I-myelofibrosis yeprayimari okanye idiopathic, eyaziwa nangokuthi i-agnogenic myeloid metaplasia, isifo sesibongo somnxeba apho umnkantsha uvelisa izicubu ze-fibrous kwaye uvelisa iiseli zegazi ezingavamile. Idiopathic myelofibrosis ngenye yeengxaki ze- myeloproliferative . Esi sifo sichaphazela amadoda kunye nabasetyhini kwaye ngokuqhelekileyo zifunyaniswe kubantu ngabanye phakathi kweminyaka engama-50 ukuya kwe-70 ubudala, kodwa ziyakwenzeka nanini na ubudala.
Kuqikelelwa ukuba kwenzeka kwi-2 kubantu abayi-1 000 000.
Iimpawu
Inani elinama-25% labantu abane-idiopathic myelofibrosis abanalo iimpawu. Abo banezibonakaliso banakho ukuba:
- Ipenti ekhulisiweyo, ebangela ukungathandeki kwisisu esisentla-kwesibini okanye intlungu ehlombe eliphezulu
- Ubuthathaka, ukukhathala
- Ukuqhawukelwa ngumphefumlo
- Ukuhla ukusinda
- Ukukhupha ebusuku
- Ukuphuma ngegazi
Ezinye iindidi ezinzulu ze-idiopathic myelofibrosis zinakho:
- Iimvumba ezenziwe ngokuvelisa iiseli zegazi ezingenza ngaphandle kwethambo lomnkantso nakweyiphi icubu emzimbeni
- Ukunciphisa ukuhamba kwegazi kwisibindi, okukhokelela kwimeko ebizwa ngokuthi "i-portal"
- Iimvumba ezixubileyo kwi-oopopus, eyaziwa ngokuba yi-varophageal varices, ezinokuthi ziphule kwaye ziphule.
Ukuxilongwa
Kwabo bantu abangenayo impawu, idiopathic myelofibrosis ingafunyanwa xa uvavanyo lwezempilo oluqhelekileyo lufumana ipeni ekhulisiweyo kunye neziphumo ezingavamile zokuhlolwa kwegazi. Ezi ziphumo zingabonisa inani elingaphantsi kweeseli ezibomvu zegazi (kubangele i- anemia ), inombolo ephakamileyo kunamalungu eeseli ezimhlophe, kunye nenani elingaqhelekanga lamaplatele (ingaba iphakamileyo okanye iphantsi).
Abanye abantu, nangona kunjalo, bangabonisa utshintsho oluncinane kumanani eeseli zegazi.
Xa isampuli yegazi lomntu ihlolwe phantsi kwe-microscope, iiseli zegazi ezingaqhelekanga ziyabonakala. Ezinye iimvavanyo zegazi zingaqhelekanga ngokunjalo. Ukukunceda ukuqinisekiswa ukuxilongwa kwe-idiopathic myelofibrosis, isampuli yentambo yesibongo (i-biopsy) iya kuthathwa iphinde ihlolwe phantsi kwe-microscope ukuba khona kwe-fibrosis.
Unyango
Abantu abangenayo impawu ngokubanzi abaphilwanga. Iimvavanyo zegazi zenziwa rhoqo ukuze zijonge i-disorder.
Kwalabo abanempawu, unyango lusekelwe ekuphuculeni ukungonakali nokunciphisa umngcipheko weengxaki. Abantu abane-anemia banokufumana i-iron, folate kunye / okanye igazi lokumpontshelwa igazi. Abanye banokuphathwa ngamachiza, njengeDeltasone (prednisone) okanye i-Zometa (zoledronic acid).
Abantu abanamanani aphezulu eeseli zegazi angaphathwa ngamachiza, njenge Hydrea (hydroxyurea), Agrylin (anagrelide) okanye i-interferon alfa.
Abanye abantu bangadinga ukuba i-spleen isuswe ngokugqithiseleyo (splenectomy), ingakumbi ukuba ibangela iingxaki. Ezinye iindlela zonyango zingabandakanywa unyango lwe-radiation okanye umongo wesithambo (isistim e-stem) .
Outlook
Ngokomyinge, abantu abane-idiopathic myelofibrosis baphila iminyaka emihlanu emva kokuxilongwa. Phantse i-20% yabantu abanenkinga, nangona kunjalo, basinda kwiminyaka eyi-10 okanye ngaphezulu. La manani ayanda kwithuba, njengoko uphuhliso olutsha luphuhliswa kwaye uphando luphucula ulwazi lwesifo.
Imithombo:
> "Idiopathic Myelofibrosis." IiNtsholongwane. 24 Septemba 2007. Umdla weLukemia & Lymphoma Society.
> Niblack, Joyce. "MF FAQ." MPD-FAQS. 3 Feb 1999. MPD Research Center, Inc.