I-Thrombotic Thrombocytopenic Purpura: Into eyiyo, oonobangela kunye nonyango

Umxholo

• Oonobangela be-PTT
• Iimpawu eziphambili kunye neempawu
• Uxilongo lwenziwa njani
• Unyango lwe-PTT

I-thrombotic thrombocytopenic purpura, okanye i-PTT, sisifo esinqabileyo kodwa esibulalayo se-hematological esibonakaliswa ngokwenziwa kwe-thrombi encinci kwimithambo yegazi kwaye ixhaphake kakhulu kubantu abaphakathi kweminyaka engama-20 nama-40 ubudala.

Kwi-PTT kukho ukuhla okuphawulweyo kwenani leeplatelets, ukongeza kumkhuhlane kwaye, kwiimeko ezininzi, ukukhubazeka kwemithambo-luvo ngenxa yotshintsho lokuhamba kwegazi kwingqondo ngenxa yamahlwili.

Ukuxilongwa kwe-PTT kwenziwa ngugqirha wegazi okanye ugqirha jikelele ngokweempawu kunye nesiphumo sobalo olupheleleyo lwegazi kunye ne-smear yegazi kwaye unyango kufuneka luqaliswe kwakamsinya emva koko, kuba esi sifo siyabulala malunga ne-95% xa singanyangwa.

Oonobangela be-PTT

I-thrombotic thrombocytopenic purpura ikakhulu ibangelwa kukusilela okanye utshintsho kwimfuza ye-enzyme, i-ADAMTS 13, enoxanduva lokwenza iimolekyuli ze-von Willebrand factor incinci, kunye nokuthanda umsebenzi wazo. I-von Willebrand factor ikhona kwiiplatelets kwaye inoxanduva lokukhuthaza ukubambelela kwiplatelet kwi-endothelium, ukunciphisa kunye nokuyeka ukopha.

Ke, ngokungabikho kwe-ADAMTS 13 enzyme, iimolekyuli ze-von Willebrand zihlala zikhulu kwaye inkqubo yokuma kwegazi iphazamisekile kwaye kukho ithuba elikhulu lokwakheka kwehlwili.

Ke, i-PTT inokuba nezizathu zofuzo, ezingqinelana nokusilela kwe-ADAMTS 13, okanye ezifunyenweyo, ezo zikhokelela ekwehleni kwinani leeplatelets, ezinje ngokusetyenziswa kweyeza lokuzikhusela emzimbeni okanye amayeza e-chemotherapeutic okanye ii-antiplatelet agents, izifo, ukusilela kwesondlo okanye izifo ezizimeleyo, umzekelo.

Iimpawu eziphambili kunye neempawu

I-PTT ihlala ibonisa iimpawu ezingacacanga, nangona kunjalo kuqhelekile kwizigulana ezinesikrokro se-PTT ukuba ubuncinci kwezi mpawu zilandelayo:

1. Thrombocythemia ephawulwe;
2. I-anemia ye-hemolytic, kuba i-thrombi yakha i-lysis yeeseli ezibomvu zegazi;
3. Ifiva;
4. I-thrombosis, enokuthi yenzeke kumalungu aliqela omzimba;
5. Iintlungu ezinzima zesisu ngenxa ye-ischemia yamathumbu;
6. Ukukhubazeka kwezintso;
7. Ukuphazamiseka kwimithambo-luvo, okunokuthi kubonwe yintloko ebuhlungu, ukudideka kwengqondo, ukozela kunye nokudinwa.

Kuqhelekile nakwizigulana ezinePTT ekrokrelwayo ukuba neempawu ze-thrombocytopenia, ezinjengokubonakala kwamabala amfusa okanye abomvu eluswini, iintsini ezophayo okanye ezimpumlweni, ukongeza kulawulo olunzima lokopha kumanxeba amancinci. Yazi ezinye iimpawu ze-thrombocytopenia.

Umsebenzi we-renal kunye ne-neurological dysfunctions zezona ngxaki ziphambili ze-PTT kwaye zivela xa i-thrombi encinci ithintela ukuhamba kwegazi kuzo zombini izintso kunye nengqondo, enokubangela ukusilela kwezintso kunye nokubetha, umzekelo. Ukuthintela iingxaki, kubalulekile ukuba kwakamsinya nje ukuba kuvele iimpawu zokuqala, ugqirha jikelele okanye ugqirha wegazi kuyaboniswana naye ukuze kuqalwe ukuxilongwa kunye nonyango.

Uxilongo lwenziwa njani

Ukuchongwa kwe-thrombotic thrombocytopenic purpura yenziwa ngokusekwe kwiimpawu eziboniswe ngumntu, ukongeza kwisiphumo sobalo lwegazi, apho kuncitshiswa inani leeplatelets, ekuthiwa yi-thrombocytopenia, kuyabonakala, ukongeza ekubonweni ukudityaniswa kweplatelet yegazi, apho amaplatelet enamathelayo, ukongeza kwii-schizocyte, eziziingceba zeeseli ezibomvu zegazi, kuba iiseli ezibomvu zegazi zidlula kwimithambo yegazi ethintelwe yimithambo encinci.

Olunye uvavanyo lunokuyalelwa ukuba luncede ekuchongeni i-PTT, enjengexesha lokopha, elonyukayo, kunye nokungabikho okanye ukunciphisa i-enzyme i-ADAMTS 13, engunobangela wokuyilwa kwe-thrombi encinci.

Unyango lwe-PTT

Unyango lwe-thrombotic thrombocytopenic purpura kufuneka luqalwe ngokukhawuleza, kuba luyabulala kwiimeko ezininzi, kuba i-thrombi eyenziweyo inokuthintela imithambo efikelela kwingqondo, ukunciphisa ukuhamba kwegazi kuloo mmandla.

Unyango oluqhelekileyo olubonakaliswe yi-hematologist yi-plasmapheresis, eyinkqubo yokuhluza igazi apho ukugqitha kwamajoni omzimba kunokubangela esi sifo kunye nokugqithisa kwe-von Willebrand factor, ukongeza kukhathalelo oluxhasayo, njenge-hemodialysis, umzekelo. , ukuba kukho ukuphazamiseka kwezintso. Qonda ukuba iplasmapheresis yenziwa njani.

Ukongeza, ukusetyenziswa kwe-corticosteroids kunye neziyobisi ezikhuselayo, umzekelo, kunokucetyiswa ngugqirha, ukuze kuliwe nonobangela we-PTT kwaye kuthintelwe iingxaki.