Isimo esivamile esivezwa ngamapuleletti egazi ngokweqile
I-thrombocythemia ebalulekile (ET) yisifo esingavamile lapho umnkantsha wethambo uveza amaplatelet amaningi kakhulu. I-ET iyingxenye yesigaba sezifo eziyaziwa ngokuthi izidakamizwa ze-myeloproliferative, iqembu lezinkinga ezibhekiswe ekukhiqizeni okwenyuka kwehlobo oluthile lwegazi.
Ama-platelets yiwona amangqamuzana aphethwe ukugaya igazi okwakunamathele ndawonye endaweni yokuqhwala noma ukulimala ukuze ayeke ukuphuma.
Kubantu abane-ET, ukutholakala kwamaplatelets amaningi ngokweqile kungabangela inkinga, kubangele ukwakheka okungavamile kwamacenge ngaphakathi kwesitsha segazi (isimo esibizwa ngokuthi i- thrombosis ).
Nakuba imbangela ethile ye-ET engaziwa, cishe amaphesenti angu-40 kuya kwangu-50 abantu abane-disorder aguquguquka ngokwezakhi ezibizwa ngokuthi i-JAK2 kinase. I-ET iyisifo esingavamile kakhulu, esithinta abantu abangaphansi kuka-3 kwabangu-100,000 ngonyaka. Lithinta abesifazane nabesilisa bazo zonke izinhlanga kodwa kubonakala kubonakala kubantu abadala abangaphezu kuka-60.
Izimpawu Ze-Essential Thrombocythemia
Abantu abane-thrombocythemia ebalulekile bavame ukutholakala ngemva kokuthuthukisa izimpawu ezithile ezihlobene ne- clot yegazi . Kuye lapho i-clot ikhona khona, izimpawu zingase zifake:
- Izinwele
- Ukuzivocavoca noma ubukhulu obunzima
- Ukungabi namandla
- Ukuphelelwa amandla
- Izinhlungu zesifuba
- Izinguquko embonweni zishintsha
- Ubumbulu, ubomvu, ukubetha, noma ukuzwa okuvuthayo ezandleni nasezinyaweni
Ngokuvamile, ukuphuma okungavamile kungakhula njengomphumela we-ET.
Ngenkathi ukubala okuphansi kweplatelet kungabangela ukuphuma kwegazi ngenxa yokuntuleka kwe-clotting, amaplatelets amaningi kakhulu angaba nomphumela ofanayo namaprotheni adingekayo ukuwahlanganisa ndawonye (okuthiwa i-von Willebrand factor) angasakazeka kakhulu ukuze asebenze. Uma kwenzeka lokhu, ukulimaza okungavamile, ukuphuza amanzi, ukuphuma kwamagciwane noma izinsini, noma igazi esitokisini kungenzeka.
Ukwakhiwa kwezindwangu zegazi ngezinye izikhathi kungabi sína futhi kungabangela ukuhlaselwa yinhliziyo, ukushaywa yisifo, ukuhlaselwa kwe-ischemic okudlulayo ("i-mini-stroke"). I- spleen ekhulisiwe ibonakala nakumaphesenti angu-20 amacala ngenxa yokuvinjelwa kwegazi.
Ukuthola i-Thrombocythemia ebalulekile
I-thrombocythemia ebalulekile ivame ukubonakala ngesikhathi sokuhlolwa kwegazi njalo kubantu abangenayo izimpawu noma izimpawu ezingacacile, ezingenakubalwa (njengokukhathala noma ikhanda). Noma yiliphi inani legazi lamaplatelets angaphezu kuka-450,000 nge-microliter ngayinye kubhekwa njengefulegi elibomvu. Labo abangaphezu kwesigidi nge-microliter ngayinye bahlotshaniswa nengozi ephezulu yokuchiza okungavamile noma ukuphuma kwegazi.
Ukuhlola ngokomzimba kungabonisa ukukhuliswa kwesipula okubonakala ngobuhlungu noma ukugcwala kwesisu esisenhla esingasese esingasakazeka ehlombe lesokunxele. Ukuhlolwa kwe-Genetic kungenziwa futhi ukuthola ukuguqulwa kwe-JAK2.
Ukuxilongwa kwe-ET ngokuyinhloko kungabandakanyi, okusho ukuthi noma yiyiphi enye imbangela yokubala kweplatelet ephakeme kufanele ikhishwe kuqala ukuze ihlolwe ngokucacile. Ezinye izimo ezihlotshaniswa ne-platelet high count zihlanganisa i- polycythemia vera , i-myeloid leukemia engapheli , ne- myelofibrosis .
Ukwelapha i-Thrombocythemia ebalulekile
Ukwelashwa kwe-thrombocythemia ebalulekile kuncike ekutheni ukuphakama kweplatelet kuphakanyiswe kanjani kanye nokuthi kungenzeka yini ukuhlupheka.
Akubona bonke abantu abane-ET abadinga ukwelashwa. Abanye badinga ukuhlolisiswa ukuqinisekisa ukuthi isimo asibi nakakhulu.
Uma kudingeka, ukwelashwa kungabandakanya i-aspirin yansuku zonke kulabo ababhekwa njengengozi ephansi. Kulabo abasengozini enkulu (ngokususelwa ekugugeni, umlando wezokwelapha, noma izinto zokuphila ezifana nokubhema noma ukukhuluphala) bangase bazuze kwizidakamizwa ezifana ne-hydroxyurea, i-anagrelide, noma i-interferon alpha, konke okukwazi ukunciphisa izinombolo zeplatelet.
Esimweni esiphuthumayo, plateletpheresis (inqubo lapho igazi lihlukaniswa khona izingxenye zalo) lingenziwa ukuze kunciphise ngokushesha inani leplatelet.
> Umthombo:
> Bleeker, J. noHogan, W. "Thrombocytosis: Ukuhlola Ukuhlola, Ukuqapha Isifo Sengculaza, kanye Nezinhlelo Zokuphatha Ezibhekele Ingozi." I-Thrombosis. 2011; Isihloko ID 536062.