I-thrombotic thrombocytopenic purpura

I-Thrombotic thrombocytopenic purpura (TTP) yisifo segazi lapho amaflatelet clumps akha khona emithanjeni yegazi emincane. Lokhu kuholela ekubalweni kweplatelet ephansi (i-thrombocytopenia).

Lesi sifo singadalwa yizinkinga nge-enzyme (uhlobo lwamaprotheni) olubandakanyeka ekunqandeni igazi. Le enzyme ibizwa nge-ADAMTS13. Ukungabikho kwale enzyme kubangela ukugoqana kweplatelet. Ama-platelet ayingxenye yegazi elisiza ekunqandeni igazi.

Njengoba ama-platelet ehlangana, amaplatelet ambalwa atholakalayo egazini kwezinye izingxenye zomzimba ukusiza ekunqandeni. Lokhu kungaholela ekopheni ngaphansi kwesikhumba.

Kwezinye izimo, lesi sifo sidluliselwa ngemindeni (ezuzwe njengefa). Kulezi zimo, abantu bazalwa benamazinga aphansi ngokwemvelo ale enzyme.

Lesi simo futhi singabangelwa:

• Umdlavuza
• Ukwelashwa ngamakhemikhali
• Ukufakelwa kabusha kwe-stem cell ye-hematopoietic
• Ukutheleleka nge-HIV
• Ukwelashwa kokufaka esikhundleni seHormone nama-estrogens
• Imithi (kufaka phakathi i-ticlopidine, i-clopidogrel, i-quinine, ne-cyclosporine A)

Izimpawu zingafaka noma yikuphi okulandelayo:

• Ukopha esikhunjeni noma kulwelwesi lwamafinyila
• Ukudideka
• Ukukhathala, ubuthakathaka
• Imfiva
• Ubuhlungu bekhanda
• Umbala wesikhumba okhanyayo noma umbala wesikhumba ophuzi
• Ukuphelelwa umoya
• Ukushaya kwenhliziyo okusheshayo (ukushaya okungaphezu kuka-100 ngomzuzu)

Izivivinyo ezinga-odwa zifaka:

• Ileveli yomsebenzi engu-ADAMTS 13
• I-Bilirubin
• Ukugcotshwa kwegazi
• Qedela ukubalwa kwegazi (CBC)
• Izinga le-Creatinine
• Izinga le-Lactate dehydrogenase (LDH)
• Ukubalwa kweplatelet
• Ukuhlolwa komchamo
• I-Haptoglobin
• Ukuhlolwa kwama-Coombs

Ungaba nokwelashwa okubizwa nge-plasma exchange. Isusa i-plasma yakho engajwayelekile bese iyifaka esikhundleni se-plasma ejwayelekile evela kumnikeli onempilo. I-Plasma yingxenye yegazi equkethe uketshezi lwegazi nama-platelets. Ukushintshaniswa kwe-Plasma nakho kungena esikhundleni se-enzyme elahlekile.

Inqubo yenziwa ngale ndlela elandelayo:

• Okokuqala, udonse igazi lakho sengathi unikela ngegazi.
• Njengoba igazi lidluliselwa emshinini ohlukanisa igazi libe izingxenye zalo ezihlukene, kukhishwa i-plasma engajwayelekile bese kugcinwa amaseli akho egazi.
• Amaseli akho egazi abe esehlanganiswa ne-plasma ejwayelekile evela kumnikeli, bese ebuyiselwa kuwe.

Lokhu kwelashwa kuyaphindwa nsuku zonke kuze kube ukuhlolwa kwegazi kukhombisa ukuthuthuka.

Abantu abangaphenduli kulokhu kwelashwa noma isimo sabo esivame ukubuya bangadinga:

• Yenza ukuhlinzwa ukuze ususe ubende lwabo
• Thola imithi ecindezela amasosha omzimba, njenge-steroids noma i-rituximab

Iningi labantu abashintshaniswa ne-plasma lilulama ngokuphelele. Kodwa abanye abantu babulawa yilesi sifo, ikakhulukazi uma singatholakali ngokushesha. Kubantu abangalulami, lesi simo singaba sesikhathini eside (esingapheli).

Izinkinga zingafaka:

• Ukuhluleka kwezinso
• Inani eliphansi leplatelet (thrombocytopenia)
• Ukubalwa kwamangqamuzana egazi abomvu aphansi (kubangelwa ukonakala kwangaphambi kwesikhathi kwamaseli abomvu egazi)
• Izinkinga zesistimu yezinzwa
• Ukopha kakhulu (ukopha)
• Unhlangothi

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma unopha okungachazeki.

Ngoba imbangela ayaziwa, ayikho indlela eyaziwayo yokunqanda lesi simo.

TTP

• Amaseli egazi

Abrams CS. I-Thrombocytopenia. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-25. IPhiladelphia, PA: Elsevier Saunders; 2016: isahluko 172.

Iwebhusayithi yeNational Heart, Lung, neGazi Institute. I-thrombotic thrombocytopenic purpura. www.nhlbi.nih.gov/health-topics/thrombotic-thrombocytopenic-purpura. Kufinyelelwe ngoMashi 1, 2019.

USchneidewend R, u-Epperla N, uFriedman KD. I-thrombotic thrombocytopenic purpura kanye ne-hemolytic uremic syndrome. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 134.