuphela yendlela yokwenziwa kweeseli zegazi elitsha ngumongo wethambo. Kwimiba ethile yegazi kunye neengxaki zegazi, abantu babuyela ekuveliseni iiseli zegazi apho bekunakho khona, kulezi ziza ezixhamlayo. Kwi-myelofibrosis, oku kudla ngokuqhelekileyo kwintente kunye nesibindi. Ngamanye amaxesha ipenethi yomntu ingaba mkhulu ngenxa ye-hematopoiesis exramedry in myelofibrosis.
Iingxaki ezinkulu ze-myelofibrosis ngokubanzi zivela ekuphelelweni komongo we-bone kunye ne -hematopoiesis exramedullary .
Kukho umngcipheko ophezulu wenguqu kwi- acute leememiaid (AML) ne-myelofibrosis, kwaye malunga neepesenti ezingama-20 zabantu abane-myelofibrosis bahlakulela i-leukemia.
Ukuxilongwa
Ukongeza kwenkcazelo ugqirha wakho ufumana iimpawu zakho kunye nokuhlolwa komzimba, kukho iimvavanyo eziliqela ezinika ulwazi oluxabisekileyo lokuxilonga. Ezi zibandakanya ukubala kwegazi, enye imisebenzi yegazi, iimvavanyo zengcamango ezifana ne-x-ray kunye neMRI, iimvavanyo zomnatha weethambo kunye neemvavanyo zomzimba. Isampula segazi okanye ithambo lomnxeba linokuthunyelwa kwibhubhoratri ukujonga ukuguqulwa kwemfuza (njengeJAK2, CALR, okanye iinguqulelo ze-MPL) ezihlala zikhona kubantu abane-myelofibrosis.
Ezinye izinto ezinokuthi zibukeke njenge-myelofibrosis, kodwa azinjalo, ziquka i-leukemia engapheliyo, i-syndromes, i-myelomonocytic leukemia, kunye ne-leukemia engummangaliso.
Unyango
Okwangoku, akukho khetho lweziyobisi oluyingozi. Injongo yezigulane ezininzi kukunciphisa iimpawu, ukunciphisa ipeni ekhulisiweyo kunye nokuphucula ukubala kweeseli zegazi. Kwikhonsathi kunye nale njongo, injongo ephezulu kukunciphisa umngcipheko weengxaki.
I-Myelofibrosis isifo esinokukhetha amanyango angamkelekanga unyango, kodwa amaninzi amatsha agqitywa kwaye aphuhliswa. Unyango lukhokelwa yimiba ethile efana nobukho beempawu, kunye nomngcipheko wecala lakho le-myelofibrosis, kunye nakwiminyaka yakho kunye nempilo / jikelele.
Kubantu abanomngcipheko ophantsi kwaye bengenazo iimpawu, ukubonwa kwedwa kunokuhle. Ngenxa yesifo esichengeni kakhulu, ukutshintshwa kwe-cell stem kwi-donor kudlalwa ingqalelo, kodwa akubona zonke izigulane ezifanelekileyo ngenxa yemingcipheko. Ezinye izigulane zizonyango ezifanelekileyo zonyango oluqhelekileyo okanye unyango lweziyobisi uphando kwilingo lonyango.
Ngo-2011, i-Food and Drug Administration (i-FDA) ivunyiwe i-ruxolitinib (Jakafi) yokonyango lwe-myelofibrosis ephezulu kunye ne-high-risk myelofibrosis, i-post-polycythemia vera myelofibrosis, kunye ne-post-polycythemia myelofibrosis.
- Imvume ye-FDA isekelwe kwimiphumo yezilingo ezimbini ezilawulwa ngandlela-thile kwizigulane ezine-intermediate or risk-risk myelofibrosis ngokuthelekisa i-ruxolitinib kwindawo ye-placebo (Isifundo 1) okanye ukufumana unyango olufumanekayo (iSifundo 2). KwiSifundo se-1, iipesenti ezingama-42 zezigulane eziphathwe nge-ruxolitinib, xa kuthelekiswa neepesenti eziyi-1 zezigulane eziphathwe nge-placebo, zineziphumo ubuncinane zokunciphisa ipesenti ezingama-35 zobungakanani bepen kwiiveki ezingama-24. Ngexesha lokuvunywa, iipesenti ezingama-75 zezigulane kwiSifundo se-1 kunye nama-67 ekhulwini kwiSifundo 2 esiye sazuza okungenani amaphesenti angama-35 ekunciphiseni umthamo womthamo wagcinwa ukunciphisa umthamo wendiza.
- I-Ruxolitinib isebenza ngokuphumelelayo ekunciphiseni ubukhulu bepen kunye nokunciphisa iimpawu ezininzi kwizigulane. Kwi-ruxolitinib yokuvavanywa klinikhi kwizigulane ezine-high-risk myelofibrosis, iqela elikhulu labathathi-nxaxheba lalinokuphucula ngokuphawulekayo kwiimpawu ezihambelana nesi sifo: babhala idayari yemihla ngemihla ebamba iimpawu eziphosakeleyo ze-myelofibrosis ezibandakanya ukuphazamiseka kwesisu, intlungu phantsi kweembambo zangasobunxele, ukuhlambalaza, ukukhupha ebusuku kunye nentlungu / isisu.
- Impendulo echaphazelekayo yezidakamizwa eziqhelekileyo, ibone ubuncinane iipesenti ezi-1 zezigulane eziphathwe nge-ruxolitinib, ziquka iplatelet ephantsi, i-anemia, i-bruising, i-flowing, ne-headache. Ukusabela kwezidakamizwa ezingalunganga kwinqanaba lokubandezeleka okwangaphezulu kwizigulane eziphathwe nge-ruxolitinib xa kuthelekiswa ne-placebo kwiSifundo se-1 ziquka iplatelets ephantsi (amava eepesenti ezingama-13 eziguliswa nge-ruxolitinib, xa kuthelekiswa neepesenti eziyi-1 zezigulane eziphathwa nge-placebo) kunye ne-anemia ( Iipesenti ezingama-45 zezigulane ziphathwa nge-ruxolitinib, xa kuthelekiswa neepesenti ezingama-19 zezigulane eziphathwe nge-placebo). Iziphumo ezifanayo zibonwe kwiSifundo 2.
Ezinye iindlela ezifunyenwe zisebenzayo kwipenti ekhulisiweyo kunye nokulawulwa kweempawu ziquka i-chemotherapies, ukususwa kwepeni okanye i-splenectomy, kunye neyeza elisezantsi le-radiation kwipeni. Ukunyelwa igazi kunokunikwa i-anemia, kunye nezigulane ezine-anemia ezixhomekeke kumpontsho, izidakamizwa zethambo-ezikhuthaza i-erythropoietin, i-androgens (umzekelo, i-danazol), kunye ne-immunomodulators (umzekelo, lenalidomide) isenokusetyenziswa.
Prognosis
Ngokusekelwe kwizifundo ezidlulileyo, amanye amaqela abantu abafumene i-myelofibrosis bahlala iminyaka emininzi, ngelixa kwamanye amaqela, amaxesha okuphila aphantsi kweminyaka engama-3 kuya kwe-5 ukusuka kuhlolwe. Phantse iipesenti ezingama-60 zezigulane ezinezi-primary / idiopathic myelofibrosis zihlala iminyaka emi-5. Kukho iqela elikhulu lezigulane, nangona kunjalo, abahlala iminyaka eyi-10 okanye ngaphezulu.
Abo bavame ukwenza kakuhle baquka abo abanamazinga e-hemoglobin angaphezulu kwe-10 g / dL, iplatelet ibalwa ngaphezu kwe-100x3 / uL kunye nalabo abanokubhentshiswa kwesibindi. Ubungakanani bePenethi kunye nobulili abubonakali ukuba lunempembelelo enkulu ekusindeni kwizifundo, nangona kwinqanaba, ukunciphisa ubukhulu bepen buyakwazi ukuguqulela kwizibonelelo zokuphila kwezinye iimeko.
ILizwi
Kuze kube ngoku, ukusinda kwabantu abane-myelofibrosis ephambili kubonakala kunento eninzi malunga nempawu zabo kunye nokubonakaliswa kwesi sifo ekuqaleni, kwaye akukho mpe mbelelo yonyango okanye unyango olulodwa; Nangona kunjalo, le nto ingumgaqo wokuziphendukela kwemvelo onokutshintsha njengoko isayensi iqhubeka. Unyango olutsha luhlala lukhula kwaye isayensi kule ndawo iyanda ngokukhawuleza.
Imithombo:
> I-Myelofibrosis: Iinkcazelo ezintsha zeNgcaciso lwezeMpilo: Ulungiso lwe-2013. IiScholarlyEditions, Julayi 22, 2013.
> Gangat N, Caramazza D, Vaidya R, et al. I-DIPSS-plus: I-System Dynamic Prognostic Scoring System (iDIPSS) ye-Myelofibrosis eyimfuneko ebandakanya i-Karyotype, i-Platelet Count ne-Transfusion Status. J Clin Oncol. 2011; 29: 392-397.
> I-Greenberg PL, i-Attar E, uBennett JM, et al. I-Myelodysplastic Syndromes: Izikhokelo zoLwazi lweeNkcazo kwi-Oncology. JNCCN. 2013; 11 (7): 838-874.