Kev kuaj mob lupus anticoagulant (LA) yog ib feem tseem ceeb ntawm kev kuaj mob hauv chaw kuaj mob rau cov tshuaj antiphospholipid thiab tau pom zoo kom siv rau ntau yam xwm txheej hauv kev kho mob, xws li kev kuaj mob hauv chaw kuaj mob ntawm antiphospholipid syndrome (APS) thiab systemic lupus erythematosus (SLE), kev ntsuam xyuas kev pheej hmoo ntawm venous thromboembolism (VTE), thiab kev piav qhia txog lub sijhawm ua haujlwm ntev ntawm cov thromboplastin (APTT) uas tsis tau piav qhia. Tsab xov xwm no yuav pab koj paub txog antiphospholipid syndrome (APS) yog dab tsi.
Kab mob Antiphospholipid syndrome (APS) yog ib yam kab mob autoimmune uas muaj cov teeb meem vascular thrombotic rov tshwm sim, rov tshwm sim spontaneous abortion, thrombocytopenia, thiab lwm yam ua cov tsos mob tseem ceeb, nrog rau cov tshuaj antiphospholipid antibody spectrum (aPLs) uas nyob ntev thiab siab titer zoo. Feem ntau nws muab faib ua primary APS thiab secondary APS, qhov tom kawg feem ntau yog qhov thib ob rau cov kab mob connective tissue xws li systemic lupus erythematosus (SLE) thiab Sjögren's syndrome. Cov tsos mob ntawm APS yog qhov nyuaj thiab ntau yam, thiab txhua lub cev ntawm lub cev tuaj yeem raug cuam tshuam, nrog rau qhov tshwm sim tseem ceeb tshaj plaws yog vascular thrombosis. Lub pathogenesis ntawm APS yog tias circulating aPL khi rau cell surface phospholipids thiab phospholipid-binding proteins, activating endothelial cells, PLTs thiab wBc, ua rau vascular thrombotic events thiab obstetric complications, thiab txhawb nqa qhov tshwm sim ntawm lwm yam autoimmune thiab inflammatory complications. Txawm hais tias aPL yog pathogenic, thrombosis tsuas yog tshwm sim qee zaum, qhia tias lub sijhawm luv luv "kev tawm tsam thib ob" xws li kev kis kab mob, o, phais, cev xeeb tub thiab lwm yam ua rau muaj kev mob thrombosis yog qhov tseem ceeb hauv cov txheej txheem ntawm thrombosis.
Qhov tseeb, APS tsis yog qhov tsis tshua muaj. Cov kev tshawb fawb tau qhia tias 25% ntawm cov neeg mob uas muaj mob stroke tsis paub meej hnub nyoog qis dua 45 xyoos yog aPLs zoo, 14% ntawm cov neeg mob uas muaj cov teeb meem venous thrombosis rov tshwm sim yog aPLs zoo, thiab 15% txog 20% ntawm cov poj niam mob uas poob cev xeeb tub rov tshwm sim yog aPLs zoo. Vim tsis nkag siab txog hom kab mob no los ntawm cov kws kho mob, lub sijhawm kuaj mob qeeb nruab nrab ntawm APS yog li 2.9 xyoo. APS feem ntau tshwm sim ntau dua rau cov poj niam, nrog rau poj niam: txiv neej piv ntawm 9: 1, thiab tshwm sim ntau dua rau cov neeg hluas thiab cov neeg laus nruab nrab, tab sis 12.7% ntawm cov neeg mob muaj hnub nyoog ntau dua 50 xyoo.
1. Cov teeb meem thrombotic
Cov tsos mob ntawm cov hlab ntsha thrombosis hauv APS nyob ntawm hom, qhov chaw thiab qhov loj ntawm cov hlab ntsha uas cuam tshuam, thiab tuaj yeem pom tau tias yog ib lossis ntau cov hlab ntsha koom nrog. Venous thromboembolism (VTE) feem ntau tshwm sim hauv APS, feem ntau yog nyob rau hauv cov hlab ntsha tob ntawm qhov qis qis. Nws kuj tseem tuaj yeem cuam tshuam rau intracranial venous sinuses, retina, subclavian, siab, raum, thiab superior thiab inferior vena cava. APS arterial thrombosis (AT) feem ntau tshwm sim hauv intracranial artery, thiab kuj tuaj yeem cuam tshuam rau renal artery, coronary artery, mesenteric artery, thiab lwm yam. Tsis tas li ntawd, cov neeg mob APS kuj tseem yuav muaj microvascular thrombosis hauv daim tawv nqaij, qhov muag, lub plawv, lub ntsws, raum thiab lwm yam kabmob. Meta-analysis pom tias lupus anticoagulant (LA) positivity muaj kev pheej hmoo ntawm thromboembolism ntau dua li antiphospholipid antibodies (acL); cov kev tshawb fawb hauv tsev kho mob tau qhia tias cov neeg mob APS uas muaj aPL zoo [piv txwv li, LA, aCL, glycoprotein I antibodies (αβGPI) positivity] qhia txog kev pheej hmoo siab ntawm thrombosis, suav nrog tus nqi thrombosis ntawm 44.2% hauv 10 xyoo.
2. Kev xeeb tub uas muaj kab mob
Qhov pathophysiology ntawm obstetric manifestations ntawm APS yog ib yam nyuaj heev thiab tej zaum yuav txawv raws li theem ntawm cev xeeb tub, ua rau muaj qhov sib txawv ntawm cov yam ntxwv kho mob pom. Kev o, kev ua kom tiav, thiab placental thrombosis yog suav tias yog cov yam ntxwv pathogenic ntawm obstetric APS. Pathological cev xeeb tub ua rau los ntawm APS yog ib qho ntawm ob peb yam ua rau uas tuaj yeem tiv thaiv thiab kho, thiab kev tswj hwm kom zoo tuaj yeem txhim kho cov txiaj ntsig cev xeeb tub zoo. Ib qho meta-analysis luam tawm xyoo 2009 pom tias qhov muaj LA thiab aCL muaj feem cuam tshuam nrog kev tuag ntawm tus menyuam hauv plab thaum ntau dua 10 lub lis piam ntawm cev xeeb tub; kev tshuaj xyuas tsis ntev los no thiab meta-analysis kuj pom tias LA positivity muaj feem cuam tshuam nrog kev tuag ntawm tus menyuam hauv plab. Hauv cov neeg mob uas paub tias muaj APS, qhov kev pheej hmoo ntawm kev tuag ntawm tus menyuam hauv plab tseem siab txog li 10% txog 12% txawm tias muaj kev kho mob heparin thiab aspirin tsawg. Rau cov neeg mob APS uas muaj cov tsos mob hnyav ntawm preeclampsia lossis placental insufficiency, qhov muaj LA thiab aCL muaj feem cuam tshuam nrog preeclampsia; Kev rho menyuam rov qab los thaum ntxov (<10 lub lis piam ntawm kev xeeb tub) yog ib qho teeb meem ntawm kev yug menyuam uas feem ntau xav txog qhov ua tau ntawm APS.
1. Cov ntshav qis qis
Kab mob ntshav qis yog ib qho ntawm cov tsos mob uas cov neeg mob APS feem ntau pom, nrog rau qhov tshwm sim ntawm 20% ~ 53%. Feem ntau, SLE theem nrab APS feem ntau yuav muaj kab mob ntshav qis dua li APS thawj zaug. Qib ntawm kab mob ntshav qis hauv cov neeg mob APS feem ntau yog me me lossis nruab nrab. Tej zaum yuav muaj cov aPLs khi ncaj qha rau cov platelets kom ua haujlwm thiab sib sau ua ke, noj cov kab mob ntshav qis me me, noj ntau cov kab mob ntshav qis, khaws cia hauv lub qhov ntswg ntau ntxiv, thiab cov kev tsis zoo uas cuam tshuam nrog cov tshuaj anticoagulant uas sawv cev los ntawm heparin. Vim tias kab mob ntshav qis yuav ua rau muaj kev pheej hmoo ntawm kev los ntshav, cov kws kho mob muaj qee qhov kev txhawj xeeb txog kev siv tshuaj antithrombotic rau cov neeg mob APS uas muaj kab mob ntshav qis, thiab txawm tias ntseeg yuam kev tias APS thrombocytopenia tuaj yeem txo qhov kev pheej hmoo ntawm kev rov tshwm sim ntawm cov xwm txheej ntshav qis hauv cov neeg mob. Qhov tseeb, ntawm qhov tsis sib xws, kev tshawb fawb tau qhia tias qhov kev pheej hmoo ntawm kev rov tshwm sim ntawm cov xwm txheej ntshav qis hauv cov neeg mob APS uas muaj kab mob ntshav qis yog nce ntxiv, yog li nws yuav tsum tau kho kom nquag dua.
2. CAPS yog ib yam kab mob tsis tshua muaj, uas ua rau tuag taus uas muaj ntau (≥3) vascular embolisms hauv cov neeg mob APS me me hauv lub sijhawm luv luv (≤7 hnub), feem ntau muaj cov titers siab, cuam tshuam rau cov hlab ntsha me me, thiab histopathological lees paub tias muaj thrombosis hauv cov hlab ntsha me me. APL positivity tseem nyob hauv 12 lub lis piam, ua rau ntau lub cev tsis ua haujlwm thiab muaj kev pheej hmoo ntawm kev tuag, hu ua catastrophic antiphospholipid syndrome. Nws qhov tshwm sim yog li 1.0%, tab sis tus nqi tuag yog siab txog 50% ~ 70%, feem ntau yog vim mob stroke, encephalopathy, hemorrhage, kab mob, thiab lwm yam. Nws qhov ua tau pathogenesis yog kev tsim ntawm thrombotic storm thiab inflammatory storm hauv lub sijhawm luv luv.
aPLs yog ib lo lus dav dav rau ib pawg ntawm cov autoantibodies nrog phospholipids thiab / lossis phospholipid-binding proteins ua lub hom phiaj antigens. aPLs feem ntau pom muaj nyob rau hauv cov neeg mob uas muaj cov kab mob autoimmune xws li APS, SLE, thiab Sjögren's syndrome. Lawv yog cov cim qhia tseem ceeb tshaj plaws ntawm APS thiab cov lus qhia tseem ceeb ntawm kev pheej hmoo ntawm thrombotic xwm txheej thiab pathological cev xeeb tub hauv cov neeg mob APS. Ntawm lawv, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), thiab anti-β-glycoprotein I (αβGPⅠ) antibodies, ua cov cim qhia hauv chav kuaj mob hauv APS kev faib tawm tus qauv, tau siv dav hauv kev kho mob thiab tau dhau los ua ib qho ntawm cov kev kuaj mob autoantibody feem ntau hauv chav kuaj mob.
Piv nrog rau aCL thiab anti-βGPⅠ antibodies, LA muaj kev sib raug zoo dua nrog thrombosis thiab cev xeeb tub pathological. LA muaj kev pheej hmoo ntawm thrombosis ntau dua li acL. Thiab nws muaj feem cuam tshuam nrog kev rho menyuam thaum cev xeeb tub ntau dua 10 lub lis piam. Hauv ntej, LA zoo tas li yog qhov kwv yees zoo tshaj plaws ntawm kev pheej hmoo thrombotic thiab kev mob cev xeeb tub.
LA yog ib qho kev kuaj mob uas txiav txim siab seb lub cev puas muaj LA raws li qhov tseeb tias LA tuaj yeem ua rau lub sijhawm coagulation ntawm ntau txoj hauv kev phospholipid-dependent hauv vitro ntev dua. Cov txheej txheem kuaj pom ntawm LA suav nrog:
1. Kev kuaj xyuas: suav nrog lub sijhawm diluted viper venom time (dRVVT), lub sijhawm ua haujlwm ib nrab thromboplastin (APTT), silica coagulation time method, giant snake coagulation time thiab snake vein enzyme time. Tam sim no, cov lus qhia thoob ntiaj teb aPLs nrhiav pom xws li International Society on Thrombosis and Haemostasis (ISTH) thiab Clinical Laboratory Standards Institute (CLSI) pom zoo kom LA raug kuaj pom los ntawm ob txoj kev coagulation sib txawv. Ntawm lawv, dRVVT thiab APTT yog cov txheej txheem nrhiav pom siv ntau tshaj plaws thoob ntiaj teb. Feem ntau dRVVT yog siv ua thawj txoj kev xaiv, thiab APTT rhiab heev dua (phospholipids qis lossis silica ua tus activator) yog siv ua txoj kev thib ob.
2. Kev sim sib xyaw: Cov ntshav ntawm tus neeg mob raug sib xyaw nrog cov ntshav noj qab haus huv (1:1) kom paub tseeb tias lub sijhawm coagulation ntev tsis yog vim tsis muaj cov yam ntxwv coagulation.
3. Kev kuaj xyuas kom paub tseeb: Qhov concentration lossis cov khoom sib xyaw ntawm phospholipids raug hloov pauv kom paub tseeb tias muaj LA.
Nws yog ib qho tsim nyog sau tseg tias cov qauv zoo tshaj plaws rau LA yuav tsum tau sau los ntawm cov neeg mob uas tsis tau txais kev kho mob anticoagulant, vim tias cov neeg mob uas tau kho nrog warfarin, heparin, thiab cov tshuaj anticoagulants tshiab hauv qhov ncauj (xws li rivaroxaban) yuav muaj cov txiaj ntsig ntawm kev kuaj LA cuav; yog li ntawd, cov txiaj ntsig ntawm kev kuaj LA ntawm cov neeg mob uas tau txais kev kho mob anticoagulant yuav tsum tau txhais nrog kev ceev faj. Tsis tas li ntawd, kev kuaj LA kuj yuav tsum tau txhais nrog kev ceev faj hauv qhov chaw kho mob ceev ceev, vim tias qhov nce siab ntawm C-reactive protein kuj tuaj yeem cuam tshuam rau cov txiaj ntsig ntawm kev kuaj.
APS yog ib yam kab mob autoimmune uas muaj cov teeb meem thrombotic vascular rov tshwm sim, rov tshwm sim spontaneous rho menyuam, thrombocytopenia, thiab lwm yam ua cov tsos mob tseem ceeb, nrog rau cov theem nruab nrab thiab siab ntawm aPLs.
APS yog ib qho ntawm ob peb yam uas kho tau uas ua rau cev xeeb tub tsis zoo. Kev tswj hwm APS kom zoo yuav ua rau cev xeeb tub zoo dua.
Hauv kev ua haujlwm kho mob, APS yuav tsum suav nrog cov neeg mob uas muaj cov tsos mob cuam tshuam nrog aPLs xws li livedo reticularis, thrombocytopenia, thiab kab mob plawv, nrog rau cov neeg uas ua tau raws li cov qauv kev faib tawm hauv kev kho mob thiab muaj cov titers qis ntawm aPLs. Cov neeg mob no kuj muaj kev pheej hmoo ntawm cov xwm txheej thrombotic thiab cev xeeb tub pathological.
Lub hom phiaj kho mob ntawm APS feem ntau suav nrog kev tiv thaiv kab mob thrombosis thiab zam kev ua tsis tiav ntawm cev xeeb tub.
Cov ntaub ntawv siv los ua piv txwv
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, thiab lwm yam. Cov lus qhia rau kev kuaj mob thiab kev kho mob rau antiphospholipid syndrome [J]. Phau Ntawv Xov Xwm Suav Txog Kev Kho Mob Sab Hauv
[2] Bu Jin, Liu Yuhong. Kev nce qib hauv kev kuaj mob thiab kev kho mob ntawm antiphospholipid syndrome [J]. Phau ntawv Journal of Clinical Internal Medicine
[3] BSH GUIDELINE Cov lus qhia txog kev tshawb nrhiav thiab kev tswj hwm ntawm antiphospholipid syndrome.
[4] Pawg Neeg Saib Xyuas Kev Mob Thrombosis thiab Hemostasis ntawm Lub Koom Haum Suav ntawm Cov Tsev Kho Mob Tshawb Fawb. Kev pom zoo ntawm kev ua kom raug ntawm kev kuaj pom thiab tshaj tawm lupus anticoagulant [J].
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