Ingabe nawe unamahlule egazi emzimbeni wakho ngaphandle kwesizathu? Ake sifunde ngale TTP eyingozi (Thrombotic Thrombocytopenic Purpura)!

Wake wezwa nge-Thrombotic Thrombocytopenic Purpura, noma i-TTP ngamafuphi? Lesi yisifo segazi esingavamile kodwa esibi kakhulu. Yisimo lapho kwakheka khona amahlule egazi (thrombi) emithanjeni yegazi emincane emzimbeni wakho. Kufana nokuvaleka kwepayipi lamanzi. Lokhu kungavimba ukugeleza kwegazi ezithweni ezibaluleke kakhulu emzimbeni wakho, njengobuchopho, izinso, nenhliziyo. Lokhu kungaholela ezinkingeni ezinkulu. Yingakho kubalulekile ukuqaphela i-TTP.

Kuyini i-TTP? Ake siqonde nje incazelo yegama.

Igama lalesi sifo lingase libonakale lide kancane. Kodwa izingxenye ezintathu zaleli gama zisinika umbono omuhle ngohlobo lwalesi sifo. Ake sibone ukuthi sinjani?

1. I-Thrombotic: Lokhu kubhekisela kuma -blood clots (thrombi) akheka ngaphakathi kwemithambo yegazi, njengoba ngishilo ekuqaleni. La ma-blood clots ayimbangela yenkinga enkulu.

2. I-Thrombocytopenic: Lokhu kusho ukuthi kunezinhlayiya ezincane egazini lakho. Izinhlayiya zifana namasosha amancane asiza ekumiseni ukopha lapho silimala. Kodwa uma kwenzeka i-TTP, lezi zinhlayiya zisetshenziswa ngokungadingekile ukwenza amahlule egazi. Khona-ke azikho izinhlayiya ezanele lapho ukopha kudingeka kume khona ngempela.

3. I-Purpura: Lokhu kusho ukuthi isikhumba sakho siveza amabala amancane ansomi noma imihuzuko. Kungase kubukeke njengokuqubuka. Empeleni lezi yizimpawu zokuphuma kwegazi okuncane ngaphansi kwesikhumba. Lokhu kuvamile kubantu abane-TTP.

Kalula nje, i-TTP yisimo esibi lapho kwakheka khona amahlule egazi emithanjeni yegazi emincane yomzimba wethu, okubangela inani eliphansi lama-platelet kanye nezimpawu zokopha esikhumbeni. Lokhu kuvame ukwenzeka ngokuzumayo, ngakho-ke ukwelashwa okuphuthumayo kuyadingeka . Abanye abantu bangaphinde babe nalesi simo ngisho nangemva kokwelashwa. Kodwa-ke, izimpawu zivame ukucaca lapho lesi sifo sisebenza. Ngezinye izikhathi singabangelwa izinto ezifana nokutheleleka ngegciwane, ukuhlinzwa, noma ukukhulelwa. Uma singalashwa, i-TTP ingaba yingozi empilweni.

Ziyini izimpawu ze-TTP?

I-TTP ivame ukufika ngokushesha kakhulu. Abanye abantu bayalulama bese bebuyela emuva, kodwa izimpawu azifiki futhi ziphele. Zihlala zinjalo futhi zisobala. Ayizipheli yodwa, futhi ngokuqinisekile idinga ukwelashwa.

I-TTP ibangela ukwakheka kwamahlule egazi emithanjeni yegazi emincane kuwo wonke umzimba. Lawa mahlule angathinta noma yisiphi isitho, kodwa izimpawu zivame ukuvela ebuchosheni kuqala.

Cabanga nje, uma la mahlule egazi akheka enhliziyweni, angathinta kakhulu ukusebenza kwenhliziyo, mhlawumbe aze abangele nokufa. I-TTP ibangela i-anemia (inani eliphansi legazi) kanye namazinga aphansi e-platelet (i-thrombocytopenia). Abanye abantu bangase babe nokopha, kodwa lokhu akuvamile.

Nazi ezinye zezimpawu ezivame kakhulu ze-TTP:

• Isimo sengqondo esishintshile: Ukuphazamiseka okungazelelwe, ukudideka, noma ukulahlekelwa ukwazi.
• Ukudideka: Ukungazi ukuthi kwenzekani noma ukuthi ukuphi.
• Ukukhathala okukhulu: Ukuzizwa ukhathele kangangokuthi awukwazi ukwenza lutho.
• Ikhanda elibuhlungu: Ikhanda elibuhlungu elinzima kakhulu kunekhanda elibuhlungu elivamile.
• Isicanucanu nokuhlanza: Ukuphazamiseka kwesisu, ukuhlanza.
• Isikhumba esiphaphathekile noma esiphuzi (i-Jaundice): Isikhumba singase sibe mhlophe ngenxa yokuntuleka kwegazi emzimbeni, futhi singase sibe phuzi ngenxa yemiphumela esibindini.
• Ubunzima bokuphefumula (Dyspnea): Ukuzizwa sengathi kunzima ukuphefumula.
• Amachashazi amancane abomvu noma ansomi ngaphansi kwesikhumba (i-Petechiae): Amachashazi amancane egazi avela esikhumbeni, njengokungathi ahlatshwe yiphini.
• I-Hematuria: Igazi emchameni .
• Ukushintsha kombono: Umbono ofiphele, ngezinye izikhathi ukubona izinto ezimbili ngesikhathi esisodwa (i-diplopia).

Uma uhlangabezana nesinye noma ngaphezulu kwalezi zimpawu ngokuzumayo, kufanele ubonane nodokotela ngaphandle kokuchitha isikhathi.

Kungani le TTP yenzeka? Ziyini izimbangela?

Amahlule egazi abangela izinkinga ku-TTP akheka lapho umzimba ungenayo i-enzyme eyanele ebizwa ngokuthi i-ADAMTS13. Lokho okwenziwa yile enzyme ye-ADAMTS13 ukuvimba ama-platelet ukuthi angahlangani futhi akhe amahlule egazi lapho singawadingi. Cabanga ngakho njengephoyisa lomgwaqo, ukuvimbela ukuminyana kwezimoto okungadingekile. Ngakho-ke, lapho le ADAMTS13 incipha, amahlule egazi aqala ukwakheka emithanjeni yegazi emincane kuwo wonke umzimba. Lapho la mahlule egazi ehlangana, ngisho namaseli abomvu egazi angaphuka futhi aphuke.

Ngenxa yokuthi ama-platelet amaningi kangaka asetshenziselwa ukwakha amahlule egazi angadingekile, lapho empeleni sidinga ukuvimba ukuphuma kwegazi ngenxa yokulimala, kusele ama-platelet ambalwa kakhulu okusetshenziswa. Uma inani lama-platelet akho lehla kakhulu, ungaphuka kalula futhi wophe igazi ngaphezu kokuvamile.

I-TTP ingenzeka ngezindlela ezimbili eziyinhloko:

1. I-TTP etholakale (lokhu kuvame kakhulu): Lokhu kwenzeka lapho amasosha omzimba wakho enza khona ama-antibodies ngephutha avimbela i-enzyme ye-ADAMTS13 ukuthi isebenze kahle. Kufana namasosha akho azihlasela wona.

2. I-TTP ezuzwe njengefa (engavamile): Lokhu kwenzeka lapho ingane izuza isici esakhiweni sofuzo esenza i-enzyme ye-ADAMTS13 kubo bobabili abazali. Izimpawu zivame ukuqala ngesikhathi sokukhulelwa noma ngezikhathi zokucindezeleka okukhulu.

Ingabe zikhona yini izimbangela ezithile ezingabangela ukuthi i-TTP yenzeke?

Ochwepheshe abakakazi kahle ukuthi yini ebangela izimpawu ze-thrombotic thrombocytopenic purpura. Kodwa-ke, abanye abantu banamathuba amaningi okuthola lesi simo uma:

• Ukutheleleka nge-HIV
• Izifo ezizenzakalelayo njenge -lupus
• Ukukhulelwa
• Eminye imithi, isibonelo imithi ye-chemotherapy , i-cyclosporine A , kanye ne-quinine .

Yiziphi izinkinga ezingaba khona ze-TTP?

I-thrombotic thrombocytopenic purpura iyisimo esisongela ukuphila uma singelashwa. Amahlule egazi angavimba ukuhamba kwegazi futhi abangele izinkinga ezinkulu, njenge:

• Ukulimala kobuchopho
• Isifo senhliziyo
• Ukungasebenzi kahle kwezinso
• Ukuphuphuma Kwesisu (Ukulahlekelwa Ukukhulelwa)
• Ukuquleka
• Isifo sohlangothi

Yingakho sithi kubaluleke kakhulu ukufuna ukwelashwa ngokushesha uma unezimpawu ze-TTP.

Odokotela bayixilonga kanjani i-TTP?

Udokotela wakho uzoqala akuhlole bese ebuza ngezimpawu zakho kanye nomlando wempilo yakho. Uma kusolwa ukuthi i-TTP iyatholakala, angase acele ukuhlolwa okuningana, okufana nalokhu:

• Ukuhlolwa kwe-ADAMTS13: Lokhu kuhlolwa kulinganisa umsebenzi we-enzyme ye-ADAMTS13. Uma izinga layo liphansi, kungaqinisekisa ukuba khona kwe-TTP.
• Ukubalwa kwegazi okuphelele (i-CBC): I-CBC ibala izinhlobo zamaseli egazini lakho. Abantu abane-TTP banenani eliphansi lamaseli abomvu egazi kanye nama-platelet.
• I-Peripheral blood smear (PBS): Lokhu kuhilela ukuthatha isampula yegazi lakho bese ulihlola ngaphansi kwe-microscope. Uma une-TTP, amangqamuzana akho abomvu egazi angase aphuke futhi adabuke (abizwa nangokuthi ama-schistocytes ).
• Ukuhlolwa kwe-Bilirubin: I-Bilirubin iwumkhiqizo ongcolile okhiqizwa amangqamuzana egazi abomvu alimele. Amazinga aphezulu e-bilirubin ayinkomba yokuthi ungase ube ne-TTP.
• Ukuhlolwa kokusebenza kwezinso: Lokhu kuhlolwa kungasiza udokotela wakho ukuthi abone ukuthi izinso zakho zisebenza kahle yini. Uma une-TTP, ungase ube namaseli egazi noma amaprotheni emchameni wakho.
• Ukuhlolwa kwe-Lactate dehydrogenase (LDH): I-Lactate dehydrogenase (LDH) iyi-enzyme ekhishwa lapho izicubu zomzimba wakho zonakele. I-TTP ibangela amazinga aphezulu e-LDH egazini.

Yiziphi izindlela zokwelapha i-TTP?

Ezimweni eziningi, odokotela basebenzisa ukwelashwa nge-plasma ukwelapha i-TTP. Ngaphezu kwalokho, kunezindlela zokwelapha ngemithi.

Ukwelashwa nge-plasma

Izindlela zokwelapha ezivame kakhulu yilezi:

• Ukushintshaniswa kwe-plasma: Lokhu kuhilela ukususa i-plasma yakho engavamile (ingxenye ewuketshezi yegazi lakho) bese uyishintsha nge-plasma evela kumnikeli onempilo. Lokhu kuyindlela yokwelapha esindisa impilo yabantu abane-TTP etholwe.
• Ama-Corticosteroids: Le mithi isiza ukulawula i-TTP ebukhali, ukunciphisa izimpawu, nokunciphisa ukuvuvukala.

Ukwelashwa nge-plasma kwenziwa esibhedlela futhi kungaqhubeka izinsuku noma amasonto kuze kube yilapho izimpawu zakho ziphela.

Imithi

Kunemithi ethile enganciphisa noma imise ukwakheka kwama-antibodies ngokumelene ne-enzyme ye-ADAMTS13 (ama-anti-ADAMTS13 antibodies). Eminye yemithi odokotela abayisebenzisayo ukwelapha i-TTP yile:

• Ama-Glucocorticoids: Lokhu kwelashwa kucindezela amasosha omzimba wakho. Lokhu kungaba usizo ku-TTP.
• I-Caplacizumab: Lo muthi welapha i-TTP ebukhali. Wandisa inani lama-platelet ngokushesha kunokushintshana kwe-plasma kuphela, okusiza ekubuyiseleni lesi sifo ngokushesha.
• I-Rituximab: Lokhu kwelashwa kusiza ukuvimbela lesi sifo ukuthi singabuyi ngemva kokwelashwa ngama-corticosteroids kanye nokushintshaniswa kwe-plasma.
• I-Recombinant ADAMTS13: Lokhu kwelashwa kubuyisela i-enzyme ye-ADAMTS13. Kuvunyelwe yi-U.S. Food and Drug Administration (FDA) ukuze kusetshenziswe ngokuphuthumayo kubantu abane-TTP ezuzwe njengefa. Ngezinye izikhathi, odokotela bayayisebenzisa futhi ukuvimbela ukopha ngokweqile. Ucwaningo luyaqhubeka ukuze lubone ukuthi luwusizo kangakanani kwi-TTP ephethwe amasosha omzimba.

Ukuhlinzwa

Akuvamile kakhulu, udokotela wakho angase ancome ukususwa kwe-spleen yakho ngokuhlinzwa (i-spleenectomy). Lokhu kwenziwa kuphela uma une-TTP enzima engaphenduli kahle ekwelashweni noma uma iqhubeka nokuvela.

Kuthatha isikhathi esingakanani ukululama ekwelashweni kwe-TTP?

Isikhathi sokululama sincike ezintweni eziningana. Ubunzima besimo sakho kanye nohlobo lokwelashwa olutholayo kungathinta lokhu. Iningi labantu abathola ukwelashwa nge-plasma kwe-TTP kuthatha izinsuku ezimbalwa kuya emavikini ambalwa ukuze balulame. Abantu abahlinzwa i-splenectomy bavame ukuthatha cishe amasonto amane kuya kwayisithupha ukuze balulame.

Kufanele ngimbone nini udokotela uma ngisola ukuthi ngine-TTP?

Izimpawu ze-TTP zivame ukuba zimbi kakhulu kangangokuthi zidinga ukuya ekamelweni eliphuthumayo. Uma usola ukuthi une-TTP, ungalindi ukubona udokotela ojwayelekile - ukwelashwa okusheshayo kubalulekile!Ubuchwepheshe be-hematologists kanye nochwepheshe bezokunakekelwa okujulile bubalulekile ekuphatheni i-TTP ebukhali.

Yini ongayilindela uma uphila ne-TTP? Iyini iphrojekthi yokululama?

I-TTP yayivame ukuba yisifo esibulalayo. Kodwa ngokwelashwa kusenesikhathi, izinga lokufa selihle lafinyelela cishe ku-10%. Njengoba lesi sifo singaphinde sibuye, kubalulekile ukuhlala useduze nodokotela wakho wegazi ngisho nangemva kokwelashwa. Ukuhlola njalo amazinga akho e-ADAMTS13 kungasiza ekutholeni noma yiziphi izinguquko kusenesikhathi. Uma amazinga e-ADAMTS13 ephansi ngaphambi kokuba izimpawu zibuye, ukwelashwa kokuvimbela njenge-Rituximab kungasiza ekuvimbeleni lesi sifo ukuthi singaphinde sibuye ngaphambi kokuba siqale.

Lingakanani izinga lokusinda kwe-TTP?

Ngaphandle kokwelashwa, umuntu oyedwa kuphela kwabayishumi abane-TTP uyasinda. Kodwa ngokwelashwa okufanele, abantu abayisishiyagalombili kuya kwabayisishiyagalolunye kwabayishumi bangasinda.

Yingakho kubalulekile ukufuna ukwelashwa uma unezimpawu. Kubalulekile futhi ukulandela imiyalelo kadokotela wakho yokuthi ungazinakekela kanjani ngemva kokuxilongwa.

Ngokwelashwa okufanele, abantu abaningi abane-Thrombotic Thrombocytopenic Purpura (TTP) baphila impilo evamile. Udokotela wakho uzochaza ukuthi udinga kangaki ukuhlolwa ukuze aqaphe isimo sakho. Uzokutshela nokuthi udinga ukwelashwa njalo yini ukuze unciphise ingozi yokopha kanye nokuqhekeka kwegazi.

Okwamanje, uma unezimpawu ze-TTP, funa ukwelashwa ngokushesha. I-TTP iyisimo esiphuthumayo sezokwelapha. Kodwa ukwelashwa kusenesikhathi kungasindisa impilo yakho.

Ngakho-ke, yini into ebaluleke kakhulu okufanele uyikhumbule kulokho esikhulume ngakho?

Kulungile, sesikhulume kakhulu nge-TTP (Thrombotic Thrombocytopenic Purpura). Izinto ezibaluleke kakhulu okufanele uzikhumbule yilezi:

• I-TTP iyisifo segazi esingavamile kodwa esibi kakhulu lapho kwakheka khona amahlule egazi emithanjeni yegazi emincane yomzimba.
• Uma uzwa izimpawu ezifana nokudideka okungazelelwe, ikhanda elibuhlungu kakhulu, amabala ansomi esikhumbeni sakho, noma ukuphaphatheka, kungaba yi-TTP. Ngakho-ke ungachithi isikhathi bese ubona udokotela ngokushesha.
• Lokhu ngeke kuphulukise ngokwakho. Ukwelashwa okuphuthumayo kuyadingeka nakanjani.
• I-TTP ingelapheka uma itholakale kusenesikhathi futhi yelashwa kahle. Njengoba kunezindlela zokwelapha ezithuthukisiwe ezitholakalayo namuhla, amathuba okusindisa izimpilo aphezulu kakhulu.
• Ngisho nangemva kokwelashwa, landela imiyalelo kadokotela bese uhlolwa. Lokhu kuzosiza ekuvimbeleni ukuthi lesi sifo singaphinde sivele.

Uma wena noma othile omaziyo esola into efana nale, ungesabi. Kodwa thatha isinyathelo ngokushesha. Ukufuna iseluleko sezokwelapha kuyinto engcono kakhulu ongayenza. Hlala uphilile!

I- Thrombotic Thrombocytopenic Purpura, i-TTP, isifo segazi, amahlule egazi, ama-platelet, izimpawu, ukwelashwa