Uvavanyo lwe-lupus anticoagulant (LA) luyinxalenye ebalulekileyo yovavanyo lwelabhoratri lwee-antiphospholipid antibodies kwaye luye lwacetyiswa ukuba lusetyenziswe kwiimeko ezahlukeneyo zeklinikhi, ezinje ngokuxilongwa kwe-antiphospholipid syndrome (APS) kunye ne-systemic lupus erythematosus (SLE), uvavanyo lomngcipheko we-venous thromboembolism (VTE), kunye nengcaciso yexesha elingachazwanga le-activated partial thromboplastin (APTT). Eli nqaku liza kukunceda uqhelane nokuba yintoni i-antiphospholipid syndrome (APS).
I-Antiphospholipid syndrome (APS) sisifo se-autoimmune esineziganeko ze-vascular thrombotic eziphindaphindayo, ukuqhomfa okuphindaphindayo, i-thrombocytopenia, njl.njl. njengeempawu eziphambili zeklinikhi, ezihamba ne-resistant medium and high titer positive antiphospholipid antibody spectrum (aPLs). Ihlala yahlulwe kwi-APS yokuqala kunye ne-secondary APS, eyokugqibela ilandela izifo zezicubu ezinxibelelanayo ezifana ne-systemic lupus erythematosus (SLE) kunye ne-Sjögren's syndrome. Iimpawu zeklinikhi ze-APS ziyinkimbinkimbi kwaye zahlukahlukene, kwaye zonke iinkqubo zomzimba zinokuchaphazeleka, apho uphawu olubonakalayo luyi-vascular thrombosis. I-pathogenesis ye-APS kukuba i-aPL ejikelezayo ibopha kwi-phospholipids yomphezulu weseli kunye neeproteni ezibopha i-phospholipid, isebenze iiseli ze-endothelial, ii-PLT kunye ne-wBc, ekhokelela kwiziganeko ze-vascular thrombotic kunye neengxaki zokukhulelwa, kwaye ikhuthaza ukwenzeka kwezinye iingxaki ze-autoimmune kunye nokudumba. Nangona i-aPL ibangela izifo, i-thrombosis yenzeka ngamaxesha athile kuphela, nto leyo ebonisa ukuba "ukuhlaselwa kwesibini" kwexesha elifutshane njengosulelo, ukudumba, utyando, ukukhulelwa kunye nezinye izinto ezibangela i-thrombosis zibalulekile kwinkqubo ye-thrombosis.
Enyanisweni, i-APS ayiqhelekanga. Izifundo zibonise ukuba ama-25% ezigulana ezinestroke esingachazwanga ezingaphantsi kweminyaka engama-45 ubudala zine-aPLs, ama-14% ezigulana ezine-venous thrombosis ephindaphindayo zine-aPLs, kwaye ama-15% ukuya kuma-20% ezigulana ezingamabhinqa ezine-recurrent pregnancy backup loss zine-aPLs. Ngenxa yokungaqondi kakuhle olu hlobo lwesifo ngabagqirha, ixesha eliqhelekileyo lokuxilonga i-APS limalunga neminyaka eyi-2.9. I-APS idla ngokuxhaphaka kakhulu kubafazi, kunye nomlinganiselo wabasetyhini: amadoda oyi-9:1, kwaye ixhaphake kakhulu kubantu abancinci nabaphakathi, kodwa ama-12.7% ezigulana angaphezulu kweminyaka engama-50 ubudala.
1. Iziganeko zeThrombotic
Iimpawu zeklinikhi ze-vascular thrombosis kwi-APS zixhomekeke kuhlobo, indawo kunye nobukhulu bemithambo yegazi echaphazelekayo, kwaye zinokubonakala njengemithambo yegazi enye okanye emininzi echaphazelekayo. I-Venous thromboembolism (VTE) ixhaphake kakhulu kwi-APS, ixhaphake kakhulu kwimithambo enzulu yemilenze esezantsi. Ingachaphazela ne-intracranial venous sinuses, i-retina, i-subclavian, isibindi, izintso, kunye ne-vena cava ephezulu nengaphantsi. I-APS arterial thrombosis (AT) ixhaphake kakhulu kwimithambo yegazi engaphakathi kwentloko, kwaye ingachaphazela nemithambo yegazi engaphakathi kwentloko, imithambo yegazi engaphakathi kwentloko, imithambo yegazi engaphakathi kwentloko, njl. Ukongeza, abaguli be-APS banokuba ne-microvascular thrombosis eluswini, emehlweni, entliziyweni, emiphungeni, kwizintso nakwamanye amalungu omzimba. Uhlalutyo lwe-Meta lufumanise ukuba i-lupus anticoagulant (LA) positivity inomngcipheko omkhulu we-thromboembolism kune-antiphospholipid antibodies (acL); Izifundo zeklinikhi zibonise ukuba izigulane ze-APS ezine-aPL [oko kukuthi, i-LA, i-aCL, ii-antibodies ze-glycoprotein I (αβGPI) ezibonisa umngcipheko ophezulu we-thrombosis, kuquka izinga le-thrombosis le-44.2% kwiminyaka eli-10.
2. Ukukhulelwa okubangelwa zizifo
I-pathophysiology yeempawu ze-APS zokubelekisa iyinkimbinkimbi ngokulinganayo kwaye inokwahluka ngokwesigaba sokukhulelwa, nto leyo ekhokelela ekuhlukeni kweempawu zeklinikhi ezibonwayo. Ukudumba, ukusebenza okuphelelisayo, kunye ne-thrombosis ye-placenta zonke zithathwa njengezinto ezibangela izifo ze-APS yokubelekisa. Ukukhulelwa okubangelwa yi-APS yenye yezizathu ezimbalwa ezinokuthintelwa nokunyangwa, kwaye ulawulo olufanelekileyo lunokuphucula ngempumelelo iziphumo zokukhulelwa. Uhlalutyo lwe-meta olupapashwe ngo-2009 lufumanise ukuba ubukho be-LA kunye ne-aCL bunxulunyaniswa kakhulu nokufa kwe-fetus kwiiveki ezingaphezulu kwe-10 zokukhulelwa; uphononongo olucwangcisiweyo lwakutshanje kunye nohlalutyo lwe-meta lufumanise ukuba i-LA positivity yayinxulunyaniswa kakhulu nokufa kwe-fetus. Kwizigulane ezaziwayo ukuba zine-APS, umngcipheko wokufa kwe-fetus usephezulu ukuya kwi-10% ukuya kwi-12% nangona unyango oluqhelekileyo lwe-heparin kunye ne-aspirin yedosi ephantsi. Kwizigulane ze-APS ezineempawu ezinzima ze-preeclampsia okanye ukungasebenzi kakuhle kwe-placenta, ubukho be-LA kunye ne-aCL bunxulunyaniswa kakhulu ne-preeclampsia; ukuphuphuma kwesisu okuphindaphindiweyo kwangethuba (ngaphantsi kweeveki ezili-10 zokukhulelwa) yingxaki yokubelekisa ehlala icinga ukuba kungenzeka i-APS.
1. I-Thrombocytopenia
I-Thrombocytopenia yenye yezona mpawu ziqhelekileyo zezonyango kwizigulana ze-APS, kwaye ixhaphake ngama-20% ~ 53%. Ngokwesiqhelo, i-SLE secondary APS ithambekele kakhulu kwi-thrombocytopenia kune-APS yokuqala. Ubungakanani be-thrombocytopenia kwizigulana ze-APS budla ngokuba bubuncinci okanye buphakathi. I-pathogenesis enokwenzeka ibandakanya ii-aPLs ezibopha ngokuthe ngqo kwiiplatelets ukuze zisebenze kwaye zihlanganise iiplatelets, ukusetyenziswa kwe-thrombotic microangiopathy, ukusetyenziswa kwe-thrombosis eninzi, ukugcinwa okuphezulu kwi-spleen, kunye neempendulo ezimbi ezinxulumene namayeza e-anticoagulant amelwe yi-heparin. Ngenxa yokuba i-thrombocytopenia inokunyusa umngcipheko wokopha, oogqirha banenkxalabo malunga nokusetyenziswa konyango lwe-antithrombotic kwizigulana ze-APS ezine-thrombocytopenia, kwaye bakholelwa ngempazamo ukuba i-APS thrombocytopenia inokunciphisa umngcipheko wokuphinda kwenzeke kweziganeko ze-thrombotic kwizigulana. Enyanisweni, ngokuchaseneyo, izifundo zibonise ukuba umngcipheko wokuphinda kwenzeke kweziganeko ze-thrombotic kwizigulana ze-APS ezine-thrombocytopenia uyanda kakhulu, ngoko ke kufuneka unyangwe ngakumbi.
2. I-CAPS sisifo esingaqhelekanga, esisongela ubomi esibonakaliswa zii-vascular embolisms ezininzi (≥3) kwinani elincinci lezigulane ze-APS kwixesha elifutshane (≤iintsuku ezi-7), ngokuqhelekileyo zine-titers eziphezulu, ezichaphazela imithambo yegazi emincinci, kunye nokuqinisekiswa kwe-histopathological kwe-thrombosis kwimithambo yegazi emincinci. I-APL positivity iyaqhubeka kwiiveki ezili-12, ibangela ukungasebenzi kakuhle kwamalungu amaninzi kunye nomngcipheko wokufa, owaziwa ngokuba yi-catastrophical antiphospholipid syndrome. Ukwanda kwayo kumalunga ne-1.0%, kodwa izinga lokufa liphezulu ukuya kuma-50% ~ 70%, rhoqo ngenxa yestroke, i-encephalopathy, ukopha kwegazi, usulelo, njl. I-pathogenesis yayo enokwenzeka kukwenziwa kwe-thrombotic storm kunye ne-inflammatory storm kwixesha elifutshane.
Ii-aPL ligama eliqhelekileyo leqela lee-autoantibodies ezinee-phospholipids kunye/okanye iiproteni ezibopha i-phospholipid njengee-antigens ezijoliswe kuzo. Ii-aPL zifumaneka ikakhulu kwizigulana ezinezifo zokuzikhusela komzimba ezifana ne-APS, i-SLE, kunye ne-Sjögren's syndrome. Zezona zimpawu zibalaseleyo ze-APS kwilabhoratri kwaye zezona zinto ziphambili ezixela umngcipheko weziganeko ze-thrombotic kunye nokukhulelwa kwe-pathological kwizigulane ze-APS. Phakathi kwazo, i-lupus anticoagulant (LA), i-anticardiolipin antibodies (aCL), kunye ne-anti-β-glycoprotein I (αβGPⅠ) antibodies, njengezalathisi zelebhu kumgangatho wokuhlelwa kwe-APS, zisetyenziswe kakhulu kwizonyango kwaye ziye zaba lolunye lovavanyo oluqhelekileyo lwe-autoantibody kwiilabhoratri zeklinikhi.
Xa kuthelekiswa ne-aCL kunye ne-anti-βGPⅠ antibodies, i-LA inolwalamano oluqinileyo ne-thrombosis kunye nokukhulelwa okubangelwa yi-pathological. I-LA inomngcipheko ophezulu we-thrombosis kune-acL. Kwaye inxulumene kakhulu nokuphuphuma kwesisu ngexesha lokukhulelwa ngaphezulu kweeveki ezili-10. Ngamafutshane, i-LA ehlala ikho yeyona nto iphambili exela umngcipheko we-thrombosis kunye nokugula kokukhulelwa.
I-LA luvavanyo olusebenzayo olumisela ukuba umzimba une-LA ngokusekelwe kwinto yokuba i-LA inokwandisa ixesha lokujiya kweendlela ezahlukeneyo ezixhomekeke kwi-phospholipid kwi-vitro. Iindlela zokufumanisa i-LA ziquka:
1. Uvavanyo lokuhlola: kubandakanya ixesha letyhefu yeviper ediluted (dRVVT), ixesha le-activated partial thromboplastin (APTT), indlela yexesha le-silica coagulation, ixesha le-giant snake coagulation kunye nexesha le-snake vein enzyme. Okwangoku, izikhokelo zokufumanisa i-aPLs zamazwe ngamazwe ezifana ne-International Society on Thrombosis and Haemostasis (ISTH) kunye ne-Clinical Laboratory Standards Institute (CLSI) zicebisa ukuba i-LA ifunyanwe ngeendlela ezimbini ezahlukeneyo ze-coagulation. Phakathi kwazo, i-dRVVT kunye ne-APTT zezona ndlela zokufumanisa ezisetyenziswa kakhulu kwihlabathi liphela. Ngokwesiqhelo i-dRVVT isetyenziswa njengendlela yokuqala yokukhetha, kwaye i-APTT enobuthathaka ngakumbi (i-phospholipids ephantsi okanye i-silica njenge-activator) isetyenziswa njengendlela yesibini.
2. Uvavanyo lokuxuba: Iplasma yesigulana ixutywa neplasma ephilileyo (1:1) ukuqinisekisa ukuba ixesha elide lokujiya kwegazi alibangelwa kukungabikho kwezinto ezijiya igazi.
3. Uvavanyo lokuqinisekisa: Uxinzelelo okanye ukwakheka kweephospholipids kuyatshintshwa ukuqinisekisa ubukho be-LA.
Kubalulekile ukuqaphela ukuba isampuli efanelekileyo ye-LA kufuneka iqokelelwe kwizigulana ezingakhange zifumane unyango lwe-anticoagulant, kuba izigulana ezinyangwa nge-warfarin, i-heparin, kunye ne-anticoagulant entsha yomlomo (efana ne-rivaroxaban) zinokuba neziphumo zovavanyo lwe-LA ezingalunganga; ngoko ke, iziphumo zovavanyo lwe-LA zezigulana ezifumana unyango lwe-anticoagulant kufuneka zitolikwe ngononophelo. Ukongeza, uvavanyo lwe-LA kufuneka lutolikwe ngononophelo kwimeko yeklinikhi ebukhali, kuba ukuphakama okukhulu kumanqanaba eeprotheyini ze-C-reactive nako kunokuphazamisa iziphumo zovavanyo.
I-APS sisifo esihlasela amasosha omzimba esineziganeko ze-vascular thrombotic eziphindaphindayo, ukukhupha isisu okuphindaphindayo, i-thrombocytopenia, njl.njl. njengeempawu eziphambili zeklinikhi, ezihamba nee-aPLs eziphakathi neziphezulu.
I-APS yenye yezona zinto zimbalwa zinokunyangeka zokukhulelwa okubangelwa zizifo. Ulawulo olufanelekileyo lwe-APS lunokuphucula ngempumelelo iziphumo zokukhulelwa.
Kumsebenzi weklinikhi, ii-APS kufuneka zibandakanye nezigulana ezineempawu zeklinikhi ezinxulumene ne-aPLs ezifana ne-liveo reticularis, i-thrombocytopenia, kunye nesifo se-valve yentliziyo, kunye nezo zihlangabezana neemfuno zokuhlelwa kweklinikhi kwaye zine-titers ezisezantsi ze-aPLs eziqhubekayo. Ezi zigulana zikwanomngcipheko weziganeko ze-thrombotic kunye nokukhulelwa okubangelwa yi-pathological.
Iinjongo zonyango ze-APS ziquka ikakhulu ukuthintela i-thrombosis kunye nokuphepha ukungaphumeleli kokukhulelwa.
Iireferensi
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, et al. Izikhokelo zokuxilonga kunye nonyango lwe-antiphospholipid syndrome[J]. Ijenali yaseTshayina yeMithi yangaphakathi
[2] Bu Jin, Liu Yuhong. Inkqubela phambili ekuxilongweni nasekunyangweni kwe-antiphospholipid syndrome[J]. Ijenali yezonyango zangaphakathi zeklinikhi
[3] Isikhokelo se-BSH Izikhokelo zophando kunye nolawulo lwe-antiphospholipid syndrome.
[4] IKomiti yeThrombosis kunye neHemostasis yeZiko laseTshayina leZibhedlele zoPhando. Imvumelwano malunga nokubekwa emgangathweni kokufunyanwa kunye nokunikwa kwengxelo kwe-lupus anticoagulant [J].
Ikhadi leshishini
I-WeChat yesiTshayina