Ukuhlolwa kwe-lupus anticoagulant (LA) kuyingxenye ebalulekile yokuhlolwa kwelabhorethri kwama-antiphospholipid antibodies futhi kuye kwanconywa ukuthi kusetshenziswe ezimweni ezahlukahlukene zezokwelapha, njengokuxilongwa kwe-antiphospholipid syndrome (APS) kanye ne-systemic lupus erythematosus (SLE), ukuhlolwa kobungozi be-venous thromboembolism (VTE), kanye nencazelo yesikhathi se-thromboplastin esingasebenzi kahle esingachazwanga (APTT). Lesi sihloko sizokusiza ukuthi ujwayelane nokuthi iyini i-antiphospholipid syndrome (APS).
I-Antiphospholipid syndrome (APS) yisifo sokuzivikela komzimba esinezigameko ze-vascular thrombotic eziphindaphindayo, ukukhipha isisu okuphindaphindiwe okuzenzakalelayo, i-thrombocytopenia, njll. njengezibonakaliso eziyinhloko zemitholampilo, ezihambisana ne-persistent medium and high titer positive antiphospholipid antibody spectrum (aPLs). Ngokuvamile ihlukaniswa ibe yi-APS eyinhloko kanye ne-APS yesibili, eyokugcina elandela kakhulu izifo zezicubu ezixhumene njenge-systemic lupus erythematosus (SLE) kanye ne-Sjögren's syndrome. Izibonakaliso zemitholampilo ze-APS ziyinkimbinkimbi futhi ziyahlukahluka, futhi zonke izinhlelo zomzimba zingathinteka, kanti ukubonakaliswa okuvelele kakhulu yi-vascular thrombosis. I-pathogenesis ye-APS ukuthi i-aPL ejikelezayo ibopha kuma-phospholipids asebusweni beseli kanye namaprotheni ahlanganisa i-phospholipid, isebenze amaseli e-endothelial, ama-PLT kanye ne-wBc, okuholela ezigamekweni ze-vascular thrombotic kanye nezinkinga zokubeletha, futhi ikhuthaza ukwenzeka kwezinye izinkinga ze-autoimmune kanye nokuvuvukala. Nakuba i-aPL ibangela izifo, i-thrombosis yenzeka ngezikhathi ezithile kuphela, okubonisa ukuthi "izigameko zesibili" zesikhashana njengokutheleleka, ukuvuvukala, ukuhlinzwa, ukukhulelwa nezinye izinto ezibangela i-thrombosis zibalulekile enkambisweni ye-thrombosis.
Eqinisweni, i-APS ayivamile. Izifundo zibonise ukuthi ama-25% eziguli ezine-stroke engachazeki ezingaphansi kweminyaka engama-45 zine-aPL positive, ama-14% eziguli ezinezehlakalo ze-venous thrombosis eziphindaphindayo ane-aPL positive, kanti ama-15% kuya kuma-20% eziguli zesifazane ezine-aPL positive yokulahlekelwa ukukhulelwa aphindaphindayo ane-aPL positive. Ngenxa yokungaqondi lolu hlobo lwesifo odokotela abaluqondi, isikhathi esimaphakathi sokuxilongwa kwe-APS sicishe sibe yiminyaka engu-2.9. I-APS ivame kakhulu kwabesifazane, ngesilinganiso sabesifazane: abesilisa esingu-9:1, futhi ivame kakhulu kubantu abasebasha nasebekhulile, kodwa ama-12.7% eziguli aneminyaka engaphezu kwengu-50 ubudala.
1. Imicimbi ye-Thrombotic
Izimpawu zemitholampilo ze-vascular thrombosis ku-APS zincike ohlotsheni, indawo kanye nosayizi wemithambo yegazi ethintekile, futhi zingabonakala njengemithambo yegazi eyodwa noma eminingi ehilelekile. I-Venous thromboembolism (VTE) ivame kakhulu ku-APS, ivame kakhulu emithanjeni ejulile yezingxenye ezingezansi. Ingathinta futhi ama-sinuses emithambo yegazi angaphakathi kwekhanda, i-retina, i-subclavian, isibindi, izinso, kanye ne-vena cava ephezulu nengaphansi. I-APS arterial thrombosis (AT) ivame kakhulu emithanjeni yegazi engaphakathi kwekhanda, futhi ingathinta nemithambo yezinso, imithambo yegazi yenhliziyo, imithambo yegazi ye-mesenteric, njll. Ngaphezu kwalokho, iziguli ze-APS zingase zibe ne-microvascular thrombosis esikhumbeni, emehlweni, enhliziyweni, emaphashini, ezinso nakwezinye izitho zomzimba. Ukuhlaziywa kwe-Meta kuthole ukuthi i-lupus anticoagulant (LA) positivity inengozi enkulu ye-thromboembolism kune-antiphospholipid antibodies (acL); Izifundo zezokwelapha zibonise ukuthi iziguli ze-APS ezine-aPL enhle [okungukuthi, i-LA, i-aCL, ama-antibodies e-glycoprotein I (αβGPI) positivity] zibonisa ingozi ephezulu ye-thrombosis, okuhlanganisa nesilinganiso se-thrombosis esingu-44.2% zingakapheli iminyaka eyi-10.
2. Ukukhulelwa okubangelwa yizifo
I-pathophysiology yezibonakaliso ze-APS zokubeletha iyinkimbinkimbi ngokulinganayo futhi ingahluka ngokuya ngesigaba sokukhulelwa, okuholela ekuhlukeni kwezici zomtholampilo ezibonwayo. Ukuvuvukala, ukusebenza okuhambisanayo, kanye ne-thrombosis ye-placenta konke kubhekwa njengezimbangela ze-APS yokubeletha. Ukukhulelwa kwe-pathological okubangelwa yi-APS kungenye yezimbangela ezimbalwa ezingavinjelwa futhi zelashwe, futhi ukuphathwa okufanele kungathuthukisa imiphumela yokukhulelwa ngempumelelo. Ukuhlaziywa kwe-meta okwanyatheliswa ngo-2009 kwathola ukuthi ukuba khona kwe-LA ne-aCL kwakuhlotshaniswa kakhulu nokufa komntwana osesiswini emasontweni angaphezulu kwe-10 okukhulelwa; ukubuyekezwa okuhlelekile kwakamuva kanye nokuhlaziywa kwe-meta nakho kwathola ukuthi i-LA positivity yayihlotshaniswa eduze nokufa komntwana osesiswini. Ezigulini ezaziwa ukuthi zine-APS, ingozi yokufa komntwana osesiswini isaphakeme njenge-10% kuya ku-12% ngisho nangokwelashwa okujwayelekile kwe-heparin kanye ne-aspirin ephansi. Ezigulini ze-APS ezinezimpawu ezinzima ze-preeclampsia noma ukungasebenzi kahle kwe-placenta, ukuba khona kwe-LA ne-aCL kuhlotshaniswa kakhulu ne-preeclampsia; ukuphuphuma kwesisu okuphindaphindiwe kusenesikhathi (ngaphansi kwamasonto ayi-10 okukhulelwa) kuyinkinga yokukhulelwa evame ukucabanga ukuthi kungenzeka i-APS.
1. I-Thrombocytopenia
I-Thrombocytopenia ingenye yezimpawu ezivamile zemitholampilo zeziguli ze-APS, enesilinganiso esingu-20% ~ 53%. Ngokuvamile, i-SLE secondary APS ithambekele kakhulu ku-thrombocytopenia kune-APS eyinhloko. Izinga le-thrombocytopenia ezigulini ze-APS livame ukuba lincane noma liphakathi. I-pathogenesis engaba khona ihlanganisa ama-aPL abophe ngqo kuma-platelet ukuze kusebenze futhi kuhlanganiswe ama-platelet, ukusetshenziswa kwe-thrombotic microangiopathy, ukusetshenziswa kwe-thrombosis eningi, ukugcinwa okukhulu kwe-spleen, kanye nokusabela okubi okuhlobene nemithi yokulwa nokugabha kwegazi emelelwa yi-heparin. Ngenxa yokuthi i-thrombocytopenia ingandisa ingozi yokopha, odokotela banezinkinga ezithile mayelana nokusetshenziswa kokwelashwa kwe-antithrombotic ezigulini ze-APS ezine-thrombocytopenia, futhi bakholelwa ngephutha ukuthi i-APS thrombocytopenia inganciphisa ingozi yokuphinda kwenzeke kwezehlakalo ze-thrombotic ezigulini. Eqinisweni, ngokuphambene nalokho, izifundo zibonise ukuthi ingozi yokuphinda kwenzeke kwezehlakalo ze-thrombotic ezigulini ze-APS ezine-thrombocytopenia iyanda kakhulu, ngakho-ke kufanele iphathwe ngenkuthalo.
2. I-CAPS yisifo esingavamile, esisongela ukuphila esibonakala ngokuvaleka kwemithambo yegazi eminingi (≥3) enanini elincane leziguli ze-APS esikhathini esifushane (≤izinsuku ezingu-7), ngokuvamile esinama-titers aphezulu, athinta imithambo yegazi emincane, kanye nokuqinisekiswa kwe-histopathological kwe-thrombosis emithanjeni yegazi emincane. Ukutholakala kwe-APL kuqhubeka zingakapheli amasonto angu-12, okubangela ukwehluleka kwezitho eziningi kanye nengozi yokufa, okwaziwa ngokuthi i-antiphospholipid syndrome eyingozi. Ukwanda kwayo kucishe kube ngu-1.0%, kodwa izinga lokufa liphezulu lifinyelela ku-50% ~ 70%, ngokuvamile ngenxa yesifo sohlangothi, i-encephalopathy, ukopha kwegazi, ukutheleleka, njll. I-pathogenesis yayo engaba khona ukwakheka kwesiphepho se-thrombotic kanye nesiphepho sokuvuvukala esikhathini esifushane.
Ama-aPL yigama elijwayelekile leqembu lama-autoantibodies anama-phospholipid kanye/noma amaprotheni abopha i-phospholipid njengama-antigen aqondiwe. Ama-aPL atholakala kakhulu ezigulini ezinezifo zokuzivikela komzimba ezifana ne-APS, i-SLE, kanye ne-Sjögren's syndrome. Ayezimpawu zelebhu eziphawulekayo ze-APS kanye nezibikezeli eziyinhloko zengozi yezehlakalo ze-thrombotic kanye nokukhulelwa kwe-pathological ezigulini ze-APS. Phakathi kwawo, ama-lupus anticoagulant (LA), ama-anticardiolipin antibodies (aCL), kanye nama-anti-β-glycoprotein I (αβGPⅠ) antibodies, njengezinkomba zelebhu kuzinga lokuhlukaniswa kwe-APS, asetshenziswe kabanzi emitholampilo futhi abe ngenye yezivivinyo ezivame kakhulu ze-autoantibody emalabhorethri emitholampilo.
Uma kuqhathaniswa nama-antibodies e-aCL kanye ne-anti-βGPⅠ, i-LA inobudlelwano obuqinile ne-thrombosis kanye nokukhulelwa okubangelwa yi-pathological. I-LA inengozi ephezulu ye-thrombosis kune-acL. Futhi ihlobene kakhulu nokuphuphuma kwesisu ekukhulelweni okungaphezu kwamasonto ayi-10. Ngamafuphi, i-LA ehlala njalo ine-positive iyona ephumelela kakhulu yokubikezela ingozi ye-thrombosis kanye nokugula kokukhulelwa.
I-LA iyisivivinyo esisebenzayo esinquma ukuthi umzimba une-LA yini ngokusekelwe eqinisweni lokuthi i-LA ingandisa isikhathi sokujiya kwezindlela ezahlukene ezincike ku-phospholipid in vitro. Izindlela zokuthola i-LA zifaka:
1. Ukuhlolwa kokuhlola: kufaka phakathi isikhathi se-viper venom esincishisiwe (i-dRVVT), isikhathi se-activated partial thromboplastin (i-APTT), indlela yesikhathi se-silica coagulation, isikhathi se-giant snake coagulation kanye nesikhathi se-snake vein enzyme. Njengamanje, iziqondiso zokuthola ama-aPL zomhlaba wonke njenge-International Society on Thrombosis and Haemostasis (ISTH) kanye ne-Clinical Laboratory Standards Institute (CLSI) zincoma ukuthi i-LA itholakale ngezindlela ezimbili ezihlukene ze-coagulation. Phakathi kwazo, i-dRVVT kanye ne-APTT yizindlela zokuthola ezisetshenziswa kakhulu emhlabeni jikelele. Ngokuvamile i-dRVVT isetshenziswa njengendlela yokuqala yokukhetha, kanti i-APTT ezwela kakhulu (ama-phospholipid aphansi noma i-silica njenge-activator) isetshenziswa njengendlela yesibili.
2. Ukuhlolwa kokuxuba: I-plasma yesiguli ixutshwa ne-plasma enempilo (1:1) ukuqinisekisa ukuthi isikhathi eside sokujiya kwegazi asibangelwa ukuntuleka kwezinto ezijiya igazi.
3. Ukuhlolwa kokuqinisekisa: Ukuhlushwa noma ukwakheka kwama-phospholipid kuyashintshwa ukuqinisekisa ukuba khona kwe-LA.
Kubalulekile ukuqaphela ukuthi isampula efanelekile ye-LA kufanele iqoqwe ezigulini ezingazange zithole ukwelashwa kwe-anticoagulant, ngoba iziguli ezelashwe nge-warfarin, i-heparin, kanye ne-anticoagulant entsha yomlomo (njenge-rivaroxaban) zingase zibe nemiphumela yokuhlolwa kwe-LA engalungile; ngakho-ke, imiphumela yokuhlolwa kwe-LA yeziguli ezithola ukwelashwa kwe-anticoagulant kufanele ihunyushwe ngokucophelela. Ngaphezu kwalokho, ukuhlolwa kwe-LA kufanele futhi kuhunyushwe ngokucophelela endaweni yokwelapha ebukhali, ngoba ukuphakama okukhulu kwamazinga e-C-reactive protein nakho kungaphazamisa imiphumela yokuhlolwa.
I-APS yisifo sokuzivikela komzimba esinezigameko ze-vascular thrombotic eziphindaphindayo, ukukhipha isisu okuphindaphindiwe okuzenzakalelayo, i-thrombocytopenia, njll. njengezibonakaliso eziyinhloko zemitholampilo, ezihambisana neziqu eziphakathi neziphezulu ze-aPL eziqhubekayo.
I-APS ingenye yezimbangela ezimbalwa ezingelapheka zokukhulelwa okubangelwa yizifo. Ukuphathwa kahle kwe-APS kungathuthukisa imiphumela yokukhulelwa ngempumelelo.
Emsebenzini wezokwelapha, ama-APS kufanele afake neziguli ezinezimpawu zemitholampilo ezihlobene ne-aPLs njenge-livedo reticularis, i-thrombocytopenia, nesifo se-valve yenhliziyo, kanye nalabo abahlangabezana nezindinganiso zokuhlukanisa imitholampilo futhi abanezinga eliphansi eliqhubekayo lama-aPLs. Iziguli ezinjalo nazo zinengozi yezehlakalo ze-thrombotic kanye nokukhulelwa kwe-pathological.
Imigomo yokwelapha ye-APS ihlanganisa ikakhulukazi ukuvimbela i-thrombosis nokugwema ukwehluleka kokukhulelwa.
Izinkomba
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, et al. Iziqondiso zokuxilonga kanye nokwelashwa kwe-antiphospholipid syndrome [J]. Ijenali YaseShayina Yezokwelapha Zangaphakathi
[2] Bu Jin, Liu Yuhong. Intuthuko ekuxilongweni nasekwelapheni i-antiphospholipid syndrome[J]. Ijenali Yezokwelapha Zangaphakathi Zemitholampilo
[3] UMHLAHLANDLELA WE-BSH Iziqondiso zokuphenya nokuphathwa kwe-antiphospholipid syndrome.
[4] IKomidi Le-Thrombosis kanye ne-Hemostasis Le-Chinese Society of Research Hospitals. Ukuvumelana ngokwenziwa kwezindinganiso zokutholwa nokubika kwe-lupus anticoagulant [J].
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