Yintoni Polycythemia Vera, isifo, iimpawu kunye nonyango

Umxholo

• Zithini iimpawu?
• Uxilongo lwenziwa njani
• Iingxaki zepolycythemia vera
• 1. Ukwenziwa kwamahlwili egazi
• 2. I-Splenomegaly
• 3. Ukuvela kwezinye izifo
• Ungazithintela njani iingxaki
• Izizathu ezinokubangela
• Unyango lwenziwa njani

I-Polycythemia Vera sisifo se-myeloproliferative seseli zehematopoietic, esibonakala ngokwanda okungalawulekiyo kweeseli ezibomvu zegazi, iiseli ezimhlophe zegazi kunye neeplatelets.

Ukwanda kwezi seli, ngakumbi kwiiseli ezibomvu zegazi, kwenza igazi lijiye, nto leyo enokuthi ikhokelele kwezinye iingxaki ezinje ngokunyusa ipeni kunye nokunyuka kwamahlwili egazi, oko ke kwandisa umngcipheko we-thrombosis, isifo sentliziyo okanye ukubetha okanye kubangele nezinye izifo i-myeloid leukemia okanye i-myelofibrosis.

Unyango lubandakanya ukwenza inkqubo ebizwa ngokuba yi-phlebotomy kunye nokuhambisa amayeza anceda ukulawula inani leeseli ezisegazini.

Zithini iimpawu?

Inani eliphezulu leeseli ezibomvu zegazi libangela ukonyuka kwehemoglobin kunye ne-viscosity yegazi, enokubangela iimpawu ze-neurological ezifana ne-vertigo, intloko ebuhlungu, ukonyuka koxinzelelo lwegazi, utshintsho olubonakalayo kunye neengozi zeschemic ezidlulayo.

Ukongeza, abantu abanesi sifo bahlala befumana ukurhawuzelela ngokubanzi, ngakumbi emva kweshawa eshushu, ubuthathaka, ukuthoba umzimba, ukudinwa, ukungaboni kakuhle, ukubila okugqithisileyo, ukudumba ngokudibeneyo, ukuphefumla okufutshane kunye nokuba ndindisholo, ukurhawuzelela, ukutshisa okanye ubuthathaka kumalungu.

Uxilongo lwenziwa njani

Ukuze kufunyaniswe isifo, kufuneka kwenziwe uvavanyo lwegazi, oluthi kubantu abanePolycythemia Vera, lubonise ukwanda kwenani leeseli ezibomvu zegazi, kwaye kwezinye iimeko, ukwanda kweeseli ezimhlophe zegazi kunye neeplatelets, amanqanaba aphezulu ehemoglobin kunye namanqanaba asezantsi erythropoietin.

Ukongeza, umnqweno wethambo okanye i-biopsy inokwenziwa ukuze ufumane isampulu eza kuhlaziywa kamva.

Iingxaki zepolycythemia vera

Kukho iimeko ezithile zabantu abanePolycythemia Vera abangazibonakalisi iimpawu kunye neempawu, nangona kunjalo, ezinye iimeko zinokubangela iingxaki ezinzulu ngakumbi:

1. Ukwenziwa kwamahlwili egazi

Ukwanda kobukhulu begazi kunye nokuhla kokuhamba nokutshintsha kwenani leeplatelets, kunokubangela ukwenziwa kwamahlwili egazi, anokukhokelela kwisifo sentliziyo, ukubetha, ukubola kwemiphunga okanye i-thrombosis. Funda ngakumbi malunga nesifo sentliziyo.

2. I-Splenomegaly

Udakada lunceda umzimba ukuba ulwe nosulelo kwaye luncede ekupheliseni iiseli zegazi ezonakeleyo. Ukwanda kwenani leeseli ezibomvu zegazi okanye ezinye iiseli zegazi, kwenza ukuba udakada asebenze nzima kunesiqhelo, kukhokelela ekwandeni kobukhulu. Bona ngakumbi malunga ne-splenomegaly.

3. Ukuvela kwezinye izifo

Nangona inqabile, iPolycythemia Vera inokubangela ezinye izifo ezinzulu, ezinje nge-myelofibrosis, myelodysplastic syndrome okanye i-leukemia ebukhali. Ngamanye amaxesha umongo wethambo unokukhulisa i-fibrosis kunye ne-hypocellularity.

Ungazithintela njani iingxaki

Ukuthintela iingxaki, ukongeza ekucetyisweni ukuba ulandele unyango ngokuchanekileyo, kubalulekile ukwamkela indlela yokuphila esempilweni, ukuzilolonga rhoqo, okuphucula ukujikeleza kwegazi kunye nokunciphisa umngcipheko wamahlwili egazi. Ukutshaya kufuneka kuphetshwe, njengoko kusonyusa umngcipheko wokuhlaselwa yintliziyo kunye nokufa icala.

Ukongeza, ulusu kufuneka luphathwe kakuhle, ukunciphisa ukurhawuzelela, ukuhlamba ngamanzi afudumeleyo, usebenzisa ijeli yeshawari ethambileyo kunye nokhilimu we-hypoallergenic kunye nokuthintela amaqondo obushushu agqithisileyo, anokwenza mandundu ukujikeleza kwegazi. Ngenxa yoku, umntu makathintele ukubekwa elangeni ngamaxesha ashushu emini kwaye akhusele umzimba ekufumaneni imeko yemozulu ebanda kakhulu.

Izizathu ezinokubangela

IPolycythemia Vera yenzeka xa ijini yeJAK2 iguquka, ebangela iingxaki kwimveliso yeeseli zegazi. Esi sifo sinqabile, esenzeka malunga ne-2 kubo bonke abantu abayi-100,000, ngokuqhelekileyo ngaphezu kweminyaka engama-60.

Ngokubanzi, umzimba ophilayo ulawula isixa semveliso nganye yeendidi ezintathu zeeseli zegazi: ezibomvu, iiseli ezimhlophe zegazi kunye neeplatelets, kodwa kwiPolycythemia Vera, kukho imveliso ebaxiweyo yoluhlobo olunye okanye nangaphezulu lweeseli zegazi.

Unyango lwenziwa njani

I-Polycythemia vera sisifo esinganyangekiyo esinganyangekiyo kwaye unyango luquka ukunciphisa iiseli zegazi ezigqithileyo, kwaye kwezinye iimeko kunokunciphisa umngcipheko weengxaki:

Unyango lwephlebotomy: Obu buchule buquka ukukhupha igazi kwimithambo, nto leyo edla ngokuba lolona nyango lokuqala kubantu abanesi sifo. Le nkqubo inciphisa inani leeseli ezibomvu zegazi, ngelixa inciphisa umthamo wegazi.

I-AspirinUgqirha unokuyalela i-aspirin ngedosi esezantsi, phakathi kwe-100 kunye ne-150 mg, ukunciphisa umngcipheko wamahlwili egazi.

Amayeza okunciphisa iiseli zegazi: Ukuba i-phlebotomy ayonelanga ukuba unyango lusebenze, kunokuba yimfuneko ukuthatha amayeza anje ngala:

• IHydroxyurea, enokunciphisa ukuveliswa kweeseli zegazi kumongo wethambo;
• I-Alpha interferon, evuselela amajoni omzimba ukulwa nokuveliswa kakhulu kweeseli zegazi, kubantu abangaphenduli kakuhle kwi-hydroxyurea;
• I-Ruxolitinib, enceda amajoni omzimba ukutshabalalisa iiseli zethumba kwaye inokuphucula iimpawu;
• Amayeza ukunciphisa ukurhawuzelela, ezifana antihistamines.

Ukuba ukurhawuzelela kuya kusiba nzima kakhulu, kunokuba yimfuneko ukuba kunyangwe ukukhanya kwe-ultraviolet okanye ukusebenzisa amayeza afana neparoxetine okanye i-fluoxetine.