Thrombotic Thrombocytopenic Purpura: Zvazviri, Zvinokonzera uye Kurapa

Zvemukati

• Zvinokonzera PTT
• Zviratidzo zvikuru nezviratidzo
• Maitiro ekuongororwa kunoitwa
• PTT Kurapa

Thrombotic thrombocytopenic purpura, kana PTT, chirwere chisingawanzo asi chinouraya chehematological icho chinozivikanwa nekuumbwa kwetrombi diki mumidziyo yeropa uye inowanzoonekwa muvanhu vari pakati pemakore makumi maviri nemakumi mana ekuberekwa.

Mu PTT pane kuderera kunozivikanwa muhuwandu hwemaplatelet, kuwedzera kune fivhiri uye, kazhinji, kukanganisika kwemitsipa nekuda kwekushandurwa kweropa kuyerera kuuropi nekuda kwekugwamba.

Kuongororwa kwePTT kunoitwa nachiremba wehematologist kana wezvechiremba zvichienderana nezviratidzo uye mhedzisiro yehuwandu hweropa uye smear yeropa uye kurapwa kunofanira kutanga munguva pfupi yapfuura, sezvo chirwere ichi chinouraya munenge 95% kana chisina kurapwa.

Zvinokonzera PTT

Thrombotic thrombocytopenic purpura inonyanya kukonzerwa nekushomeka kana kushanduka kwemajini enzyme, ADAMTS 13, inova nebasa rekugadzira mamorekuru evon Willebrand chinhu chidiki, nekufarira mashandiro avo. Iyo von Willebrand factor irimo mumaplatelet uye ine basa rekusimudzira kuomerera kweplatelet kune iyo endothelium, kudzikira uye kumisa kubuda kweropa.

Nekudaro, mukushayikwa kweADAMTS 13 enzyme, iyo von Willebrand factor mamorekuru anoramba akakura uye nzira yekumira kweropa yakakanganisika uye kune mukana mukuru wekuumbika kwegadziro.

Nekudaro, PTT inogona kunge iine zvikonzero zvekugara nhaka, zvinoenderana neAdAMTS 13 kushomeka, kana kuwanikwa, zvinova izvo zvinotungamira kudzikira kwenhamba yemaplatelet, sekushandisa kwemununusuppressive kana chemotherapeutic zvinodhaka kana antiplatelet agents, hutachiona, kushomeka kwehutano autoimmune hosha, semuenzaniso.

Zviratidzo zvikuru nezviratidzo

PTT inowanzo kuratidza zviratidzo zvisina kunyatsojeka, asi zvakajairika kune varwere vane fungidziro PTT kuve neinenge matatu eanotevera maitiro.

1. Yakaratidzwa thrombocythemia;
2. Hemolytic kupererwa neropa, sezvo thrombi yakagadzira kufadza iyo lysis yemasero matsvuku eropa;
3. Kupindwa nechando;
4. Thrombosis, iyo inogona kuitika mune dzakawanda nhengo dzemuviri;
5. Kurwadziwa kwakanyanya mudumbu nekuda kwematumbo ischemia;
6. Kukanganiswa kweRenal;
7. Kukanganiswa kweiyo Neurological, iyo inogona kucherechedzwa kuburikidza nemusoro, kuvhiringidzika kwepfungwa, kukotsira uye kunyangwe coma.

Zvakajairika kune varwere vane fungidziro PTT kuva nezviratidzo zvetrombocytopenia, senge kutaridzika kwepepuru kana kutsvukuruka paganda, kubuda ropa mazino kana kuburikidza nemumhino, kuwedzera kune iyo yakaoma kudzora pakubuda ropa kubva kumaronda madiki. Ziva zvimwe zviratidzo zvetrombocytopenia.

Renal uye neurological dysfunctions ndiwo matambudziko akakosha ePTT uye anomuka apo diki thrombi inodzivisa kufamba kweropa kune itsvo nehuropi, izvo zvinogona kukonzeresa itsvo kukundikana uye sitiroko, semuenzaniso. Kuti udzivise matambudziko, zvakakosha kuti pachangotanga zviratidzo zvekutanga, chiremba mukuru kana hematologist anobvunzwa kuitira kuti kuongororwa uye kurapwa kutange.

Maitiro ekuongororwa kunoitwa

Kuongororwa kwe thrombotic thrombocytopenic purpura kunoitwa zvichibva pane zviratidzo zvinoratidzwa nemunhu, kuwedzera kune mhedzisiro yehuwandu hweropa, umo kuderera kweuwandu hwemaplatelet, unonzi thrombocytopenia, kunoonekwa, kuwedzera pakuonekwa mune ropa smear platelet kuunganidzwa, inova ndiyo apo maplatelet anonamira pamwechete, kuwedzera kune schizocyte, ayo ari zvidimbu zvemasero matsvuku eropa, nekuti masero matsvuku eropa anodarika nemidziyo yeropa yakavharirwa nemidziyo midiki.

Dzimwe bvunzo dzinogona zvakare kurairwa kubatsira kuongororwa kwePNT, senge nguva yekubuda ropa, iyo inowedzerwa, uye kusavapo kana kuderedzwa kweiyo enzyme ADAMTS 13, inova imwe yezvikonzero zvekuumbwa kwetrombi diki.

PTT Kurapa

Kurapa kwe thrombotic thrombocytopenic purpura kunofanirwa kutanga nekukurumidza sezvazvinogona kuuraya muzviitiko zvakawanda, sezvo thrombi yakaumbwa inogona kutadzisa tsinga dzinosvika kuuropi, zvichideredza kuyerera kweropa kunharaunda iyoyo.

Mushonga unowanzo kuratidzwa nahematologist iplasmapheresis, inova nzira yekusefa kweropa umo kuwanda kwema antibodies anogona kukonzeresa chirwere ichi uye kuwanda kweVon Willebrand factor, mukuwedzera pakutsigira kwekutsigira, senge hemodialysis, semuenzaniso. , kana paine kukanganiswa kweitsvo. Nzwisisa kuti plasmapheresis inoitwa sei.

Uye zvakare, kushandiswa kwecorticosteroids uye immunosuppressive zvinodhaka, semuenzaniso, zvinogona kukurudzirwa nachiremba, kuitira kurwisa chinokonzera PTT uye kudzivirira kunetseka.