Ngaba nawe unamahlwili egazi emzimbeni wakho ngaphandle kwesizathu? Masifunde ngale TTP iyingozi (Thrombotic Thrombocytopenic Purpura)!

Ngaba wakha weva ngeThrombotic Thrombocytopenic Purpura, okanye iTTP ngamafutshane? Esi sisifo segazi esingaqhelekanga kodwa esibi kakhulu. Yimeko apho amahlwili egazi (i-thrombi) eyenzeka kwimithambo yegazi emincinci emzimbeni wakho. Kufana nokuvaleka kombhobho wamanzi. Oku kunokuthintela ukuhamba kwegazi ukuya kumalungu abalulekileyo emzimbeni wakho, njengengqondo, izintso kunye nentliziyo. Oku kunokukhokelela kwiingxaki ezinkulu. Yiyo loo nto kubalulekile ukuba uqaphele iTTP.

Yintoni i-TTP? Masiqonde nje intsingiselo yegama.

Igama lesi sifo lisenokubonakala lide kancinci. Kodwa la macandelo mathathu eli gama asinika umbono olungileyo ngohlobo lwesi sifo. Makhe sibone ukuba sinjani?

1. I-Thrombotic: Oku kubhekisa kumahlwili egazi (i-thrombi) ayenzeka ngaphakathi kwemithambo yegazi, njengoko benditshilo ngaphambili. La mahlwili egazi yimbangela yengxaki ephambili.

2. I-Thrombocytopenic: Oku kuthetha ukuba kukho iiplatelets ezimbalwa egazini lakho. Iiplatelets zifana namajoni amancinci anceda ekumiseni ukopha xa senzakele. Kodwa xa i-TTP isenzeka, ezi platelets zisetyenziswa ngokungeyomfuneko ukwenza amahlwili egazi. Emva koko akukho platelets zaneleyo apho ukopha kufuneka kume khona.

3. I-Purpura: Oku kuthetha ukuba ulusu lwakho luphuma amabala amancinci, amfusa okanye imivumbo. Isenokubonakala ngathi yirhashalala. Ezi ziimpawu zokopha okuncinci phantsi kolusu. Oku kuqhelekile kubantu abane-TTP.

Ngamafutshane, i-TTP yimeko enzima apho amahlwili egazi enziwa khona kwimithambo yegazi emincinci yomzimba wethu, nto leyo ebangela ukuba inani leeplatelet libe liphantsi kwaye kubekho neempawu zokopha eluswini. Oku kudla ngokuvela ngequbuliso, ngoko ke unyango olungxamisekileyo luyimfuneko . Abanye abantu banokuba nale meko kwakhona nasemva konyango. Nangona kunjalo, iimpawu zihlala zicacile xa isifo sisebenza. Ngamanye amaxesha sinokubangelwa zizinto ezifana nosulelo lwentsholongwane, utyando, okanye ukukhulelwa. Ukuba ayinyangwa, i-TTP inokuba yingozi ebomini.

Zithini iimpawu ze-TTP?

I-TTP idla ngokuvela ngokukhawuleza kakhulu. Abanye abantu bayachacha baze baphinde babuye, kodwa iimpawu azifiki ziphele. Zihlala zihleli kwaye zicacile. Ayizipheli yodwa, kwaye ngokuqinisekileyo ifuna unyango.

I-TTP ibangela ukuba kubekho amahlwili egazi kwimithambo yegazi emincinci emzimbeni wonke. La mahlwili anokuchaphazela naliphi na ilungu lomzimba, kodwa iimpawu zihlala ziqala ukubonakala engqondweni.

Khawucinge nje, ukuba la mahlwili egazi akheka entliziyweni, anokuchaphazela kakhulu ukusebenza kwentliziyo, mhlawumbi ade abangele nokufa. I-TTP ibangela i-anemia (umlinganiselo wegazi ophantsi) kunye namanqanaba aphantsi eeplatelet (thrombocytopenia). Abanye abantu banokufumana ukopha, kodwa oku akuvamile.

Nazi ezinye zezona mpawu zixhaphakileyo ze-TTP:

• Imeko yengqondo etshintshileyo: Ukuphazamiseka ngequbuliso, ukudideka, okanye ukulahlekelwa zingqondo.
• Ukudideka: Ukungazi ukuba kwenzeka ntoni okanye ukuba ukuphi.
• Ukudinwa kakhulu: Ukuziva udiniwe kangangokuba awukwazi kwenza nto.
• Intloko ebuhlungu: Intloko ebuhlungu kakhulu kunentloko eqhelekileyo.
• Isicaphucaphu nokuhlanza: Ukuphazamiseka kwesisu, ukuhlanza.
• Ulusu oluphaphathekileyo okanye olumthubi (i-Jaundice): Ulusu lunokuba mthubi ngenxa yokunqongophala kwegazi emzimbeni, kwaye lunokuba mthubi ngenxa yeziphumo esibindini.
• Ubunzima bokuphefumla (i-Dyspnea): Ukuziva ngathi kunzima ukuphefumla.
• Amachaphaza amancinci abomvu okanye amfusa phantsi kwesikhumba (iPetechiae): Amachaphaza amancinci egazi avela eluswini, ngokungathi ahlatywe yipini.
• I-Hematuria: Igazi elisemchameni .
• Utshintsho lombono: Umbono ofipheleyo, maxa wambi ubona izinto ezimbini ngaxeshanye (i-diplopia).

Ukuba ngequbuliso ufumana enye okanye ngaphezulu kwezi mpawu, kufuneka ubone ugqirha ngaphandle kokuchitha ixesha.

Kutheni le nto i-TTP yenzeka? Zithini izizathu?

Amahlwili egazi abangela iingxaki kwi-TTP enziwa xa umzimba ungenayo i-enzyme ebizwa ngokuba yi-ADAMTS13. Oko kwenziwa yile enzyme ye-ADAMTS13 kukuthintela iiplatelets ukuba zingadibani kwaye zenze amahlwili egazi xa singawadingi. Cinga ngako njengepolisa lendlela, ukuthintela ukuxinana kwezithuthi okungeyomfuneko. Ngoko ke, xa le ADAMTS13 inciphile, amahlwili egazi aqala ukwakheka kwimithambo yegazi emincinci emzimbeni wonke. Xa la mahlwili egazi edibana, neeseli ezibomvu zegazi zinokuqhekeka kwaye ziqhekeke.

Ngenxa yokuba iiplatelets ezininzi zisetyenziselwa ukwenza amahlwili egazi angafunekiyo, xa kufuneka simise ukopha ngenxa yokwenzakala, zimbalwa kakhulu iiplatelets eziseleyo ekufuneka zisetyenziswe. Ukuba inani leeplatelets zakho liyehla kakhulu, unokukrweleka lula kwaye wophe igazi ngaphezu kwesiqhelo.

I-TTP inokwenzeka ngeendlela ezimbini eziphambili:

1. I-TTP efunyenweyo (le yeyona ixhaphakileyo): Kulapho inkqubo yomzimba wakho yokuzikhusela isenza ngempazamo ii-antibodies ezithintela i-enzyme ye-ADAMTS13 ukuba isebenze kakuhle. Kufana namajoni akho azihlaselayo.

2. I-TTP ezuzwe njengelifa (engaqhelekanga): Oku kwenzeka xa umntwana ezuza isiphene kwi-gene eyenza i-enzyme ye-ADAMTS13 kubazali bobabini. Iimpawu zihlala ziqala ngexesha lokukhulelwa okanye ngamaxesha oxinzelelo olukhulu.

Ngaba kukho naziphi na izinto ezithile ezinokubangela ukuba i-TTP yenzeke?

Iingcali azikayazi kakuhle into ebangela iimpawu ze-thrombotic thrombocytopenic purpura. Nangona kunjalo, abanye abantu banokuba nale meko ukuba:

• Usulelo lwe-HIV
• Izifo ezizikhuselayo emzimbeni ezifana ne -lupus
• Ukukhulelwa
• Amanye amayeza, umzekelo amayeza e-chemotherapy , i-cyclosporine A , kunye ne -quinine .

Ziziphi iingxaki ezinokubakho ze-TTP?

I-Thrombotic thrombocytopenic purpura yimeko esongela ubomi ukuba ayinyangwa. Amahlwili egazi anokuthintela ukuhamba kwegazi aze abangele iingxaki ezinkulu, ezinje:

• Umonakalo wobuchopho
• Ukuhlaselwa yintliziyo
• Ukungasebenzi kakuhle kwezintso
• Ukuphuphuma kwesisu (Ukulahlekelwa kukukhulelwa)
• Ukuxhuzula
• Istroke

Yiyo loo nto sithi kubaluleke kakhulu ukufuna unyango ngokukhawuleza ukuba uneempawu ze-TTP.

Oogqirha bayixilonga njani i-TTP?

Ugqirha wakho uza kuqala akuhlole aze akubuze ngeempawu zakho kunye nembali yempilo yakho. Ukuba kurhanelwa ukuba une-TTP, banokuyalela iimvavanyo ezininzi, ezinje:

• Uvavanyo lwe-ADAMTS13: Olu vavanyo lulinganisa umsebenzi we-enzyme ye-ADAMTS13. Ukuba inqanaba layo liphantsi, linokuqinisekisa ubukho be-TTP.
• Ubalo lwegazi olupheleleyo (CBC): I-CBC ibala iintlobo zeeseli egazini lakho. Abantu abane-TTP banenani eliphantsi leeseli ezibomvu zegazi kunye neeplatelet.
• I-Peripheral blood smear (PBS): Oku kuquka ukuthatha isampuli yegazi lakho uze uyihlole phantsi kwe-microscope. Ukuba une-TTP, iiseli zakho ezibomvu zegazi zisenokuqhekeka kwaye zikrazuke (ezikwabizwa ngokuba yi -schistocytes ).
• Uvavanyo lweBilirubin: IBilirubin yinkunkuma eveliswa ziiseli ezibomvu zegazi ezonakeleyo. Amanqanaba aphezulu ebilirubin yenye yeempawu zokuba usenokuba ne-TTP.
• Uvavanyo lokusebenza kwezintso: Olu vavanyo lunokunceda ugqirha wakho abone ukuba izintso zakho zisebenza kakuhle na. Ukuba une-TTP, usenokuba neeseli zegazi okanye iiproteni kumchamo wakho.
• Uvavanyo lwe-Lactate dehydrogenase (LDH): I-Lactate dehydrogenase (LDH) yi-enzyme ekhutshwayo xa izicubu zomzimba wakho zonakele. I-TTP ibangela amanqanaba aphezulu e-LDH egazini.

Ziziphi iindlela zonyango ze-TTP?

Kwiimeko ezininzi, oogqirha basebenzisa unyango lwe-plasma ukunyanga i-TTP. Ukongeza, kukho unyango ngamayeza.

Unyango lwePlasma

Ezona ndlela zonyango zixhaphakileyo zezi:

• Ukutshintshiselana ngeplasma: Oku kuquka ukususa iplasma yakho engaqhelekanga (inxalenye engamanzi egazini lakho) uze uyifake endaweni yayo iplasma evela kumnikeli ophilileyo. Olu lunyango olusindisa ubomi kubantu abane-TTP efunyenweyo.
• IiCorticosteroids: La mayeza anceda ukulawula i-TTP ebukhali, ukunciphisa iimpawu, kunye nokunciphisa ukudumba.

Unyango lwe-plasma lunikezelwa esibhedlele kwaye lunokuqhubeka kangangeentsuku okanye iiveki de iimpawu zakho ziphele.

Amayeza

Kukho amayeza athile anokunciphisa okanye ayeke ukwakheka kwee-antibodies ezichasene ne-enzyme ye-ADAMTS13 (ii-anti-ADAMTS13 antibodies). Amanye amayeza asetyenziswa ngoogqirha ukunyanga i-TTP ngala:

• Iiglucocorticoids: Olu nyango luthintela amajoni akho omzimba. Oku kunokuba luncedo kwi-TTP.
• I-Caplacizumab: Eli yeza linyanga i-TTP ebukhali. Linyusa inani leeplatelet ngokukhawuleza kunokutshintshana kweplasma yodwa, linceda ekubuyiseleni isifo ngokukhawuleza.
• I-Rituximab: Olu nyango lunceda ukuthintela ukuba esi sifo singaphindi sibuye emva konyango lwe-corticosteroids kunye notshintsho lwe-plasma.
• I-ADAMTS13 ephinda isetyenziselwe ukwenziwa: Olu nyango lubuyisela i-enzyme ye-ADAMTS13. Luvunyiwe yi-U.S. Food and Drug Administration (FDA) ukuba lusetyenziswe ngokukhawuleza kubantu abane-TTP ezuzwe njengelifa. Ngamanye amaxesha, oogqirha bayayisebenzisa ukuthintela ukopha kakhulu. Uphando luyaqhubeka ukuze babone ukuba iluncedo kangakanani kwi-TTP elawulwa ngamajoni omzimba.

Utyando

Kunqabile kakhulu, ugqirha wakho unokucebisa ukuba kususwe i-spleen yakho ngotyando (i-spleenectomy). Oku kwenziwa kuphela ukuba une-TTP enzima engaphenduli kakuhle kunyango okanye ukuba ihlala iphindaphinda.

Kuthatha ixesha elingakanani ukuchacha emva konyango lwe-TTP?

Ixesha lokuchacha lixhomekeke kwizinto ezininzi. Ubunzima bemeko yakho kunye nohlobo lonyango olufumanayo lunokuchaphazela oku. Uninzi lwabantu abafumana unyango lwe-plasma lwe-TTP bathatha iintsuku ezimbalwa ukuya kwiiveki ezimbalwa ukuze baphile. Abantu abafakelwe i-splenectomy badla ngokuthatha malunga neeveki ezine ukuya kwezintandathu ukuze baphile.

Ndingambona nini ugqirha xa ndicinga ukuba ndine-TTP?

Iimpawu ze-TTP zihlala zinzima kangangokuba zifuna ukuya kwigumbi likaxakeka. Ukuba ukrokrela ukuba une-TTP, ungalindi ukuya kubona ugqirha oqhelekileyo - unyango olukhawulezileyo lubalulekile!Ubuchule beengcali zegazi kunye neengcali zonyango oluphezulu bubalulekile ekulawuleni i-TTP ebukhali.

Yintoni onokuyilindela xa uphila ne-TTP? Ithini ingqikelelo yokuchacha?

I-TTP yayiyeyona sifo sibulalayo. Kodwa ngonyango lwangethuba, izinga lokufa liye lehla laya kwi-10%. Ekubeni esi sifo sinokuphinda sibuye, kubalulekile ukuhlala unxibelelene nogqirha wakho wegazi nokuba sele ufumene unyango. Ukujonga rhoqo amanqanaba akho e-ADAMTS13 kunokunceda ekufumaneni naluphi na utshintsho kwangethuba. Ukuba amanqanaba e-ADAMTS13 aphantsi ngaphambi kokuba iimpawu zibuye, unyango lokuthintela olufana neRituximab lunokunceda ekuthinteleni isifo ukuba singaphindi sibuye kwangaphambi kokuba siqale.

Ingakanani izinga lokusinda kwe-TTP?

Ngaphandle konyango, ngumntu omnye kuphela kwabalishumi abane-TTP osindayo. Kodwa ngonyango olufanelekileyo, abantu abasibhozo ukuya kwabasithoba kwabalishumi banokusinda.

Kungoko ke kubalulekile ukufuna unyango ukuba uneempawu. Kubalulekile nokulandela imiyalelo kagqirha wakho malunga nendlela yokuzinyamekela emva kokuba uxilongiwe.

Ngonyango olufanelekileyo, uninzi lwabantu abane-Thrombotic Thrombocytopenic Purpura (TTP) baphila ubomi obuqhelekileyo. Ugqirha wakho uza kuchaza ukuba kufuneka wenze uvavanyo kangaphi ukuze ajonge imeko yakho. Uza kukuxelela nokuba ufuna unyango oluqhelekileyo ukunciphisa umngcipheko wokopha kunye nokuqhekeka kwegazi.

Okwangoku, ukuba uneempawu ze-TTP, funa unyango ngokukhawuleza. I-TTP yingxamiseko yezonyango. Kodwa unyango lwangethuba lunokusindisa ubomi bakho.

Ngoko ke, yintoni eyona nto ibalulekileyo ekufuneka uyikhumbule kwinto esithethe ngayo?

Kulungile, sithethe kakhulu nge-TTP (Thrombotic Thrombocytopenic Purpura). Ezona zinto zibalulekileyo ekufuneka uzikhumbule zezi:

• I-TTP sisifo segazi esingaqhelekanga kodwa esibi kakhulu apho amahlwili egazi akhiwa kwimithambo yegazi emincinci yomzimba.
• Ukuba ufumana iimpawu ezifana nokudideka ngequbuliso, intloko ebuhlungu kakhulu, amabala amfusa eluswini, okanye ukuphaphatheka, kusenokwenzeka ukuba yi-TTP. Ngoko ke musa ukuchitha ixesha kwaye ubone ugqirha ngoko nangoko.
• Oku akuyi kuphola ngokwako. Unyango olungxamisekileyo ngokuqinisekileyo luyafuneka.
• I-TTP inokunyangeka ukuba ifunyaniswe kwangethuba kwaye inyangwa ngokufanelekileyo. Ngonyango oluphambili olufumanekayo namhlanje, amathuba okusindisa ubomi aphezulu kakhulu.
• Nokuba sele ufumene unyango, landela imiyalelo kagqirha uze uvavanywe. Oku kuya kunceda ukuthintela ukuba esi sifo singaphindi sibuye.

Ukuba wena okanye umntu omaziyo urhanela into efana nale, musani ukoyika. Kodwa thabatha amanyathelo ngokukhawuleza. Ukufuna ingcebiso kagqirha yeyona nto ilungileyo onokuyenza. Hlala usempilweni!

I- Thrombotic Thrombocytopenic Purpura, i-TTP, isifo segazi, amahlwili egazi, iiplatelets, iimpawu, unyango