I-Polycythemia vera yinkinga lapho umnkantsha wesithambo wenza amangqamuzana amaningi obomvu egazi, amangqamuzana egazi amhlophe, namaplatelet, okungaholela engozini enkulu yokweqa igazi.
Ukuba namaseli amaningi abomvu amaningi kakhulu isibonakaliso esivelele kunazo zonke se-polycythemia vera. Ukuguqulwa kwesigcawu se-JAK2, iphrotheni elihilelekile ekutheni lisayine ngaphakathi kweseli, litholakala kubantu abaningi abanalesi simo.
Ubani Othola Polycythemia Vera?
I-Polycythemia vera ingenzeka kunoma yikuphi ubudala kodwa ngokuvamile iyenzeka kamuva ekuphileni. Uma ubheka iminyaka yabo bonke abantu abanalesi simo, inamba ephakathi yobudala ekuxilongwa kuyoba yiminyaka engama-60, futhi ngokuvamile ayibonakali kubantu abangaphansi kweminyaka engama-40. Isimo se polycythemia vera sinabantu abakhulu kunabesifazane futhi iphakeme kakhulu emadodeni aneminyaka engu-70 kuya kweyengu-79.
Bangaki Abantu Abathintekayo?
Kunzima ukukusho ngokuqinisekile, ngoba umuntu angase abe nalesi simo isikhathi eside futhi angazi, kodwa ukulinganiselwa kungabantu oyedwa noma ababili abavela ku-100,000.
Ngokusho kwe-Incyte Corporation, inkampani eWilmington, eDelaware, esekelwe yinkampani ye-biopharmaceutical ekhethekile e-oncology, kunabantu abangaba ngu-25 000 e-United States abahlala ne-polycythemia vera futhi babhekwa njengalawuliwe ngoba bahlakulela ukungahambisani nokungahambisani nokuphikisana kwezidakamizwa, i-hydroxyurea .
I-Incyte ibambisana nezikhungo zezokwelapha ezingaba ngu-200 zomphakathi nezifundo ukuze ziqhube isifundo e-US esibizwa ngokuthi i-REVEAL, esanda kuqala ukubhalisa iziguli, futhi kulindeleke ukuthi ifake iziguli ezingu-2 000 ngaphansi kokuqondiswa udokotela ngokuphathwa okusebenzayo kwalesi sifo isikhathi iminyaka emithathu.
Ingabe I-Cancer Noma Isifo?
I-Polycythemia vera inezici ezithile ezifana nomdlavuza njengoba kuhilela ukuhlukana okungalawulwa kweseli elincane futhi akulona ukwelapha. Ukwazi lokhu, ukufunda ukuthi wena noma othandekayo unalesi sifo kungacindezeleka ngokucacile. Noma kunjalo, wazi ukuthi lesi simo singaphathwa ngokuphumelelayo isikhathi eside kakhulu.
I-National Cancer Institute ichaza i-polycythemia vera ngale ndlela elandelayo: "Isifo lapho kunamaseli abomvu amaningi kakhulu emnothweni wegazi kanye negazi, okwenza igazi likhube. Inombolo yamaseli amhlophe wegazi namaplatelets ingase futhi ikhule. Amangqamuzana egazi angeziwe angase aqoqe kulesi sitha bese enza ukuba ikhuliswe. Kungase kubangele izinkinga zokuphuma kwegazi futhi kwenziwe amafomu emigodini yegazi. "
Ngokusho kwe-Leukemia & Lymphoma Society, abantu abane polycythemia vera basengozini encane kakhulu kunabantu abaningi ekuthuthukiseni i-leukemia ngenxa yesifo kanye / noma ezinye zokwelapha izidakamizwa ezithile. Nakuba lesi simo esingapheli esingelapheki, khumbula ukuthi ngokuvamile singaphathwa ngokuphumelelayo isikhathi eside, isikhathi eside-futhi asifinyeleli isikhathi eside sokuphila. Ngaphezu kwalokho, izinkinga zingelashwa futhi zivinjelwe ngokuphathwa kwezokwelapha.
Ingabe Ithuthuka?
Yebo, kodwa imininingwane mayelana nengozi yokuqhubeka isacwaningwa. Nakuba abantu bangase bangabi nezimpawu iminyaka eminingi, i-polycythemia vera ingaholela ekuthuthukiseni kwezinqwaba zampawu nezimpawu, kuhlanganise ukukhathala, ukuhlushwa, ukujula ebusuku, ubuhlungu emathanjeni, umkhuhlane kanye nokulahleka kwesisindo. Amaphesenti angaba ngu-30 kuya kuma-40 abantu abaphethe i-polycythemia vera banesipula esikhulisiwe. Kwamanye abantu, kungabangela izimpawu ezikhungathekisayo nezinkinga zomzimba. Umthwalo walesi sifo usacwaningwa ngentshiseko.
Utholakala kanjani?
Isivivinyo esibizwa ngokuthi i-hematocrit concentration sisetshenziswa kokubili ukuhlola i-polycythemia vera nokukala impendulo yomuntu ekwelapheni.
I-Hematocrit iyinani lamaseli abomvu obomvu ngegazi, futhi ngokuvamile linikezwa njengephesenti noma ukwanda kwe-hemoglobin concentration in the blood.
Kubantu abanempilo, izingxube ze-hematocrit eziphakathi kwamaphesenti angu-36 kuya kwangu-46 kwabesifazane namaphesenti angu-42 kuya kwangu-52 kumadoda. Olunye ulwazi olukwazi ukuqoqwa ekuhlolweni kwegazi lubuye luzuze ekuxilongweni kwegazi, kufaka phakathi ukuguqulwa kwemvelo-ukuguqulwa kwe-JAK2-kumaseli egazi. Nakuba kungadingeki ukuba kutholakale ukuxilongwa, abanye abantu bangabuye babe nokuhlaziywa komnkantsha wamathambo njengengxenye yokuphinda nokuhlola kwabo.
Ukuphathwa Kanjani?
Ngokusho kwe -Leukemia & Lymphoma Society , i-phlebotomy, noma ukususwa kwegazi kusuka emthanjeni, kuyindlela ejwayelekile yokuqala yokwelapha iziguli eziningi. Lokhu kunganciphisa ukuhlushwa kwe-hematocrit, okuvame ukuphumela ekwenzeni ngcono izimpawu ezithile ezifana nokukhanda ikhanda, ukukhala ezindlebeni, nokuzondwa.
Ukwelapha izidakamizwa kungase kuhlanganise nama-agent anganciphisa iseli elibomvu noma ama-platelet concentrations-amakhemikhali abizwa ngokuthi ama-myelosuppressive agents. I-myelosuppressive agent ejwayelekile kakhulu ye-polycythemia vera i-hydroxyurea, enikezwa ngomlomo. Imiphumela emibi kakhulu evame ukufaka ukukhwehlela noma ukuphazamiseka, umkhuhlane noma izikhukhula, ubuhlungu obuphansi noma ubuhlungu obuhlangene, nokuhlanza okubuhlungu noma okunzima.
Mayelana neJakafi yePolythemia Vera
I-Ruxolitinib (i-Jakafi) imithi yokwelapha esetshenziselwa ukuphatha abantu abane-polycythemia vera asebethathe kakade i-hydroxyurea futhi ayizange isebenze kahle noma ayikwazanga ukuyibekezelela. I-Jakafi nayo isetshenziselwa ukuphatha izinhlobo ezithile ze- myelofibrosis -ukushaywa komnyosa wethambo.
Ngokusho kwe-FDA, uJakafi usebenza ngokuvimbela ama-enzyme okuthiwa uJanus Associated Kinase (JAK) 1 no-2 ahilelekile ekulawuleni igazi nokusebenza kwe-immunological. Ukuvunyelwa kwezidakamizwa zokwelapha i-polycythemia vera kuzokusiza ukunciphisa ukutholakala kwe-spleen-splenomegaly ekhulisiwe-nesidingo se-phlebotomy, inqubo yokukhipha igazi elingaphezu komzimba.
Imiphumela emibi kakhulu ehambisana nokusetshenziswa kukaJakafi kubathintekayo nge-polycythemia vera ekuhloleni izivivinyo ezifanelekile kwakuyizinombolo ezibomvu zamangqamuzana egazi (i-anemia) kanye nokubala okuphansi kweplatelet (thrombocytopenia). Imiphumela ejwayelekile ehlobene ne-non-blood ehlobene negazi kwakuyizizungu, ukuqothulwa, nokukhwabanisa.