Thrombotic Thrombocytopenic Purpura: Zomwe Zili, Zomwe Zimayambitsa ndi Chithandizo

Zamkati

• Zomwe zimayambitsa PTT
• Zizindikiro zazikulu
• Momwe matendawa amapangidwira
• Chithandizo cha PTT

Thrombotic thrombocytopenic purpura, kapena PTT, ndi matenda osowa koma owopsa a hematological omwe amadziwika ndi mapangidwe a thrombi yaying'ono m'mitsempha yamagazi ndipo imakonda kwambiri anthu azaka zapakati pa 20 ndi 40.

Mu PTT pamakhala kuchepa kwakukulu kwa ma platelet, kuwonjezera pa malungo ndipo, nthawi zambiri, kuwonongeka kwa mitsempha chifukwa chosintha kwa magazi kulowa muubongo chifukwa cha kuundana.

Matenda a PTT amapangidwa ndi a hematologist kapena wothandizila malinga ndi zizindikilo zake komanso zotsatira za kuchuluka kwathunthu kwa magazi ndi kupaka magazi ndipo chithandizocho chiyenera kuyambika posachedwa, chifukwa matendawa amapha pafupifupi 95% osachiritsidwa.

Zomwe zimayambitsa PTT

Thrombotic thrombocytopenic purpura imayambitsidwa makamaka chifukwa cha kuchepa kapena kusintha kwa majini a enzyme, ADAMTS 13, yomwe imayambitsa ma molekyulu a von Willebrand factor kukhala ocheperako, ndikukonda magwiridwe ake. Chowonera cha von Willebrand chilipo m'maplateleti ndipo ali ndi udindo wolimbikitsa kumangiriza ma platelet ku endothelium, kuchepa ndikutaya magazi.

Chifukwa chake, pakalibe ma enzyme a ADAMTS 13, ma molekyulu a von Willebrand amakhalabe akulu ndipo njira yamagazi imalephera ndipo pamakhala mwayi waukulu wopanga ma clot.

Chifukwa chake, PTT itha kukhala ndi zoyambitsa zakubadwa, zomwe zikufanana ndi kuchepa kwa ADAMTS 13, kapena kuzipeza, zomwe ndizomwe zimayambitsa kuchepa kwa ma platelet, monga kugwiritsa ntchito mankhwala osokoneza bongo kapena chemotherapeutic kapena antiplatelet agents, matenda, kuperewera kwa zakudya kapena Mwachitsanzo, matenda amadzimadzi.

Zizindikiro zazikulu

PTT nthawi zambiri imawonetsa zisonyezo zapadera, komabe sizachilendo kwa odwala omwe akuganiziridwa kuti ali ndi PTT amakhala ndi zinthu zitatu izi:

1. Chizindikiro cha thrombocythemia;
2. Hemolytic anemia, popeza thrombi idapangira kukondera kwa maselo ofiira ofiira;
3. Malungo;
4. Thrombosis, yomwe imatha kupezeka m'magulu angapo amthupi;
5. Kupweteka kwambiri m'mimba chifukwa cham'mimba ischemia;
6. Kuwonongeka kwa impso;
7. Kuwonongeka kwa minyewa, komwe kumatha kuzindikirika kudzera mutu, kusokonezeka kwamaganizidwe, kugona komanso kukomoka.

Zimakhalanso zachilendo kwa odwala omwe akuganiza kuti ali ndi PTT amakhala ndi zizindikiro za thrombocytopenia, monga mawonekedwe ofiira kapena ofiira pakhungu, nkhama zotuluka magazi kapena mphuno, kuphatikiza pakuletsa kovuta kotuluka m'magazi ang'onoang'ono. Dziwani zizindikiro zina za thrombocytopenia.

Matenda a impso ndi amitsempha ndizovuta zazikulu za PTT ndipo zimayamba pomwe thrombi yaying'ono imalepheretsa magazi kupita ku impso ndi ubongo, zomwe zingayambitse impso ndi sitiroko, mwachitsanzo. Pofuna kupewa zovuta, ndikofunikira kuti zikangoyamba kuwonekera, dokotala kapena hematologist akafunsidwa kuti matenda ndi matenda ayambe.

Momwe matendawa amapangidwira

Matenda a thrombotic thrombocytopenic purpura amapangidwa kutengera zomwe zimaperekedwa ndi munthuyo, kuphatikiza pazotsatira za kuchuluka kwa magazi, momwe kuchepa kwa ma platelet, otchedwa thrombocytopenia, kumawonekeranso, kuphatikiza pakuwonedwa mu magazi smear platelet aggregation, ndipamene mapaleti amaphatikizana, kuphatikiza ma schizocyte, omwe ndi zidutswa za maselo ofiira, chifukwa maselo ofiira amagazi amadutsa mumitsempha yamagazi yomwe imatsekedwa ndi timitsempha tating'ono.

Mayesero ena atha kulamulidwanso kuti athandizire kuzindikira kwa PTT, monga nthawi yotaya magazi, yomwe yawonjezeka, komanso kupezeka kapena kuchepetsa kwa enzyme ADAMTS 13, chomwe ndi chimodzi mwazomwe zimayambitsa kupangika kwa thrombi yaying'ono.

Chithandizo cha PTT

Chithandizo cha thrombotic thrombocytopenic purpura chiyenera kuyambika mwachangu, chifukwa nthawi zambiri chimapha, popeza thrombi yopangidwa imatha kulepheretsa mitsempha yomwe imafikira muubongo, kuchepa kwa magazi kuderalo.

Chithandizo chomwe chimawonetsedwa ndi hematologist ndi plasmapheresis, yomwe ndi njira yosefera magazi momwe kuchuluka kwa ma antibodies omwe angayambitse matendawa komanso kuchuluka kwa von Willebrand factor, kuphatikiza chisamaliro chothandizira, monga hemodialysis, mwachitsanzo. , ngati pali kuwonongeka kwa impso. Mvetsetsani momwe plasmapheresis imachitikira.

Kuphatikiza apo, kugwiritsa ntchito ma corticosteroids ndi mankhwala osokoneza bongo, mwachitsanzo, atha kulimbikitsidwa ndi adotolo, kuti athane ndi vuto la PTT ndikupewa zovuta.