Thrombotic Thrombocytopenic Purpura: Seo e leng sona, lisosa le kalafo

Litaba

• Lisosa tsa PTT
• Matšoao le matšoao a mantlha
• Mokhoa oa ho hlahloba o etsoa joang
• Kalafo ea PTT

Thrombotic thrombocytopenic purpura, kapa PTT, ke lefu le sa tloaelehang empa le bolaeang la hematological le khetholloang ke ho thehoa ha thrombi e nyane methapong ea mali mme le atile haholo bathong ba pakeng tsa lilemo tse 20 le 40.

PTT ho na le phokotso e hlakileng ea palo ea liplatelete, ntle le feberu 'me, maemong a mangata, ho holofala ha methapo ea kutlo ka lebaka la phetoho ea phallo ea mali bokong ka lebaka la ho koaloa.

Ho fumanoa ha PTT ho etsoa ke ngaka ea methapo ea mafu kapa ngaka e akaretsang ho latela matšoao le sephetho sa palo e felletseng ea mali le smear ea mali mme kalafo e tlameha ho qalisoa haufinyane kamora moo, kaha lefu lena le bolaea hoo e ka bang 95% ha le sa alafshoe.

Lisosa tsa PTT

Thrombotic thrombocytopenic purpura e bakoa haholo ke khaello kapa phetoho ea liphatsa tsa lefutso ea enzyme, ADAMTS 13, e ikarabellang ho etsa hore limolek'hule tsa von Willebrand factor e be nyane, le ho ts'ehetsa tšebetso ea tsona. Lebaka la von Willebrand le teng ka liplatelete mme le ikarabella ho khothaletsa khokahano ea platelet ho endothelium, e fokotsehang le ho emisa ho tsoa mali.

Ka hona, ka lebaka la ho ba sieo ha li-enzyme tsa ADAMTS 13, limolek'hule tsa von Willebrand factor li lula li le kholo mme ts'ebetso ea ho ema ha mali e senyehile mme ho na le monyetla o moholo oa ho thehoa ha lesela.

Kahoo, PTT e kanna ea ba le lisosa tsa lefutso, tse tsamaellanang le khaello ea ADAMTS 13, kapa e fumanoeng, e leng tsona tse lebisang ho fokotseheng ha palo ea liplatelete, joalo ka ts'ebeliso ea lithethefatsi tsa immunosuppressive kapa chemotherapeutic kapa li-antiplatelet agents, tšoaetso, khaello ea phepo e nepahetseng kapa mohlala, mafu a itšireletsang mafung.

Matšoao le matšoao a mantlha

PTT hangata e bonts'a matšoao a sa totobalang, leha ho le joalo ho tloaelehile hore bakuli ba belaelloang ba PTT ba be le bonyane litšobotsi tse 3 tse latelang:

1. Tšoauoa thrombocythemia;
2. Hemolytic anemia, hobane thrombi e ile ea khetha lysis ea lisele tse khubelu tsa mali;
3. Feberu;
4. Thrombosis, e ka hlahang likarolong tse 'maloa tsa' mele;
5. Bohloko bo boholo ba mpeng ka lebaka la ischemia ea mala;
6. Ho senyeha ha renal;
7. Ho holofala ha methapo ea kutlo, ho ka bonoang ka ho opeloa ke hlooho, ho ferekana kelellong, ho otsela esita le ho akheha.

Ho boetse ho tloaelehile hore bakuli ba belaelloang ba PTT ba be le matšoao a thrombocytopenia, joalo ka ponahalo ea likhahla tse pherese kapa tse khubelu letlalong, marenene a tsoang mali kapa ka nko, ntle le taolo e thata ea ho tsoa mali maqeba a manyane. Tseba matšoao a mang a thrombocytopenia.

Mathata a renal le methapo ea kutlo ke mathata a mantlha a PTT mme a hlaha ha thrombi e nyane e sitisa ho fetela ha mali ho ea liphio le bokong, ka mohlala ho ka fella ka ho hloleha ha liphio le stroke. Ho qoba mathata, ho bohlokoa hore hang ha lipontšo tsa pele li hlaha, ngaka e akaretsang kapa hematologist e botsoa e le hore tlhahlobo le kalafo li ka qala.

Mokhoa oa ho hlahloba o etsoa joang

Ho fumanoa ha thrombotic thrombocytopenic purpura ho etsoa ho latela matšoao a motho eo, ntle le phello ea palo ea mali, eo ho eona ho fokotsoang palo ea liplatelete, e bitsoang thrombocytopenia, ntle le ho bonoa ho mali smear platelet aggregation, e leng ha liplatelete li khomarelana, ntle le li-schizocyte, e leng likotoana tsa lisele tse khubelu tsa mali, hobane lisele tse khubelu tsa mali li feta methapong ea mali e koetsoeng ke methapo e menyenyane.

Liteko tse ling le tsona li ka laeloa ho thusa ho sibolla PTT, joalo ka nako ea ho tsoa mali, e eketsoang, le ho ba sieo kapa phokotso ea enzyme ea ADAMTS 13, e leng e 'ngoe ea lisosa tsa ho theoa ha thrombi e nyane.

Kalafo ea PTT

Kalafo ea thrombotic thrombocytopenic purpura e lokela ho qalisoa kapele kamoo ho ka khonehang, kaha e ea bolaea maemong a mangata, kaha thrombi e thehiloe e ka sitisa methapo e fihlang bokong, e fokotsa phallo ea mali sebakeng seo.

Kalafo e atisang ho bontšoa ke hematologist ke plasmapheresis, e leng ts'ebetso ea ho sefa mali eo ho eona lithibela-mafu tse ngata tse ka bakang lefu lena le ho feta ha von Willebrand factor, ho kenyelletsa le tlhokomelo e tšehetsang, joalo ka hemodialysis, mohlala. , haeba ho na le ho senyeha ha renal. Utloisisa hore na plasmapheresis e etsoa joang.

Ntle le moo, ts'ebeliso ea li-corticosteroids le lithethefatsi tsa immunosuppressive, ka mohlala, li ka khothaletsoa ke ngaka, molemong oa ho loants'a sesosa sa PTT le ho qoba mathata.