Teko ea lupus anticoagulant (LA) ke karolo ea bohlokoa ea teko ea laboratori bakeng sa li-antibodies tsa antiphospholipid 'me e khothalelitsoe hore e sebelisoe maemong a fapaneng a bongaka, joalo ka tlhahlobo ea laboratori ea antiphospholipid syndrome (APS) le systemic lupus erythematosus (SLE), tlhahlobo ea kotsi ea venous thromboembolism (VTE), le tlhaloso ea nako e sa hlalosoang ea thromboplastin e activated (APTT). Sengoloa sena se tla u thusa ho tloaelana le hore na antiphospholipid syndrome (APS) ke eng.
Antiphospholipid syndrome (APS) ke lefu la autoimmune le nang le liketsahalo tse iphetang tsa vascular thrombotic, ho ntšoa ha mpa ka tšohanyetso khafetsa, thrombocytopenia, jj. e le lipontšo tse ka sehloohong tsa kliniki, tse tsamaeang le antiphospholipid antibody spectrum e sa feleng ea medium le high titer positive (aPLs). Hangata e arotsoe ka APS ea mantlha le APS ea bobeli, eo ea morao-rao e leng ea bobeli ka lebaka la mafu a lisele tse hokahaneng joalo ka systemic lupus erythematosus (SLE) le Sjögren's syndrome. Lipontšo tsa kliniki tsa APS li rarahane ebile lia fapana, 'me litsamaiso tsohle tsa' mele li ka angoa, 'me ponahalo e hlahelletseng ka ho fetisisa ke vascular thrombosis. Pathogenesis ea APS ke hore aPL e potolohang e tlama ho phospholipids ea bokaholimo ba sele le liprotheine tse tlamang phospholipid, e kenya tšebetsong lisele tsa endothelial, PLTs le wBc, e lebisang liketsahalong tsa vascular thrombotic le mathata a pelehi, le ho khothalletsa ho hlaha ha mathata a mang a autoimmune le a ho ruruha. Leha aPL e baka tshwaetso, thrombosis e etsahala ka nako e itseng feela, e leng se bontshang hore "dikotsi tsa bobedi" tsa nako e kgutshwane tse kang tshwaetso, ho ruruha, opereishene, boimana le mabaka a mang a bakang thrombosis di bohlokwa tshebetsong ya thrombosis.
Ha e le hantle, APS ha se ntho e sa tloaelehang. Lithuto li bontšitse hore 25% ea bakuli ba nang le stroke e sa hlalosoang ba ka tlase ho lilemo tse 45 ba na le aPLs tse ntle, 14% ea bakuli ba nang le liketsahalo tse pheta-phetoang tsa venous thrombosis ba na le aPLs tse ntle, 'me 15% ho isa ho 20% ea bakuli ba basali ba nang le tahlehelo ea boimana bo pheta-phetoang ba na le aPLs tse ntle. Ka lebaka la ho se utloisise mofuta ona oa lefu ke lingaka, nako e tloaelehileng ea ho hlahlojoa ha APS e liehang ke lilemo tse ka bang 2.9. APS hangata e atile haholo ho basali, ka karolelano ea basali: banna ea 9:1, 'me e atile haholo ho batho ba banyenyane le ba lilemo tse mahareng, empa 12.7% ea bakuli ba lilemo tse ka holimo ho 50.
1. Liketsahalo tsa thrombotic
Lipontšo tsa kliniki tsa thrombosis ea methapo ea mali ho APS li itšetlehile ka mofuta, sebaka le boholo ba methapo ea mali e amehileng, 'me li ka bonahala e le methapo ea mali e le 'ngoe kapa e mengata e amehang. Venous thromboembolism (VTE) e atile haholo ho APS, hangata methapong e tebileng ea lipheletsong tse ka tlase. E ka boela ea ama sinuses ea venous intracranial, retina, subclavian, sebete, liphio, le vena cava e phahameng le e tlase. APS arterial thrombosis (AT) e atile haholo methapong ea intracranial, 'me e ka boela ea ama methapo ea liphio, methapo ea coronary, methapo ea mesenteric, jj. Ho phaella moo, bakuli ba APS ba ka 'na ba ba le thrombosis ea microvascular letlalong, mahlong, pelong, matšoafong, liphiong le lithong tse ling. Tlhahlobo ea meta e fumane hore lupus anticoagulant (LA) positivity e na le kotsi e kholo ea thromboembolism ho feta antiphospholipid antibodies (acL); Lithuto tsa bongaka li bontšitse hore bakuli ba APS ba nang le aPL [ke hore, LA, aCL, glycoprotein I antibodies (αβGPI) positivity] ba bontša kotsi e kholo ea thrombosis, ho kenyeletsoa le sekhahla sa thrombosis sa 44.2% nakong ea lilemo tse 10.
2. Boimana ba mafu
Pathophysiology ea lipontšo tsa pelehi tsa APS e rarahane ka ho lekana 'me e ka fapana ho latela mohato oa boimana, e leng se fellang ka ho se tšoane ha litšobotsi tsa kliniki tse bonoang. Ho ruruha, ts'ebetso ea tlatsetso, le thrombosis ea placenta kaofela li nkoa e le mabaka a bakang pathogenic a APS ea pelehi. Boimana ba pathological bo bakoang ke APS ke e 'ngoe ea lisosa tse' maloa tse ka thibeloang le ho phekoloa, 'me tsamaiso e nepahetseng e ka ntlafatsa liphello tsa boimana ka katleho. Tlhahlobo ea meta e phatlalalitsoeng ka 2009 e fumane hore boteng ba LA le aCL bo ne bo amana haholo le lefu la lesea le sa tsoa tsoaloa libeke tse ka holimo ho 10 tsa boimana; tlhahlobo ea morao tjena e hlophisitsoeng le tlhahlobo ea meta le tsona li fumane hore LA positivity e ne e amana haufi-ufi le lefu la lesea le sa tsoa tsoaloa. Ho bakuli ba tsejoang hore ba na le APS, kotsi ea lefu la lesea le sa tsoa tsoaloa e ntse e le holimo ho 10% ho isa ho 12% esita le ka phekolo e tloaelehileng ea heparin le aspirin e nang le tekanyo e tlase. Bakeng sa bakuli ba APS ba nang le matšoao a matla a preeclampsia kapa ho se sebetse hantle ha placenta, boteng ba LA le aCL bo amahanngoa haholo le preeclampsia; ho senyeheloa ke mpa khafetsa pele ho nako (
1.Thrombocytopenia
Thrombocytopenia ke e 'ngoe ea lipontšo tse tloaelehileng tsa bongaka tsa bakuli ba APS, ka palo ea 20% ~ 53%. Hangata, APS ea bobeli ea SLE e na le tšekamelo ea ho tšoaroa ke thrombocytopenia ho feta APS ea mantlha. Tekanyo ea thrombocytopenia ho bakuli ba APS hangata e bobebe kapa e itekanetseng. Pathogen e ka bang teng e kenyelletsa aPLs e tlamang ka kotloloho ho li-platelet ho kenya tšebetsong le ho kopanya li-platelet, ho noa thrombotic microangiopathy, ho noa thrombosis e ngata, ho boloka ho eketsehileng ha spleen, le liketso tse mpe tse amanang le meriana ea anticoagulant e emeloang ke heparin. Hobane thrombocytopenia e ka eketsa kotsi ea ho tsoa mali, lingaka li na le matšoenyeho a mang mabapi le tšebeliso ea phekolo ea antithrombotic ho bakuli ba APS ba nang le thrombocytopenia, 'me li bile li lumela ka phoso hore thrombocytopenia ea APS e ka fokotsa kotsi ea ho boela ha liketsahalo tsa thrombotic ho bakuli. Ha e le hantle, ho fapana le hoo, lithuto li bontšitse hore kotsi ea ho khutla ha liketsahalo tsa thrombotic ho bakuli ba APS ba nang le thrombocytopenia e eketseha haholo, kahoo e lokela ho phekoloa ka mafolofolo haholoanyane.
2. CAPS ke lefu le sa tloaelehang, le behang bophelo kotsing le khetholloang ke ho ruruha ha methapo ea mali ka makhetlo a mangata (≥3) ho bakuli ba fokolang ba APS ka nako e khutšoanyane (≤matsatsi a 7), hangata ka li-titers tse phahameng, tse amang methapo e menyenyane ea mali, le tiiso ea histopathological ea thrombosis methapong e menyenyane ea mali. APL positivity e tsoela pele nakong ea libeke tse 12, e bakang ho hloleha ha litho tse ngata le kotsi ea lefu, e tsejoang e le catastrophic antiphospholipid syndrome. Ho ata ha lona ke hoo e ka bang 1.0%, empa sekhahla sa lefu se holimo ho fihla ho 50% ~ 70%, hangata ka lebaka la stroke, encephalopathy, ho tsoa mali, tšoaetso, jj. Pathogene ea lona e ka bang teng ke ho thehoa ha sefefo sa thrombotic le sefefo sa ho ruruha ka nako e khutšoanyane.
aPLs ke lentsoe le akaretsang bakeng sa sehlopha sa li-autoantibodies tse nang le li-phospholipid le/kapa liprotheine tse tlamang phospholipid e le li-antigen tse reretsoeng. Li-aPLs li fumanoa haholo ho bakuli ba nang le mafu a autoimmune a kang APS, SLE, le Sjögren's syndrome. Ke matšoao a laboratori a ikhethang ka ho fetisisa a APS le li-ponelopele tse ka sehloohong tsa kotsi ea liketsahalo tsa thrombosis le boimana ba pathological ho bakuli ba APS. Har'a tsona, li-antibody tsa lupus anticoagulant (LA), li-antibody tsa anticardiolipin (aCL), le li-antibody tsa anti-β-glycoprotein I (αβGPⅠ), e le matšoao a laboratori maemong a sehlopha sa APS, li 'nile tsa sebelisoa haholo ts'ebetsong ea bongaka' me li fetohile e 'ngoe ea liteko tse tloaelehileng tsa autoantibody lilaboratoring tsa bongaka.
Ha ho bapisoa le li-antibodies tsa aCL le anti-βGPⅠ, LA e na le kamano e matla le thrombosis le boimana ba pathological. LA e na le kotsi e kholo ea thrombosis ho feta acL. 'Me e amana haufi-ufi le ho senyeheloa ke mpa nakong ea boimana > libeke tse 10. Ka bokhutšoanyane, LA e lulang e le teng ke eona e sebetsang ka ho fetisisa e bolelang kotsi ea thrombosis le bokuli ba boimana.
LA ke teko e sebetsang e etsang qeto ea hore na 'mele o na le LA ho latela taba ea hore LA e ka lelefatsa nako ea ho hoama ha litsela tse fapaneng tse itšetlehileng ka phospholipid ka vitro. Mekhoa ea ho lemoha LA e kenyelletsa:
1. Teko ea tlhahlobo: ho kenyeletsoa nako ea chefo ea viper e hlapolotsoeng (dRVVT), nako ea thromboplastin e kentsoeng tšebetsong (APTT), mokhoa oa nako ea ho kopanya silica, nako ea ho kopanya noha e kholo le nako ea enzyme ea mothapo oa noha. Hona joale, litataiso tsa machaba tsa ho lemoha aPL tse kang Mokhatlo oa Machaba oa Thrombosis le Haemostasis (ISTH) le Setsi sa Maemo a Bongaka sa Laboratori (CLSI) li khothaletsa hore LA e fumanoe ka litsela tse peli tse fapaneng tsa ho kopanya. Har'a tsona, dRVVT le APTT ke mekhoa ea ho lemoha e sebelisoang haholo machabeng. Hangata dRVVT e sebelisoa e le mokhoa oa pele oa khetho, 'me APTT e bonolo haholoanyane (phospholipids e tlase kapa silica e le activator) e sebelisoa e le mokhoa oa bobeli.
2. Teko ea ho kopanya: Plasma ea mokuli e kopanngoa le plasma e phetseng hantle (1:1) ho netefatsa hore nako e telele ea ho kopanya mali ha e bakoe ke khaello ea lintho tse kopanyang mali.
3.Teko ea netefatso: Khakanyo kapa motsoako oa phospholipids lia fetoloa ho netefatsa boteng ba LA.
Ho bohlokoa ho hlokomela hore sampole e loketseng LA e lokela ho bokelloa ho bakuli ba sa kang ba fumana phekolo ea anticoagulant, hobane bakuli ba phekoloang ka warfarin, heparin, le li-anticoagulant tse ncha tsa molomo (joalo ka rivaroxaban) ba ka 'na ba ba le liphetho tsa liteko tsa LA tse fosahetseng; ka hona, liphetho tsa liteko tsa LA tsa bakuli ba fumanang phekolo ea anticoagulant li lokela ho hlalosoa ka hloko. Ho phaella moo, liteko tsa LA le tsona li lokela ho hlalosoa ka hloko tikolohong e matla ea kliniki, hobane keketseho e matla maemong a protheine ea C-reactive le eona e ka sitisa liphetho tsa liteko.
APS ke lefu la autoimmune le nang le liketsahalo tse iphetang tsa thrombosis ea methapo ea mali, ho ntšoa ha mpa ka tšohanyetso khafetsa, thrombocytopenia, jj. e le matšoao a mantlha a kliniki, a tsamaeang le maemo a mahareng le a phahameng a aPL.
APS ke e 'ngoe ea lisosa tse 'maloa tse ka phekolehang tsa boimana bo sa foleng. Tsamaiso e nepahetseng ea APS e ka ntlafatsa liphello tsa boimana ka katleho.
Mosebetsing oa bongaka, APS e lokela ho kenyelletsa bakuli ba nang le matšoao a kliniki a amanang le aPLs joalo ka livedo reticularis, thrombocytopenia, le lefu la valve ea pelo, hammoho le ba fihlelang litekanyetso tsa tlhahlobo ea bongaka 'me ba na le maemo a tlase a sa feleng a aPLs. Bakuli ba joalo ba boetse ba na le kotsi ea liketsahalo tsa thrombosis le boimana ba pathological.
Lipakane tsa kalafo tsa APS li kenyelletsa haholo-holo ho thibela thrombosis le ho qoba ho hloleha ha boimana.
Litšupiso
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, jj. Litataiso tsa tlhahlobo le kalafo bakeng sa antiphospholipid syndrome [J]. Chinese Journal of Internal Medicine
[2] Bu Jin, Liu Yuhong. Tsoelo-pele ho fumaneng le ho phekola lefu la antiphospholipid [J]. Journal of Clinical Internal Medicine
[3] BSH TAELO Tataiso mabapi le lipatlisiso le taolo ea antiphospholipid syndrome.
[4] Komiti ea Thrombosis le Hemostasis ea Lipetlele tsa Mokhatlo oa Chaena oa Lipatlisiso. Tumellano mabapi le ho leka-lekanya ho fumanoa le ho tlaleha ha lupus anticoagulant [J].
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