I-Myelofibrosis yisimiso lapho ukubola (fibrosis) kufaka emnothweni wethambo. Lokhu kunzima kwenza kube nzima ukuba umnkantsha wakho wenze amangqamuzana egazi ngokuvamile.
Izimpawu
I-Myelofibrosis ingabangela izimpawu. Kodwa-ke, kungase kuthathwe umsebenzi wokusebenza wegazi okwenziwa ngumdokotela wakho. Ezinye izimpawu zihlobene nokwehlisa amaseli egazi abomvu (i- anemia ) namaplatelets ( thrombocypenia ), afana:
- Ukukhathala noma ukukhathala
- Pallor
- Amachashazi abomvu esikhumbeni esibizwa ngokuthi i-petechiae
- Ukulimala okwandisiwe
- Ukuphuma ngokweqile
- Ipeni ekhulisiwe kanye / noma isibindi - ungase ubone ukugcwala ngaphansi kwezimbambo zakho.
- Ubuhlungu be-Bony
- Ukushisa noma ukutheleleka njalo
Ubani Osesengozini
Ngokuvamile i-Myelofibrosis ivela kubantu abangaphezu kweminyaka engama-50. Kungenzeka ezinganeni kodwa kungavamile kakhulu. Iziguli ezine-polycythemia vera noma i-thrombocythemia ebalulekile ingaqhubeka nokuthuthukisa imyelofibrosis.
Kungani Ipeni Lakho Landa?
I- spleen iyisitho se-hematopoietic, okusho ukuthi sinamandla okwenza amangqamuzana egazi. Esikhathini se-myelofibrosis, lapho umnkantsha wethambo unenkinga yokwenza amangqamuzana egazi, i-spleen ikhulisa ukuzama ukuthuthukisa ukukhiqizwa.
Izimbangela
I-myelofibrosis eyinhloko iyinhlobo engavamile yesifo somdlavuza wegazi (ingxenye yezifo ezingapheliyo eziphathelene nesifo se-myeloproliferative). Lokhu kubangelwa ukuguqulwa kwezakhi zofuzo. Ngalesi sikhathi asiqiniseki ukuthi yini eyenza ukuguqulwa kwemvelo kwenzeke.
I-Myelofibrosis ingabangelwa ezinye izimo futhi ibizwa ngokuthi i-myelofibrosis yesibili.
Lokhu kufaka:
- Ezinye izidakamizwa zegazi, ikakhulukazi i-megakaryocytic leukemia (uhlobo lwe -lemonemic acute leyemia ) noma i-myelodysplastic syndrome
- I-metastasis yomdlavuza ithambo
- Zenza izifo ezizenzakalelayo njenge-lupus, scleroderma
- Umfutho wegazi ophezulu we-pulmonary: Kulesi simo, kunomfutho wegazi ophakeme emithanjeni yegazi emaphashini.
- I-Hyperparathyroidism ehlotshaniswa nokuntuleka kwe-vitamin D
- I-grey platelet syndrome
- I-osteeodystrophy yama-renal: Lokhu kuyisifo se-mineral kanye ne-bone metabolism esenzeka ezigulini ezinesifo esingenasifo sezinso.
Ukuxilongwa
Okokuqala, ukwehla kwegazi kwehla kubonakala ekubaleni kwegazi okuphelele . Ngokuvamile, i-anemia kanye / noma i-thrombocytopenia ikhona. Umonakalo wamangqamuzana egazi ngokuvamile ungabonakala ku-blood smear ye-peripheral, isilayidi se-microscope yehla yegazi. Amaseli abomvu ajwayele ukuchazwa njengokubukeka njenge-teardrop.
Ukuxilongwa kokugcina kudinga umthambo we-bone umthambo, inqubo lapho kususwa khona ucezwana oluthile lomnyosa. Ngamabala akhethekile, ama-fibrous strings emnothweni wethambo angabonakala.
Ngesikhathi umsebenzi wokuhlola, udokotela wakho uzama ukuthola ukuthi yini ebangela i-myelofibrosis. Kuhlanganisiwe kulo msebenzi kuzoba ukuhlolwa kwezakhi zofuzo zokuguqulwa okuqondile okuthiwa i-JAK2, MPL, ne-CALR.
Ukwelapha
Ukwelashwa kuxhomeke ekubambeni okuyinhloko. Ukwelashwa kwe-myelofibrosis eyinhloko kunqunywe ingozi yokuqhubeka kwezifo nokuphila konke.
- Ukuqaphela: Uma ungenayo izimpawu ezibalulekile, ukulinda okulindile kungase kube ukhetho oluhle kakhulu lwezokwelapha.
- Ukuguqulwa : I-anemia kanye ne-thrombocytopenia ebonakala ku-myelofibrosis ingaba nzima futhi ingadinga ukumpontshelwa igazi njalo. Ukuvama kwamaphesenti kungancuma uma kudingwa ukwelashwa okukhulu.
- Ukufakelwa kabusha kwe-marrow cell (stem cell): Isinqumo sokufakelwa kufanele silinganiswe ngokucophelela kokubili izingozi nezinzuzo.
- I-Ruxolitinib: Lo muthi uvimbela i-JAK2 ingasetshenziswa kuziguli ezine-JAK2 zinguquko ezinempawu eziphawulekayo futhi azifanelekile ukufakelwa kwe-marrow bone.
- I-Splenectomy : Uma i-spleen inganciphisi ngobukhulu besifo, ingasuswa ngokuhlinzwa.
- I-Hydroxyurea: I- Hydroxyurea imithi yokwelapha ngomlomo ongasetshenziswa ukunciphisa usayizi we-spleen nezinye izimpawu.
- Eminye imithi efana ne-androgen ne-thalidomide
- Ukwelashwa kwezidakamizwa kungasetshenziselwa ukunciphisa ubukhulu bestile
Ukwelashwa kwe-myelofibrosis yesibili kuqondiswa imbangela eyimbangela. Ngakho-ke uma i-myelofibrosis ibangelwa umdlavuza onjenge-acute myeloid leukemia (AML), iphathwa nge-chemotherapy. Uma i-myelofibrosis iyingxenye yesifo esizimele, ukwelashwa kwalesi sifo kungase kuthuthukise igazi elibala.
Kungakhathaliseki ukuthi ukwelashwa, ithimba lakho lezokwelapha lizokuhamba kuzo zonke izinyathelo okudingayo ukuthatha ukulawula impilo yakho. Unganqikazi ukwabelana ngemicabango yakho, imibuzo, nemizwa nabo.