I-thrombocythemia ebalulekile

I-thrombocythemia ebalulekile (i-ET) yisimo lapho umnkantsha wethambo ukhiqiza khona amaplatelet amaningi kakhulu. Ama-platelet ayingxenye yegazi elisiza ekunqandeni igazi.

Imiphumela ye-ET ivela ekukhiqizweni ngokweqile kwama-platelets. Njengoba la ma-platelet engasebenzi ngokujwayelekile, amahlule egazi nokopha kuyizinkinga ezivamile. Ukungalashwa, i-ET iba yimbi ngokuhamba kwesikhathi.

I-ET iyingxenye yeqembu lezimo ezaziwa njengezinkinga ze-myeloproliferative. Ezinye zifaka:

• I-leukemia engapheli (umdlavuza oqala emnkantsheni wamathambo)
• I-Polycythemia vera (isifo semnkantsha esiholela ekwandeni okungavamile kwenani lamaseli egazi)
• I-myelofibrosis eyinhloko (ukuphazamiseka komnkantsha wamathambo lapho umnkantsha uthathelwa khona yizicubu ezibomvu)

Abantu abaningi abane-ET banokushintshwa kofuzo (i-JAK2, i-CALR, noma i-MPL).

I-ET ivame kakhulu kubantu asebekhulile. Kungabonakala nakubantu abasha, ikakhulukazi abesifazane abangaphansi kweminyaka engama-40.

Izimpawu zamahlule egazi zingafaka noma yikuphi okulandelayo:

• Ukuphathwa ikhanda (okuvame kakhulu)
• Ukulinganisa, ukubanda, noma ukuba luhlaza ezandleni nasezinyaweni
• Ukuzizwa unesiyezi noma unekhanda elikhanyayo
• Izinkinga zombono
• Imivimbo emincane (ukuhlaselwa yischemic okwedlulayo) noma isifo sohlangothi

Uma ukuphuma kwegazi kuyinkinga, izimpawu zingafaka noma yikuphi okulandelayo:

• Ukulimala okulula nokopha kwamakhala
• Ukopha okuphuma emathunjini, uhlelo lokuphefumula, umgudu wokuchama, noma isikhumba
• Ukopha ophuma ezinsinini
• Ukopha isikhathi eside kusuka ezinqubweni zokuhlinzwa noma ekususweni kwamazinyo

Isikhathi esiningi, i-ET itholakala ngokuhlolwa kwegazi okwenzelwe ezinye izinkinga zempilo ngaphambi kokuba kuvele izimpawu.

Umhlinzeki wakho wezokunakekelwa kwempilo angabona isibindi esikhulisiwe noma ubende ekuhlolweni komzimba. Ungaba nokugeleza kwegazi okungavamile ezinzwaneni noma ezinyaweni ezidala ukulimala kwesikhumba kulezi zindawo.

Ezinye izivivinyo zingafaka:

• I-bone marrow biopsy
• Qedela ukubalwa kwegazi (CBC)
• Ukuhlolwa kofuzo (ukubheka ushintsho kuhlobo lwe-JAK2, CALR, noma lwe-MPL)
• Izinga le-uric acid

Uma unezinkinga ezisongela impilo, ungahle ube nokwelashwa okubizwa ngeplatelet pheresis. Ngokushesha inciphisa amaplatelet egazini.

Isikhathi eside, imithi isetshenziselwa ukunciphisa inani leplatelet ukugwema izinkinga. Imithi ejwayelekile esetshenzisiwe ifaka phakathi i-hydroxyurea, i-interferon-alpha, noma i-anagrelide. Kwabanye abantu abane-JAK2 mutation, i-inhibitors ethize ye-JAK2 protein ingasetshenziswa.

Kubantu abasengozini enkulu yokuvala ama-clotting, i-aspirin ngomthamo ophansi (u-81 kuya ku-100 mg ngosuku) inganciphisa iziqephu zokujiya

Abantu abaningi abadingi ukwelashwa, kepha kufanele balandelwe eduze ngumhlinzeki wabo.

Imiphumela ingahluka. Iningi labantu lingahamba isikhathi eside ngaphandle kwezinkinga futhi libe nempilo ejwayelekile. Kubantu abambalwa, izinkinga ezivela ekuphumeni kwegazi nasezingxenyeni zegazi zingadala izinkinga ezinkulu.

Ezimweni ezingavamile, lesi sifo singashintsha sibe yi-acute leukemia noma i-myelofibrosis.

Izinkinga zingafaka:

• I-leukemia enamandla noma i-myelofibrosis
• Ukopha kakhulu (ukopha)
• Unhlangothi, isifo senhliziyo, noma amahlule egazini ezandleni noma ezinyaweni

Shayela umhlinzeki wakho uma:

• Unokuphuma kwegazi okungaqondakali okuqhubeka isikhathi eside kunalokho okufanele.
• Uyaqaphela ubuhlungu besifuba, ubuhlungu bomlenze, ukudideka, ubuthakathaka, ukuba ndikindiki, noma ezinye izimpawu ezintsha.

I-thrombocythemia eyinhloko; I-thrombocytosis ebalulekile

• Amaseli egazi

UMascarenhas J, Iancu-Rubin C, uKremyanskaya M, uNajfeld V, uHoffman R. Okubalulekile kwe-thrombocythemia. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 69.

I-Tefferi A. Polycythemia vera, i-thrombocythemia ebalulekile, ne-myelofibrosis eyinhloko. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 166.