I-Thrombotic Thrombocytopenic Purpura: Kuyini, Izimbangela Nokwelapha

-Delile

• Izimbangela ze-PTT
• Izimpawu nezimpawu eziyinhloko
• Ukuxilongwa kwenziwa kanjani
• Ukwelashwa kwe-PTT

I-Thrombotic thrombocytopenic purpura, noma i-PTT, isifo esingajwayelekile kodwa esibulalayo se-hematological esibonakala ngokwakhiwa kwe-thrombi encane emithanjeni yegazi futhi sivame kakhulu kubantu abaphakathi kweminyaka engama-20 nengama-40 ubudala.

Ku-PTT kukhona ukwehla okuphawulekayo kwenani lama-platelet, ngaphezu komkhuhlane futhi, ezimweni eziningi, ukukhubazeka kwezinzwa ngenxa yokuguqulwa kokugeleza kwegazi kuya ebuchosheni ngenxa yamahlule.

Ukuxilongwa kwe-PTT kwenziwa ngudokotela wezifo zegazi noma udokotela ojwayelekile ngokuya ngezimpawu nomphumela wesibalo segazi esiphelele kanye ne-smear yegazi kanti ukwelashwa kufanele kuqalwe ngemuva nje kwalokho, ngoba lesi sifo siyabulala cishe kuma-95% uma singelashwa.

Izimbangela ze-PTT

I-thrombotic thrombocytopenic purpura ibangelwa kakhulu ukuntuleka noma ukuguqulwa kofuzo kwe-enzyme, i-ADAMTS 13, enesibopho sokwenza ama-molecule we-von Willebrand factor abe mancane, futhi athande umsebenzi wawo. Isici se-von Willebrand sikhona kuma-platelet futhi sinesibopho sokukhuthaza ukunamathela kweplatelet ku-endothelium, kunciphise futhi kumise ukopha.

Ngakho-ke, ekungabikho kwe-enzyme ye-ADAMTS 13, ama-molecule we-von Willebrand factor ahlala emakhulu futhi inqubo yokumiswa kwegazi ikhubazekile futhi kunethuba elikhulu lokwakheka kwamahlwili.

Ngakho-ke, i-PTT ingaba nezimbangela zofuzo, ezihambelana nokushoda kwe-ADAMTS 13, noma okutholakele, okuyizona eziholela ekwehleni kwenani lama-platelet, njengokusebenzisa imishanguzo yokuvikela umzimba noma yamakhemikhali yokwelapha noma ama-antiplatelet agents, izifo, ukushoda kokudla okunomsoco noma izifo ezizimele, isibonelo.

Izimpawu nezimpawu eziyinhloko

I-PTT ivamise ukukhombisa izimpawu ezingacacisiwe, kepha kuvamile ezigulini ezine-PTT okusolwa ukuthi okungenani zinezici ezintathu ezilandelayo:

1. I-thrombocythemia ephawulwe;
2. I-hemolytic anemia, ngoba i-thrombi yakha ukuthandwa kwamaseli abomvu egazi;
3. Imfiva;
4. I-thrombosis, engenzeka ezingxenyeni eziningana zomzimba;
5. Ubuhlungu obukhulu besisu ngenxa ye-ischemia yamathumbu;
6. Ukukhubazeka kwezinso;
7. Ukukhubazeka kwemizwa, okungabonakala ngokuqaqanjelwa yikhanda, ukudideka kwengqondo, ukozela ngisho nokuquleka.

Kujwayelekile futhi ezigulini ezine-PTT okusolwa ukuthi zinezimpawu ze-thrombocytopenia, ezinjengokubonakala kwezimagqabhagqabha ezibomvu noma ezibomvu esikhunjeni, izinsini ezophayo noma ngamakhala, ngaphezu kolawulo olunzima lokopha ekhaleni elincane. Yazi ezinye izimpawu ze-thrombocytopenia.

Ukungasebenzi kahle kwezinso nezinzwa yizinkinga eziyinhloko ze-PTT futhi kuvela lapho i-thrombi encane ivimbela ukudlula kwegazi liye kuzo zombili izinso nobuchopho, okungaholela ekuhlulekeni kwezinso nokushaywa unhlangothi, ngokwesibonelo. Ukugwema izinkinga, kubalulekile ukuthi lapho nje kuvela izimpawu zokuqala, kuboniswane nodokotela ojwayelekile noma i-hematologist ukuze kuqale ukuxilongwa nokwelashwa.

Ukuxilongwa kwenziwa kanjani

Ukuxilongwa kwe-thrombotic thrombocytopenic purpura kwenziwa ngokuya ngezimpawu ezethulwe ngumuntu, ngaphezu komphumela wesibalo segazi, lapho kwehla khona inani lamaplatelets, okuthiwa i-thrombocytopenia, ngaphezu kokubonwa i-blood smear platelet aggregation, okuyilapho amaplatelet enamathelana khona, ngaphezu kwama-schizocyte, okuyizingcezu zamaseli abomvu egazi, ngoba amangqamuzana abomvu egazi adlula emithanjeni yegazi evinjwe yimithambo emincane.

Olunye uvivinyo lungahle futhi luyelelwe ukusiza ukuxilongwa kwe-PTT, njengesikhathi sokopha, esandiswayo, nokungabikho noma ukwehliswa kwe-enzyme i-ADAMTS 13, okungenye yezimbangela zokwakheka kwe-thrombi encane.

Ukwelashwa kwe-PTT

Ukwelashwa kwe-thrombotic thrombocytopenic purpura kufanele kuqalwe ngokushesha okukhulu, ngoba kuyabulala ezimweni eziningi, ngoba i-thrombi eyakhiwe ingavimba imithambo efinyelela ebuchosheni, yehlise ukugeleza kwegazi kuleyo ndawo.

Ukwelashwa okuvame ukukhonjiswa yi-hematologist yi-plasmapheresis, okuyinqubo yokuhlunga igazi lapho ukwedlula kwama-antibody angadala lesi sifo nokudlulela kwe-von Willebrand factor, ngaphezu kokunakekelwa okusekelayo, njenge-hemodialysis, isibonelo. , uma kukhona ukukhubazeka kwezinso. Qonda ukuthi i-plasmapheresis yenziwa kanjani.

Ngaphezu kwalokho, ukusetshenziswa kwama-corticosteroids nemithi yokuzivikela emzimbeni, ngokwesibonelo, kunganconywa ngudokotela, ukuze kuliwe nezimbangela ze-PTT futhi kugweme nezinkinga.