I-myelofibrosis eyinhloko noma idiopathic, eyaziwa nangokuthi i-agnogenic myeloid metaplasia, iyisifo somnkantso wamathambo lapho umnkantsha uveza izicubu ezinomzimba futhi ukhiqiza amangqamuzana egazi angavamile. Idiopathic myelofibrosis ingenye yezinkinga zemithi yokwelapha . Lesi sifo sithinta kokubili amadoda nabesifazane futhi kuvame ukutholakala kubantu abaphakathi kweminyaka engama-50 kuya kwangu-70 ubudala, kodwa kungenzeka noma kunini.
Kucatshangwa ukuthi kwenzeka ku-2 kwabangu-1,000 000 abantu.
Izimpawu
Abantu abangaba ngu-25% abane-idiopathic myelofibrosis abanalo izimpawu. Labo abanayo izimpawu bangase babe nalokhu:
- Ipeni ekhulisiwe, ebangela ukungahambi kahle esiswini lesokunxele kwesisu noma ubuhlungu ehlombe elingenhla kwesokunxele
- Ubuthakathaka, ukukhathala
- Ukuphelelwa umoya
- Ukulahlekelwa kwesisindo
- Izithukuthuku ebusuku
- Ukusuka okungaqondakali
Ezinye izinhlobo ezingathí sina ze-idiopathic myelofibrosis zingase zibe nalezi:
- Izicubu ezenziwe ngamaseli egazi angase akhiwe ngaphandle komnkantso wamathambo kunoma yiziphi izicubu emzimbeni
- Ukujula kwegazi kugijima esibindi, okuholela esimweni esibizwa ngokuthi "i-portal hypertension"
- Izindwangu ezixubile emgodleni, owaziwa ngokuthi i-esophageal varices, okungase kuphume futhi kuphume.
Ukuxilongwa
Kulabo bantu abangenayo izimpawu, idiopathic myelofibrosis ingatholwa lapho kuhlolwa ukuhlolwa kwezempilo njalo kutholakala ipeni ekhulisiwe nemiphumela engavamile yokuhlolwa kwegazi. Le miphumela ingabonisa inombolo ephansi kunaleyo yamangqamuzana egazi obomvu (okubangela i- anemia ), inombolo ephakeme kunaleyo yamaseli amhlophe egazi, nenombolo engavamile yamaplatelets (angase abe phezulu noma aphansi).
Abanye abantu, noma kunjalo, bangabonisa ushintsho oluncane ezinombolo zamaseli egazi.
Uma isampula segazi lomuntu ngamunye ihlolwe ngaphansi kwe-microscope, amangqamuzana egazi angavamile angabonakala. Okunye ukuhlolwa kwegazi kungase kube okungavamile futhi. Ukusiza ukuqinisekisa ukuxilongwa kwe-idiopathic myelofibrosis, isampula yamathambo (i-biopsy) izothathwa futhi ihlolwe ngaphansi kwe-microscope ukuba khona kwe-fibrosis.
Ukwelapha
Abantu abangenayo izimpawu ngokuvamile abaphathwa. Ukuhlolwa kwegazi kwenziwe njalo ukuze kuqaphele lesi sifo.
Kulabo abanezimpawu, ukwelashwa kusekelwe ekunciphiseni ukukhululeka nokunciphisa ingozi yezinkinga. Abantu abane-anemia bangathola i-iron, folate kanye / noma igazi lokumpontshelwa igazi. Abanye bangaphathwa ngemithi, njenge-Deltasone (prednisone) noma i-Zometa (zoledronic acid).
Abantu abanezinombolo eziphezulu zamaseli egazi bangaphathwa ngemithi, njenge-Hydrea (hydroxyurea), i-Agrylin (i-anagrelide) noma i-interferon alfa.
Abanye abantu bangase badinge ukuthi i-spleen isuswe ngokuhlinzwa (i-splenectomy), ikakhulukazi uma ibangela izinkinga. Ezinye izindlela zokwelapha zingase zibandakanye ukwelashwa kwemishanguzo noma ukuguqulwa kwamathambo (stem cell) .
I-Outlook
Ngokwesilinganiso, abantu abane-idiopathic myelofibrosis baphila iminyaka emihlanu ngemva kokuxilongwa. Kodwa-ke abantu abangaba ngu-20% abanenkinga, basinda eminyakeni eyishumi noma ngaphezulu. Lezi zibalo ziyanda ngokuhamba kwesikhathi njengoba kwenziwa ukwelashwa okusha futhi ucwaningo luthuthukisa ulwazi lwe-disorder.
Imithombo:
> "I-Idiopathic Myelofibrosis." Ulwazi Lwezifo. 24 Sep 2007. I-Leukemia & Lymphoma Society.
> Niblack, Joyce. "MF FAQ." I-MPD-FAQS. 3 Feb 1999. MPD Research Center, Inc.