I-Thrombocythemia ebalulekileyo kunye nokuvalwa kwegazi okungavumelekanga

Iimeko eziqhelekileyo ezibonakaliswe ngeeplatelets zegazi

I-thrombocythemia (ET) ibalulekileyo yintlupheko engaqhelekanga apho umongo wethambo uvelisa iiplatelet ezininzi. I-ANDI inxalenye yesigaba sezifo ezibizwa ngokuba yi-myeloproliferative disorders, iqela leengxaki ezibonakaliswe ngokuveliswa kwemveliso ethile yelungu legazi.

Iiplateslet ziseli lijongene nokuqhawula kwegazi ezithintela ndawonye kwisiza sokuqhwala okanye ukulimala ukwenzela ukuyeka ukuphuma kwegazi.

Kubantu abane-ET, ubukho beeplatelets ezigqithisileyo bunokuba yingxaki, kubangele ukubunjwa okungavumelekanga kwinqanawa ngaphakathi kwegazi (imeko eyaziwa ngokuba yi- thrombosis ).

Nangona isizathu esithile se-ET singaziwa, malunga nama-40 ukuya kuma-50 ekhulwini abantu abanesifo banokuguqulwa kofuzo olubizwa ngokuba yi-JAK2 kinase. ET isisifo esingavamile kakhulu, esichaphazela ngaphantsi kwethathu kubantu abayi-100,000 ngonyaka. Ichaphazela abantu besetyhini kunye namadoda abo bonke abavela kwimimandla kodwa kubonakala kubonakala kubantu abadala abangaphezu kwama-60.

Iimpawu ze-Essential Thrombocythemia

Abantu abane-thrombocythemia ebalulekileyo badla ngokuxilongwa emva kokuvelisa iimpawu ezithile eziphathelene ne- blood clot . Ngokuxhomekeka apho i-clot ikhona khona, iimpawu zingabandakanya:

Ngokuqhelekanga, ukuphuma kwamanzi okungavamile kungakhula ngenxa ye-ET.

Nangona ukubalwa kweplatelet ephantsi kunokubangela ukuphuma ngegazi ngenxa yokungahambi kakuhle, iiplatelet ezigqithiseleyo zinokuchaphazeleka okufanayo kunye neeprotheni ezifunekayo ukuzinamathela ndawonye (ezibizwa ngokuba yi-von Willebrand factor) zingasasazeka kakhulu ukuba ziphumelele. Xa oko kwenzeka, ukutyumla okungavumelekanga, ukuphuza i-nosebleeds, ukuphuma kumlonyeni okanye kwizinsini, okanye igazi kwi-stool .

Ukwakhiwa kwamacwecwe egazi ngamanye amaxesha kunokuba lukhulu kwaye kungakhokelela ekuhlaselweni kwentliziyo, ukuphazamiseka, ukuhlaselwa kwe-ischemic (i "mini-stroke"). I- spleen ekwandisiweyo ibonakala nakwi-20 ekhulwini yamatyala ngenxa yokuphazamiseka kwegazi.

Ukuchonga i-Essential Thrombocythemia

I-thrombocythemia ebalulekileyo ibonakala rhoqo ngexesha lokuhlolwa kwegazi rhoqo kubantu abangenazo iimpawu okanye iimpawu ezingacacanga, ezingabonakaliyo (ezifana nokukhathala okanye intloko). Naliphi inani legazi lama-platelet angaphezu kwama-450,000 nganye nge-microliter ithathwa njengeflegi ebomvu. Abo bangaphezulu kwesigidi nganye nge-microliter banxulumene nomngcipheko ophezulu wokuqhwala okanye ukuphuma.

Uvavanyo lomzimba lungabonakalisa ukwandiswa kwesipen ebonakaliswe ngentlungu okanye ukuzaliswa kwesisu esasentla esingasasazeka kwiphepha lasekhohlo. Ukuvavanya kwe-Genetic kungenziwa kwakhona ukufumana ukuguqulwa kweJAK2.

Ukuxilongwa kwe-ET kubaluleke kakhulu ukuxhomekeka, oku kuthetha ukuba nayiphi na isizathu esithile sokubala kweplatelet ephakamileyo kufuneka ikhutshwe kuqala ukuze kwenziwe ukuxilongwa ngokucacileyo. Ezinye iimeko ezinxulumene nomlinganiselo weplatelet ephezulu ziquka i- polycythemia vera , i-leukemia engapheliyo kunye ne- myelofibrosis .

UkuPhatha i-Thrombocythemia ebalulekileyo

Unyango lwe-thrombocythemia ebalulekileyo luxhomekeka ngokukodwa malunga nendlela ephakamileyo yokubala kweplatelet kunye nokufumaneka kweengxaki.

Akunabo bonke abantu abane-ET abafuna unyango. Abanye bafuna nje ukubekwa iliso ukuqinisekisa ukuba imeko ayibubi.

Ukuba kuyimfuneko, unyango lunokubandakanya i-aspirin yansuku zonke kulabo bacingelwa ukuba basengozini ephantsi. Kwabo abasemngciphekweni ophezulu (ngokususelwa kwiminyaka yobudala, imbali yonyango, okanye izinto zokuphila ezifana nokutshaya okanye ukukhuluphala) zinganceda kwizidakamizwa ezifana ne-hydroxyurea, i-anagrelide, okanye i-alphaferre alpha, zonke ezikwazi ukunciphisa amanani eeplatelet.

Kwimeko engxamisekileyo, iplletletpheresis (inkqubo apho igazi lihlukaniswe khona kumacandelo alo) lingaqhutywa ukwenzela ukunciphisa ngokukhawuleza inani leplatelet.

> Umthombo:

> UBleeker, J. kunye noHogan, W. "iThrombocytosis: UkuVavanya ukuHlola, ukuCwangciswa kweNgcipheko yeThrombotic, kunye neCandelo lokuPhathwa koMngcipheko. Thrombosis. 2011; I-ID ye-ID 536062.