Kuongororwa kwe lupus anticoagulant (LA) chikamu chakakosha chekuongororwa kwerabhoritari che antiphospholipid antibodies uye kwakakurudzirwa kushandiswa mumamiriro akasiyana-siyana ekiriniki, akadai sekuongororwa kwerabhoritari kwe antiphospholipid syndrome (APS) uye systemic lupus erythematosus (SLE), kuongororwa kwengozi ye venous thromboembolism (VTE), uye tsananguro yenguva isina kutsanangurwa yakaitwa partial thromboplastin (APTT). Chinyorwa chino chichakubatsira kuti uzive kuti antiphospholipid syndrome (APS) chii.
Antiphospholipid syndrome (APS) chirwere che autoimmune chine zviitiko zve vascular thrombotic zvinodzokororwa, kubvisa pamuviri kusingadzokororwi, thrombocytopenia, nezvimwewo sezviratidzo zvikuru zvekiriniki, pamwe chete ne persistent medium and high titer positive antiphospholipid antibody spectrum (aPLs). Inowanzo kamurwa kuva primary APS ne secondary APS, iyo yekupedzisira inonyanya kukonzerwa nezvirwere zve connective tissue zvakaita se systemic lupus erythematosus (SLE) ne Sjögren's syndrome. Zviratidzo zvekiriniki zveAPS zvakaoma uye zvakasiyana-siyana, uye masisitimu ese emuviri anogona kukanganiswa, uye chinonyanya kuoneka chiri vascular thrombosis. Pathogenesis yeAPS ndeyekuti aPL inotenderera inobatanidza kune phospholipids yepamusoro pemasero uye mapuroteni anosunga phospholipid, ichimutsa masero e endothelial, PLTs ne wBc, zvichikonzera zviitiko zve vascular thrombotic uye matambudziko ekuzvitakura, uye zvichikurudzira kuitika kwemamwe matambudziko e autoimmune nekuzvimba. Kunyange hazvo aPL ichikonzera hutachiona, thrombosis inoitika nguva nenguva, zvichiratidza kuti "kurwiswa kwechipiri" kwenguva pfupi senge hutachiona, kuzvimba, kuvhiyiwa, pamuviri nezvimwe zvinhu zvinokonzera thrombosis zvakakosha mukuitika kwethrombosis.
Kutaura zvazviri, APS haisi chinhu chinowanzoitika. Zvidzidzo zvakaratidza kuti 25% yevarwere vane sitiroko isina tsananguro vari pasi pemakore 45 vane aPL positive, 14% yevarwere vane venous thrombosis inowanzoitika vane aPL positive, uye 15% kusvika 20% yevarwere vechikadzi vane pamuviri inowanzoitika vane aPL positive. Nekuda kwekusanzwisisa rudzi urwu rwechirwere nemakiriniki, avhareji yenguva yekunonoka kuongororwa kweAPS inguva yemakore angangoita 2.9. APS inowanzoonekwa zvakanyanya muvakadzi, ine chiyero chevakadzi: varume che9:1, uye inowanikwa zvakanyanya muvanhu vechidiki nevepakati, asi 12.7% yevarwere vane makore anopfuura 50 ekuberekwa.
1. Zviitiko zveThrombotic
Zviratidzo zvechirwere che vascular thrombosis muAPS zvinoenderana nerudzi, nzvimbo uye saizi yetsinga dzeropa dzakakanganiswa, uye zvinogona kuratidzwa sedzimwe kana dzakawanda dzinobatanidzwa. Venous thromboembolism (VTE) inowanikwa zvakanyanya muAPS, kazhinji mutsinga dzakadzika dzemigumo yepasi. Inogonawo kukanganisa intracranial venous sinuses, retina, subclavian, chiropa, itsvo, uye superior and inferior vena cava. APS arterial thrombosis (AT) inowanikwa zvakanyanya mutsinga dzemukati memusoro, uye inogonawo kukanganisa tsinga dzeitsvo, coronary arteries, mesenteric arteries, nezvimwewo. Pamusoro pezvo, varwere veAPS vanogonawo kuva ne microvascular thrombosis muganda, maziso, moyo, mapapu, itsvo nedzimwe nhengo. Meta-analysis yakawana kuti lupus anticoagulant (LA) positivity ine njodzi yakakura ye thromboembolism kupfuura antiphospholipid antibodies (acL); Zvidzidzo zvekiriniki zvakaratidza kuti varwere veAPS vane aPL yakanaka [kureva, LA, aCL, glycoprotein I antibodies (αβGPI) positivity] vanoratidza njodzi yakakura yethrombosis, kusanganisira thrombosis rate ye44.2% mukati memakore gumi.
2. Pamuviri wezvirwere
Maitiro ekudzivirira chirwere cheAPS kubva mukusununguka akaomarara zvakaenzana uye anogona kusiyana zvichienderana nedanho repamuviri, zvichikonzera kusiyana kwezviratidzo zvekiriniki zvinoonekwa. Kuzvimba, kushanda kwemuviri, uye thrombosis yeplacenta zvese zvinoonekwa sezvikonzero zvinokonzera chirwere cheAPS chekuberekwa. Kubata pamuviri kunokonzerwa neAPS ndechimwe chezvikonzero zvishoma zvinogona kudzivirirwa nekurapwa, uye kutarisirwa kwakakodzera kunogona kuvandudza mhedzisiro yekuzvitakura zvinobudirira. Kuongororwa kwakaitwa muna 2009 kwakawana kuti kuvapo kweLA neaCL kwakabatana zvakanyanya nerufu rwemwana asati aberekwa pamavhiki anopfuura gumi ekuzvitakura; ongororo yakarongeka uye kuongorora kwakaitwa munguva pfupi yapfuura kwakawanawo kuti LA positivity yaive yakabatana zvakanyanya nerufu rwemwana asati aberekwa. Muvarwere vanozivikanwa kuti vane APS, njodzi yekufa kwemwana asati aberekwa ichiri yakakura se10% kusvika 12% kunyangwe nekurapwa kwakajairika kweheparin neaspirin shoma. Kune varwere veAPS vane zviratidzo zvakakomba zve preeclampsia kana placenta insufficiency, kuvapo kweLA neaCL kwakabatana zvakanyanya ne preeclampsia; kupora kwepamuviri kunowanzoitika (pasi pemavhiki gumi ekuzvitakura) idambudziko rekusununguka rinowanzo funga nezve mukana weAPS.
1. Kuora kweThrombocytopenia
Kurwara kweThrombocytopenia ndeimwe yezviratidzo zvinowanzoonekwa nevarwere veAPS, ine huwandu hwe20% ~ 53%. Kazhinji, SLE secondary APS inonyanya kukonzera thrombocytopenia kupfuura primary APS. Huwandu hwethrombocytopenia muvarwere veAPS hunowanzova hushoma kana hune mwero. Pathogenesis inogona kusanganisira aPLs zvakananga kunamatira kumaplatelet kuti iite kuti maplatelet ashande uye aunganidzwe, kushandisa thrombotic microangiopathy, kushandisa thrombosis yakawanda, kuramba kwakawanda muspleen, uye maitiro asina kunaka ane chekuita nemishonga inorwisa ropa inomiririrwa neheparin. Nekuti thrombocytopenia inogona kuwedzera njodzi yekubuda ropa, vanachiremba vane zvimwe zvavanonetseka nazvo nezvekushandiswa kwemishonga inorwisa thrombotic muvarwere veAPS vane thrombocytopenia, uye vanototenda zvisirizvo kuti APS thrombocytopenia inogona kuderedza njodzi yekudzoka kwezviitiko zvethrombotic muvarwere. Kutaura zvazviri, zvakasiyana, zvidzidzo zvakaratidza kuti njodzi yekudzoka kwezviitiko zvethrombotic muvarwere veAPS vane thrombocytopenia yakawedzera zvakanyanya, saka inofanira kurapwa zvakanyanya.
2. CAPS chirwere chisingawanzoitiki, chinotyisidzira hupenyu chinoratidzwa nekuva netsinga dzakawanda (≥3) muvarwere vashoma veAPS mukati menguva pfupi (≤mazuva manomwe), kazhinji kacho nematinji akakwira, zvichikanganisa mitsipa midiki yeropa, uye kusimbiswa kwe thrombosis mumitsipa midiki yeropa. APL positivity inoramba iripo mukati memavhiki gumi nemaviri, zvichikonzera kutadza kushanda kwenhengo dzakawanda uye njodzi yekufa, inozivikanwa se catastrophical antiphospholipid syndrome. Kuwanda kwayo kunenge 1.0%, asi huwandu hwerufu hwakakwira kusvika 50% ~ 70%, kazhinji nekuda kwestroke, encephalopathy, kubuda ropa, hutachiona, nezvimwewo. Pathogen yayo inogona kuitika kuumbwa kwe thrombotic storm uye inflammatory storm munguva pfupi.
aPLs izwi rekuti anowanzo shandiswa pakutsvaga ma antibodies ane ma phospholipids uye/kana ma phospholipid-binding proteins se target antigens. Ma aPLs anonyanya kuwanikwa muvarwere vane zvirwere zve autoimmune zvakaita seAPS, SLE, uye Sjögren's syndrome. Ndiwo ma APS anonyanya kuzivikanwa mu laboratory uye ndiwo anonyanya kuratidza njodzi ye thrombosis uye pathological pregnancy muvarwere vane APS. Pakati pawo, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), uye anti-β-glycoprotein I (αβGPⅠ) antibodies, sezviratidzo zve laboratory mu APS classification standard, ave achishandiswa zvakanyanya mu clinical practice uye ave imwe ye autoantibody tests dzakajairika mu clinical laboratory.
Kana tichienzanisa ne aCL ne anti-βGPⅠ antibodies, LA ine hukama hwakasimba ne thrombosis uye pathological pregnancy. LA ine njodzi yakakura ye thrombosis kupfuura acL. Uye ine hukama hwakasimba nekubvisa pamuviri panguva yekuzvitakura > mavhiki gumi. Muchidimbu, LA inogara iine positive ndiyo inonyanya kushanda pakuratidza njodzi ye thrombosis uye chirwere chepamuviri.
LA bvunzo inoshanda inoratidza kana muviri une LA zvichibva pakuti LA inogona kuwedzera nguva yekugwamba kwenzira dzakasiyana dzinoenderana nephospholipid in vitro. Nzira dzekuongorora LA dzinosanganisira:
1. Kuongororwa kwebvunzo: kusanganisira nguva yehuturu hwenyoka yakaderedzwa (dRVVT), nguva yeropa yakasanganiswa (APTT), nzira yenguva yeropa yakasanganiswa (silica coagulation time), nguva yeropa yakasanganiswa (giant snake coagulation time) uye nguva ye snake vein enzyme. Parizvino, gwara rekuongorora repasi rose re aPLs rakaita seInternational Society on Thrombosis and Haemostasis (ISTH) neClinical Laboratory Standards Institute (CLSI) rinokurudzira kuti LA ionekwe nenzira mbiri dzakasiyana dzeropa. Pakati padzo, dRVVT neAPTT ndidzo nzira dzinonyanya kushandiswa pasi rose. Kazhinji dRVVT inoshandiswa senzira yekutanga yekusarudza, uye APTT inonyanya kubatwa (low phospholipids kana silica se activator) inoshandiswa senzira yechipiri.
2. Kuongororwa kwekusanganisa: Plasma yemurwere inosanganiswa neplasma ine hutano (1:1) kusimbisa kuti nguva yakareba yekugwinha kweropa haisi yekushaikwa kwezvinhu zvinogwinha ropa.
3. Kuongororwa kwekusimbisa: Kuwanda kana kuumbwa kwe phospholipids kunochinjwa kusimbisa kuvapo kwe LA.
Zvakakosha kuziva kuti sampuro yakakodzera yeLA inofanira kutorwa kubva kuvarwere vasina kurapwa nemishonga inorwisa ropa, nekuti varwere vanorapwa newarfarin, heparin, nemishonga mitsva inorwisa ropa (yakadai serivaroxaban) vanogona kunge vaine mhedzisiro yeLA yekuyedzwa kwenhema; saka, mhedzisiro yeLA yekuyedzwa kwevarwere vanorapwa nemishonga inorwisa ropa inofanira kududzirwa nekungwarira. Pamusoro pezvo, kuongororwa kweLA kunofanirawo kududzirwa nekungwarira munzvimbo yekiriniki, nekuti kukwira kwakanyanya kwemazinga eC-reactive protein kunogona kukanganisawo mhedzisiro yebvunzo.
APS chirwere chinokonzerwa ne autoimmune chinokonzerwa nezviitiko zve vascular thrombosis zvinodzokororwa, kubvisa pamuviri kunoitika kamwe kamwe, thrombocytopenia, nezvimwewo sezviratidzo zvikuru zvekiriniki, pamwe chete ne aPLs dzinogara dziripo uye dzakakwira.
APS ndeimwe yezvikonzero zvishoma zvinogona kurapwa zvinokonzera hutachiona hwepamuviri. Kutarisirwa zvakanaka kweAPS kunogona kuvandudza mhedzisiro yepamuviri zvinobudirira.
Mukurapa, APS inofanirawo kusanganisira varwere vane zviratidzo zvekurapa zvine chekuita neaPLs zvakaita se livedo reticularis, thrombocytopenia, uye chirwere chevharuvhu yemoyo, pamwe chete nevaya vanozadzisa zvinodiwa zvekurapa uye vane matita mashoma e aPLs. Varwere vakadaro vanewo njodzi yezviitiko zve thrombosis uye pamuviri une chirwere.
Zvinangwa zvekurapa zveAPS zvinosanganisira kudzivirira thrombosis uye kudzivirira kutadza kubata pamuviri.
Mareferensi
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, nevamwe. Kuongororwa uye nhungamiro dzekurapa chirwere che antiphospholipid [J]. Chinese Journal of Internal Medicine
[2] Bu Jin, Liu Yuhong. Kufambira mberi mukuongorora nekurapa chirwere che antiphospholipid [J]. Journal of Clinical Internal Medicine
[3] BSH GWARO REKUTUNGAMIRIRA Nhungamiro dzekuongorora nekutarisira chirwere che antiphospholipid.
[4] Komiti yeThrombosis neHemostasis yeChinese Society of Research Hospitals. Kubvumirana pamusoro pekuenzanisa kwe lupus anticoagulant detection and reporting[J].
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