I-thrombocythemia ebalulekileyo

I-thrombocythemia ebalulekileyo (ET) yimeko apho umongo wethambo uvelisa iiplatelets ezininzi. Iiplatelets ziyinxalenye yegazi encedisa ekunqandeni igazi.

Iziphumo ze-ET ezivela kukuveliswa kakhulu kweeplatelets. Njengoko ezi platelet zingasebenzi ngesiqhelo, amahlwili egazi kunye nokopha ziingxaki eziqhelekileyo. Ukunganyangwa, i-ET iba mandundu ngokuhamba kwexesha.

I-ET yinxalenye yeqela leemeko ezaziwa ngokuba ziingxaki ze-myeloproliferative. Ezinye zibandakanya:

• I-leukemia engapheliyo (umhlaza oqala kumongo wethambo)
• IPolycythemia vera (isifo somongo wethambo esikhokelela kukonyuka okungaqhelekanga kwinani leeseli zegazi)
• I-myelofibrosis yaseprayimari (ukuphazamiseka komongo wethambo apho umongo uthatyathelwa indawo sisihlunu esibomvu)

Abantu abaninzi abane-ET banokutshintsha kwemfuza (JAK2, CALR, okanye MPL).

I-ET ixhaphake kakhulu kubantu abadala. Ingabonakala nakubantu abancinci, ngakumbi abasetyhini abangaphantsi kweminyaka engama-40.

Iimpawu zokuqina kwegazi zinokubandakanya nayiphi na kwezi zinto zilandelayo:

• Intloko (ixhaphake kakhulu)
• Ukulinganisa, ukubanda, okanye ukuba luhlaza ezandleni nasezinyaweni
• Ukuziva unesiyezi okanye unentloko elula
• Iingxaki zombono
• Imivumbo emincinci (ukuhlaselwa okwenzeka okwexeshana) okanye ukubetha

Ukuba ukuphuma kwegazi kuyingxaki, iimpawu zingabandakanya nayiphi na kwezi zilandelayo:

• Ukutyumka ngokulula kunye nokuphuma kweempumlo
• Ukopha kuphetshana lesisu, inkqubo yokuphefumla, umchamo, okanye ulusu
• Ukopha kwiintsini
• Ukopha ixesha elide kwiinkqubo zotyando okanye ukususwa kwamazinyo

Uninzi lwexesha, i-ET ifumaneka kuvavanyo lwegazi olwenziwe kwezinye iingxaki zempilo ngaphambi kokuba kuvele iimpawu.

Umboneleli wakho wezempilo unokuqaphela isibindi esandisiweyo okanye udakada kuvavanyo lomzimba. Unokuba nokuhamba gazi ngokungaqhelekanga ezinzwaneni okanye ezinyaweni ezibangela ukonakala kwezi ndawo.

Olunye uvavanyo lunokubandakanya:

• Umongo wethambo
• Gcwalisa ubalo lwegazi (CBC)
• Uvavanyo lwe-Genetic (ukukhangela utshintsho kwiJAK2, CALR, okanye kwi-MPL gene)
• Inqanaba leUric acid

Ukuba unengxaki yokusongela ubomi, unokufumana unyango olubizwa ngokuba yi-platelet pheresis. Ngokukhawuleza kunciphisa iiplatelets egazini.

Ixesha elide, amayeza asetyenziselwa ukunciphisa ukubalwa kweplatelet ukunqanda iingxaki. Amayeza aqhelekileyo asetyenziswayo afaka ihydroxyurea, interferon-alpha, okanye anagrelide. Abanye abantu abanokutshintsha kweJAK2, inhibitors ezithile zeprotein yeJAK2 zinokusetyenziswa.

Kubantu abasemngciphekweni omkhulu wokujiya, iasprini ngedosi ephantsi (i-81 ukuya kwi-100 mg ngemini) inokunciphisa iziqendu zokuvala.

Abantu abaninzi abadingi nyango, kodwa kufuneka balandelwe kufutshane ngumboneleli wabo.

Iziphumo zinokwahluka. Uninzi lwabantu lunokuhamba ixesha elide ngaphandle kweengxaki kwaye lube nobomi obuqhelekileyo. Inani elincinci labantu, iingxaki ezivela ekuphumeni kwegazi kunye neengqungquthela zegazi zingabangela iingxaki ezinzulu.

Kwiimeko ezinqabe kakhulu, esi sifo sinokutshintsha sibe yi-leukemia okanye i-myelofibrosis ebukhali.

Iingxaki zinokubandakanya:

• Leukemia Acute okanye myelofibrosis
• Ukopha kakhulu (ukopha)
• Stroke, isifo sentliziyo, okanye amahlwili egazi ezandleni nasezinyaweni

Fowunela umnikezeli wakho ukuba:

• Ukopha okungachazwanga okuqhubeka ixesha elide kunokuba kufanelekile.
• Uqaphela iintlungu zesifuba, iintlungu zomlenze, ukudideka, ubuthathaka, ukuba ndindisholo, okanye ezinye iimpawu ezintsha.

Thrombocythemia yamabanga aphantsi; I-thrombocytosis ebalulekileyo

• Iiseli zegazi

IMascarenhas J, Iancu-Rubin C, Kremyanskaya M, Najfeld V, Hoffman R. Kubalulekile i-thrombocythemia. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 69.

I-Tefferi A. Polycythemia vera, i-thrombocythemia ebalulekileyo, kunye ne-myelofibrosis ephambili. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Umhla wama-25. IPhiladelphia, PA: Elsevier Saunders; I-2016: isahluko 166.