Nnwale lupus anticoagulant (LA) bụ akụkụ dị mkpa nke ule ụlọ nyocha maka ọgwụ mgbochi antiphospholipid ma akwadoro ka e jiri ya mee ihe n'ọtụtụ ọnọdụ ahụike dị iche iche, dịka nchọpụta ụlọ nyocha nke ọrịa antiphospholipid (APS) na systemic lupus erythematosus (SLE), nyocha ihe egwu nke thromboembolism venous (VTE), na nkọwa nke oge thromboplastin nke a na-arụ ọrụ ogologo oge (APTT) nke a na-akọwaghị. Isiokwu a ga-enyere gị aka ịmata ihe ọrịa antiphospholipid (APS) bụ.
Ọrịa Antiphospholipid (APS) bụ ọrịa autoimmune nke nwere ihe omume thrombosis vascular ugboro ugboro, ime ime na-agagharị ugboro ugboro, thrombocytopenia, wdg. dị ka ihe ngosipụta ahụike bụ isi, ya na spectrum antiphospholipid antibody spectrum (aPLs) na-adịgide adịgide. A na-ekewakarị ya na APS mbụ na APS nke abụọ, nke ikpeazụ bụ nke abụọ na ọrịa anụ ahụ jikọtara ọnụ dịka lupus erythematosus systemic (SLE) na Sjögren's syndrome. Ihe ngosi ahụike nke APS dị mgbagwoju anya ma dị iche iche, sistemụ niile nke ahụ nwekwara ike imetụta, ihe ngosi kachasị pụta ìhè bụ thrombosis vascular. Ihe kpatara APS bụ na aPL na-agagharị na-ejikọ na phospholipids elu sel na protein phospholipid-binding, na-eme ka mkpụrụ ndụ endothelial rụọ ọrụ, PLTs na wBc, na-eduga na ihe omume thrombotic vascular na nsogbu obstetric, ma na-akwalite mmepe nke nsogbu autoimmune na mbufụt ndị ọzọ. Ọ bụ ezie na aPL bụ ọrịa na-akpata ọrịa, thrombosis na-eme naanị mgbe ụfọdụ, na-egosi na "ọgbụgba nke abụọ" dị mkpirikpi dịka ọrịa, mbufụt, ịwa ahụ, afọ ime na ihe ndị ọzọ na-akpalite dị mkpa na usoro nke thrombosis.
N'eziokwu, APS abụghị ihe a na-ahụkarị. Nnyocha egosila na 25% nke ndị ọrịa nwere ọrịa strok na-enweghị nkọwa nke na-erubeghị afọ 45 bụ ndị nwere aPLs, 14% nke ndị ọrịa nwere ihe omume thrombosis venous ugboro ugboro bụ ndị nwere aPLs, ebe 15% ruo 20% nke ndị ọrịa nwanyị nwere ọnwụ afọ ime ugboro ugboro bụ ndị nwere aPLs. N'ihi enweghị nghọta nke ụdị ọrịa a site n'aka ndị dọkịta, nkezi oge nchọpụta oge APS bụ ihe dị ka afọ 2.9. APS na-abụkarị ihe a na-ahụkarị n'ime ụmụ nwanyị, yana oke nwoke nwanyị: 9:1, ọ na-abụkarịkwa ihe a na-ahụkarị n'etiti ndị ntorobịa na ndị agadi, mana 12.7% nke ndị ọrịa karịrị afọ 50.
1. Ihe omume Thrombotic
Ngosipụta ahụike nke thrombosis vaskụla na APS dabere n'ụdị, ebe na nha nke arịa ọbara emetụtara, a pụkwara igosipụta ya dị ka otu ma ọ bụ ọtụtụ arịa ọbara metụtara. Venous thromboembolism (VTE) na-adịkarị na APS, nke a na-ahụkarị na akwara miri emi nke nsọtụ ala. Ọ nwekwara ike imetụta intracranial venous sinuses, retina, subclavian, imeju, akụrụ, na superior na inferior vena cava. APS arterial thrombosis (AT) na-adịkarị na intracranial artery, ọ nwekwara ike imetụta renal artery, coronary artery, mesenteric artery, wdg. Na mgbakwunye, ndị ọrịa APS nwekwara ike inwe microvascular thrombosis na akpụkpọ ahụ, anya, obi, akpa ume, akụrụ na akụkụ ahụ ndị ọzọ. Meta-analysis chọpụtara na lupus anticoagulant (LA) positivity nwere nnukwu ihe ize ndụ nke thromboembolism karịa antibodies antiphospholipid (acL); Nnyocha ahụike egosila na ndị ọrịa APS nwere aPL dị mma [ya bụ, LA, aCL, glycoprotein I antibodies (αβGPI) positivity] na-egosi nnukwu ihe egwu nke thrombosis, gụnyere ọnụego thrombosis nke 44.2% n'ime afọ 10.
2. Afọ ime na-akpata ọrịa
Ọrịa nke ngosipụta afọ ime nke APS dị mgbagwoju anya ma nwee ike ịdị iche dịka ọkwa afọ ime si dị, na-ebute ọdịiche dị n'etiti njirimara ahụike a hụrụ. A na-ewere mbufụt, mmetụ aka, na thrombosis nke placental dị ka ihe ndị na-akpata ọrịa APS obstetric. Afọ ime nke APS kpatara bụ otu n'ime ihe ole na ole enwere ike igbochi ma gwọọ, njikwa kwesịrị ekwesị nwekwara ike imeziwanye nsonaazụ afọ ime nke ọma. Nnyocha meta nke e bipụtara na 2009 chọpụtara na ọnụnọ nke LA na aCL nwere njikọ dị ukwuu na ọnwụ nwa ebu n'afọ na ihe karịrị izu iri nke afọ ime; nyocha usoro na meta-analysis na nso nso a chọpụtakwara na positivity LA nwere njikọ chiri anya na ọnwụ nwa ebu n'afọ. N'ime ndị ọrịa a maara na ha nwere APS, ihe ize ndụ nke ọnwụ nwa ebu n'afọ ka dị elu ruo 10% ruo 12% ọbụlagodi na ọgwụgwọ ọkọlọtọ nke heparin na obere dose aspirin. Maka ndị ọrịa APS nwere mgbaàmà siri ike nke preeclampsia ma ọ bụ enweghị oke placental, ọnụnọ nke LA na aCL nwere njikọ dị ukwuu na preeclampsia; ime ọpụpụ ugboro ugboro n'oge (<10 izu nke afọ ime) bụ nsogbu afọ ime nke na-atụlekarị ohere nke APS.
1. Thrombocytopenia
Thrombocytopenia bụ otu n'ime ihe ndị a na-ahụkarị n'ọrịa APS, nke nwere ihe dị ka 20% ~ 53%. Ọtụtụ mgbe, SLE secondary APS na-adịkarị mfe ibute thrombocytopenia karịa primary APS. Ọkwa thrombocytopenia na ndị ọrịa APS na-adịkarị obere ma ọ bụ nke na-adịghị ala ala. Nsogbu ndị nwere ike ibute gụnyere aPLs na-ejikọ ozugbo na platelets iji mee ka platelets rụọ ọrụ ma chịkọta ha, oriri nke thrombotic microangiopathy, oriri nke nnukwu thrombosis, mmụba na njigide na spleen, na mmeghachi omume ọjọọ metụtara ọgwụ anticoagulant nke heparin na-anọchite anya. N'ihi na thrombocytopenia nwere ike ịbawanye ohere nke ọbara ọgbụgba, ndị dọkịta nwere nchegbu ụfọdụ gbasara iji ọgwụgwọ antithrombotic na ndị ọrịa APS nwere thrombocytopenia, ọbụnakwa kwenyere n'ụzọ na-ezighị ezi na APS thrombocytopenia nwere ike ibelata ihe egwu nke nlọghachi nke ihe omume thrombotic na ndị ọrịa. N'ezie, n'ụzọ megidere nke ahụ, ọmụmụ ihe egosila na ihe egwu nke nlọghachi nke ihe omume thrombotic na ndị ọrịa APS nwere thrombocytopenia na-abawanye nke ukwuu, yabụ a ga-agwọ ya nke ọma.
2.CAPS bụ ọrịa na-adịghị ahụkebe, nke na-eyi ndụ egwu nke a na-eji ọtụtụ (≥3) vaskụla embolisms eme ihe n'ime obere ọnụọgụgụ ndị ọrịa APS n'ime obere oge (≤ ụbọchị 7), na-enwekarị oke titers, na-emetụta obere arịa ọbara, na nkwenye histopathological nke thrombosis na obere arịa ọbara. APL positivity na-adịgide n'ime izu 12, na-akpata ọtụtụ akụkụ ahụ na ihe ize ndụ nke ọnwụ, nke a maara dị ka catastrophic antiphospholipid syndrome. Ọ na-eme ihe dị ka 1.0%, mana ọnụọgụ ọnwụ dị elu ruo 50% ~ 70%, ọtụtụ mgbe n'ihi ọrịa strok, encephalopathy, ọbara ọgbụgba, ọrịa, wdg. Ihe kpatara ya bụ ịmepụta oké ifufe thrombotic na oké ifufe mkpali n'ime obere oge.
aPLs bụ okwu izugbe maka otu ndị na-alụso ọrịa ọgụ autoantibodies nwere phospholipids na/ma ọ bụ protein ndị na-ejikọta phospholipid dị ka antigens lekwasịrị anya. A na-ahụkarị aPLs na ndị ọrịa nwere ọrịa autoantibodies dịka APS, SLE, na Sjögren's syndrome. Ha bụ ihe nrịbama ụlọ nyocha kachasị mara nke APS na ihe ndị bụ isi na-egosi ihe egwu nke ihe omume thrombotic na afọ ime na ndị ọrịa APS. N'ime ha, lupus anticoagulant (LA), antibodies anticardiolipin (aCL), na antibodies anti-β-glycoprotein I (αβGPⅠ), dị ka ihe ngosi ụlọ nyocha na ọkọlọtọ nhazi APS, ejirila ya nke ọma na omume ahụike ma ghọọ otu n'ime ule autoantibody kachasị na ụlọ nyocha ahụike.
Ma e jiri ya tụnyere ọgwụ mgbochi aCL na anti-βGPⅠ, LA nwere njikọ siri ike na thrombosis na afọ ime. LA nwere nnukwu ihe ize ndụ nke thrombosis karịa acL. Ọ na-ejikọkwa ya na ime ime n'oge afọ ime karịrị izu iri. Na nkenke, LA na-adịgide adịgide bụ otu ihe na-egosi ihe egwu thrombosis na ọrịa afọ ime kachasị dị irè.
LA bụ ule ọrụ nke na-ekpebi ma ahụ nwere LA dabere na eziokwu ahụ bụ na LA nwere ike ịgbatị oge coagulation nke ụzọ dị iche iche dabere na phospholipid na vitro. Usoro nchọpụta nke LA gụnyere:
1. Nnwale nyocha: gụnyere oge nsị viper a gwakọtara agwakọta (dRVVT), oge thromboplastin nke a na-arụ ọrụ (APTT), usoro oge coagulation silica, oge coagulation nke agwọ na oge enzyme nke akwara agwọ. Ugbu a, ntuziaka nchọpụta aPLs mba ụwa dịka International Society on Thrombosis and Haemostasis (ISTH) na Clinical Laboratory Standards Institute (CLSI) na-akwado ka a chọpụta LA site na ụzọ coagulation abụọ dị iche iche. N'ime ha, dRVVT na APTT bụ ụzọ nchọpụta a na-ejikarị eme ihe n'ụwa niile. A na-ejikarị dRVVT eme ihe dị ka ụzọ mbụ a họọrọ, a na-ejikwa APTT dị nro karị (phospholipids dị ala ma ọ bụ silica dị ka ihe na-eme ka ihe rụọ ọrụ) dị ka usoro nke abụọ.
2. Nnwale ngwakọta: A na-agwakọta plasma onye ọrịa na plasma dị mma (1:1) iji gosi na oge coagulation ogologo oge abụghị n'ihi enweghị ihe coagulation.
3. Nnwale nkwenye: A na-agbanwe ntinye ma ọ bụ nhazi nke phospholipids iji gosi na LA dị.
Ọ dị mkpa ịmara na a ga-anakọta ihe nlele kacha mma maka LA site n'aka ndị ọrịa na-enwetabeghị ọgwụgwọ anticoagulant, n'ihi na ndị ọrịa ejiri warfarin, heparin, na ọgwụ anticoagulant ọhụrụ a na-aṅụ (dịka rivaroxaban) gwọọ nwere ike inwe nsonaazụ ule LA nke na-adịghị mma; ya mere, a ga-eji nlezianya kọwaa nsonaazụ ule LA nke ndị ọrịa na-enweta ọgwụgwọ anticoagulant. Na mgbakwunye, a ga-akọwakwa nnwale LA nke ọma n'ọnọdụ ahụike dị oke njọ, n'ihi na mmụba dị elu na ọkwa protein C-reactive nwekwara ike igbochi nsonaazụ ule ahụ.
APS bụ ọrịa autoimmune nke nwere ihe omume thrombosis vascular ugboro ugboro, ime ime na-aga n'ihu ugboro ugboro, thrombocytopenia, wdg. dị ka ihe ngosipụta ahụike bụ isi, yana ọkwa dị elu na nke ọkara na nke dị elu nke aPLs.
APS bụ otu n'ime ihe ole na ole a pụrụ ịgwọta nke na-akpata afọ ime n'ọrịa. Nlekọta kwesịrị ekwesị nke APS nwere ike ime ka nsonaazụ afọ ime ka mma nke ọma.
N'ọrụ ahụike, APS kwesịkwara ịgụnye ndị ọrịa nwere ihe mgbaàmà ahụike metụtara aPLs dịka livedo reticularis, thrombocytopenia, na ọrịa valvụ obi, yana ndị ruru eru na nhazi nkewa ahụike ma nwee obere titers nke aPLs na-adịgide adịgide. Ndị ọrịa dị otú ahụ nwekwara ihe ize ndụ nke ihe omume thrombotic na afọ ime na-adịghị mma.
Ebumnuche ọgwụgwọ nke APS gụnyere igbochi thrombosis na izere ọdịda afọ ime.
Ntụaka
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, wdg. Ntuziaka maka nchọpụta na ọgwụgwọ maka ọrịa antiphospholipid [J]. Akwụkwọ akụkọ China nke Ọgwụ Ime
[2] Bu Jin, Liu Yuhong. Ọganihu n'ịchọpụta na ịgwọ ọrịa antiphospholipid syndrome[J]. Akwụkwọ akụkọ nke Ọgwụ Ahụike Ime
[3] Ntuziaka BSH maka nyocha na njikwa nke ọrịa antiphospholipid.
[4] Kọmitii Thrombosis na Hemostasis nke Òtù Ụlọ Ọgwụ Nnyocha nke China. Nkwekọrịta na nhazi nke nchọpụta na akụkọ gbasara ọgwụ mgbochi lupus [J].
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