Kuyesa kwa lupus anticoagulant (LA) ndi gawo lofunika kwambiri pa mayeso a labotale a ma antibodies a antiphospholipid ndipo kwalimbikitsidwa kuti kugwiritsidwe ntchito pazochitika zosiyanasiyana zachipatala, monga kuzindikira kwa labu ya antiphospholipid syndrome (APS) ndi systemic lupus erythematosus (SLE), kuwunika chiopsezo cha venous thromboembolism (VTE), ndi kufotokozera kwa nthawi yosasinthika ya activated partial thromboplastin (APTT). Nkhaniyi ikuthandizani kudziwa bwino tanthauzo la antiphospholipid syndrome (APS).
Matenda a Antiphospholipid (APS) ndi matenda a autoimmune omwe amachititsa kuti magazi azithamanga m'mitsempha yamagazi, kuchotsedwa mimba mwadzidzidzi, thrombocytopenia, ndi zina zotero monga zizindikiro zazikulu zachipatala, zomwe zimatsatiridwa ndi antiphospholipid antibody spectrum (aPLs) yomwe imachitika nthawi zonse komanso nthawi zambiri. Nthawi zambiri imagawidwa m'magulu a APS oyambira ndi APS yachiwiri, yomwe yachiwiri imayamba chifukwa cha matenda olumikizana ndi minofu monga systemic lupus erythematosus (SLE) ndi Sjögren's syndrome. Zizindikiro za matenda a APS ndi zovuta komanso zosiyanasiyana, ndipo machitidwe onse a thupi amatha kukhudzidwa, ndipo chizindikiro chachikulu kwambiri ndi thrombosis ya mitsempha yamagazi. Matenda a APS ndi akuti aPL yozungulira imalumikizana ndi ma phospholipids a pamwamba pa maselo ndi mapuloteni omangira phospholipid, kuyambitsa maselo a endothelial, PLTs ndi wBc, zomwe zimapangitsa kuti magazi azithamanga m'mitsempha yamagazi komanso mavuto obereka, ndikulimbikitsa kuchitika kwa zovuta zina za autoimmune ndi kutupa. Ngakhale kuti aPL ndi yowopsa, thrombosis imachitika nthawi zina, zomwe zikusonyeza kuti "kuukira kwachiwiri" kwakanthawi kochepa monga matenda, kutupa, opaleshoni, mimba ndi zina zomwe zimayambitsa thrombosis ndizofunikira kwambiri pakuyambitsa thrombosis.
Ndipotu, matenda a APS si achilendo. Kafukufuku wasonyeza kuti 25% ya odwala omwe ali ndi sitiroko yosamvetsetseka osakwana zaka 45 ali ndi aPL, 14% ya odwala omwe ali ndi vuto la venous thrombosis yobwerezabwereza ali ndi aPL, ndipo 15% mpaka 20% ya odwala achikazi omwe ali ndi mimba yobwerezabwereza ali ndi aPL. Chifukwa cha kusamvetsetsa mtundu uwu wa matenda ndi madokotala, nthawi yodziwika bwino ya matenda a APS ndi pafupifupi zaka 2.9. APS nthawi zambiri imapezeka kwambiri mwa akazi, ndipo chiŵerengero cha akazi: amuna ndi 9:1, ndipo imapezeka kwambiri mwa achinyamata ndi azaka zapakati, koma 12.7% ya odwala ali ndi zaka zoposa 50.
1. Zochitika za Thrombotic
Zizindikiro zachipatala za thrombosis ya mitsempha yamagazi mu APS zimadalira mtundu, malo ndi kukula kwa mitsempha yamagazi yomwe yakhudzidwa, ndipo zitha kuonekera ngati mitsempha yamagazi imodzi kapena zingapo zomwe zakhudzidwa. Venous thromboembolism (VTE) imapezeka kwambiri mu APS, makamaka m'mitsempha yakuya ya miyendo yapansi. Ingakhudzenso intracranial venous sinuses, retina, subclavian, chiwindi, impso, ndi vena cava yapamwamba komanso yotsika. APS arterial thrombosis (AT) imapezeka kwambiri m'mitsempha yamagazi yamkati, ndipo ingakhudzenso mitsempha ya impso, mitsempha yamtima, mitsempha ya mesenteric, ndi zina zotero. Kuphatikiza apo, odwala a APS amathanso kukhala ndi thrombosis ya mitsempha yamagazi pakhungu, m'maso, mumtima, m'mapapo, m'impso ndi ziwalo zina. Kusanthula kwa meta kunapeza kuti lupus anticoagulant (LA) positivity ili ndi chiopsezo chachikulu cha thromboembolism kuposa antiphospholipid antibodies (acL); Kafukufuku wazachipatala wasonyeza kuti odwala a APS omwe ali ndi aPL [monga LA, aCL, glycoprotein I antibodies (αβGPI) positivity] ali pachiwopsezo chachikulu cha thrombosis, kuphatikizapo thrombosis ya 44.2% mkati mwa zaka 10.
2. Mimba yodwala
Matenda a APS oyembekezera ndi ovuta kwambiri ndipo amatha kusiyana malinga ndi gawo la mimba, zomwe zimapangitsa kuti zizindikiro zachipatala zisiyane. Kutupa, kuyambitsa kowonjezera, ndi thrombosis ya placenta zonse zimaonedwa kuti ndi zinthu zomwe zimayambitsa matenda a APS oyembekezera. Mimba yoyambitsidwa ndi APS ndi imodzi mwa zifukwa zochepa zomwe zingapewedwe ndikuchiritsidwa, ndipo kuyang'aniridwa koyenera kungathandize bwino zotsatira za mimba. Kusanthula kwa meta komwe kunafalitsidwa mu 2009 kunapeza kuti kupezeka kwa LA ndi aCL kunagwirizana kwambiri ndi imfa ya mwana wosabadwayo patatha milungu yoposa 10 ya mimba; kuwunikanso kwaposachedwa komanso kusanthula kwa meta kunapezanso kuti LA positivity inali yogwirizana kwambiri ndi imfa ya mwana wosabadwayo. Kwa odwala omwe amadziwika kuti ali ndi APS, chiopsezo cha imfa ya mwana wosabadwayo chimakhala chokwera mpaka 10% mpaka 12% ngakhale atalandira chithandizo cha heparin ndi aspirin yochepa. Kwa odwala a APS omwe ali ndi zizindikiro zazikulu za preeclampsia kapena placenta insufficiency, kupezeka kwa LA ndi aCL kumagwirizanitsidwa kwambiri ndi preeclampsia; kubwerezabwereza kutayika msanga (
1. Kuchuluka kwa magazi m'thupi (thrombocytopenia)
Matenda a Thrombocytopenia ndi chimodzi mwa zizindikiro zodziwika bwino za odwala APS, ndipo amapezeka ndi 20% ~ 53%. Kawirikawiri, matenda achiwiri a SLE amakhala ndi vuto la thrombocytopenia kuposa matenda oyamba a APS. Mlingo wa thrombocytopenia mwa odwala APS nthawi zambiri umakhala wofatsa kapena wocheperako. Matenda omwe angachitike ndi monga aPLs omwe amamangirirana mwachindunji ndi ma platelet kuti ayambitse ndikusonkhanitsa ma platelet, kumwa mankhwala a thrombotic microangiopathy, kumwa mankhwala ambiri a thrombosis, kuchuluka kwa thrombosis mu ndulu, komanso zotsatira zoyipa zokhudzana ndi mankhwala oletsa magazi omwe amaimiridwa ndi heparin. Popeza thrombocytopenia ingawonjezere chiopsezo cha kutuluka magazi, asing'anga ali ndi nkhawa zina pankhani yogwiritsa ntchito mankhwala oletsa magazi mwa odwala APS omwe ali ndi thrombocytopenia, ndipo amakhulupirira molakwika kuti matenda a APS amatha kuchepetsa chiopsezo cha kubwereranso kwa matenda a thrombotic mwa odwala. M'malo mwake, kafukufuku wasonyeza kuti chiopsezo cha kubwereranso kwa matenda a thrombotic mwa odwala APS omwe ali ndi thrombocytopenia chikuwonjezeka kwambiri, kotero chiyenera kuthandizidwa mwachangu.
2. CAPS ndi matenda osowa, omwe amaika moyo pachiswe omwe amadziwika ndi ma vascular embolism angapo (≥3) mwa odwala ochepa a APS mkati mwa nthawi yochepa (≤masiku 7), nthawi zambiri amakhala ndi ma titers ambiri, omwe amakhudza mitsempha yaing'ono yamagazi, komanso kutsimikizika kwa histopathological kwa thrombosis m'mitsempha yaying'ono yamagazi. APL positivity imapitirira mkati mwa masabata 12, zomwe zimapangitsa kuti ziwalo zambiri zilephereke komanso chiopsezo cha imfa, chomwe chimadziwika kuti catastrophical antiphospholipid syndrome. Kuchuluka kwake ndi pafupifupi 1.0%, koma chiwerengero cha imfa ndi chokwera kufika pa 50% ~ 70%, nthawi zambiri chifukwa cha sitiroko, encephalopathy, hemorrhage, matenda, ndi zina zotero. Matenda ake omwe angayambitse ndi kupangika kwa thrombotic storm ndi inflammatory storm munthawi yochepa.
Ma aPL ndi mawu ofala a gulu la ma autoantibodies okhala ndi ma phospholipid ndi/kapena mapuloteni omangira ma phospholipid ngati ma antigen omwe amawaganizira. Ma aPL amapezeka makamaka mwa odwala omwe ali ndi matenda a autoimmune monga APS, SLE, ndi Sjögren's syndrome. Ndiwo zizindikiro zodziwika bwino za APS m'ma laboratories komanso zomwe zimawonetsa chiopsezo chachikulu cha zochitika za thrombosis ndi mimba yodwala mwa odwala APS. Pakati pawo, lupus anticoagulant (LA), anticardiolipin antibodies (aCL), ndi ma anti-β-glycoprotein I (αβGPⅠ), monga zizindikiro za labotale mu muyezo wa gulu la APS, akhala akugwiritsidwa ntchito kwambiri m'machipatala ndipo akhala amodzi mwa mayeso ofala kwambiri a autoantibodies m'ma laboratories azachipatala.
Poyerekeza ndi aCL ndi ma antibodies a anti-βGPⅠ, LA ili ndi mgwirizano wamphamvu ndi thrombosis ndi mimba yodwala. LA ili ndi chiopsezo chachikulu cha thrombosis kuposa acL. Ndipo imagwirizana kwambiri ndi kutaya mimba panthawi ya mimba yoposa masabata 10. Mwachidule, LA yokhazikika ndiyo njira imodzi yabwino kwambiri yodziwira chiopsezo cha thrombosis ndi matenda a mimba.
LA ndi mayeso ogwira ntchito omwe amatsimikiza ngati thupi lili ndi LA kutengera mfundo yakuti LA ikhoza kutalikitsa nthawi yotseka ya njira zosiyanasiyana zodalira phospholipid mu vitro. Njira zodziwira LA zikuphatikizapo:
1. Kuyesa koyezera: kuphatikiza nthawi ya poizoni wa njoka wochepetsedwa (dRVVT), nthawi yoyambitsa thromboplastin (APTT), njira ya nthawi yozungulira ya silica, nthawi yozungulira njoka yayikulu komanso nthawi ya enzyme ya mtsempha wa njoka. Pakadali pano, malangizo apadziko lonse lapansi ozindikira aPLs monga International Society on Thrombosis and Haemostasis (ISTH) ndi Clinical Laboratory Standards Institute (CLSI) amalimbikitsa kuti LA izindikirike ndi njira ziwiri zosiyana zozungulira. Pakati pawo, dRVVT ndi APTT ndi njira zodziwira zomwe zimagwiritsidwa ntchito kwambiri padziko lonse lapansi. Nthawi zambiri dRVVT imagwiritsidwa ntchito ngati njira yoyamba yosankhira, ndipo APTT yodziwika bwino (phospholipids yotsika kapena silica ngati activator) imagwiritsidwa ntchito ngati njira yachiwiri.
2. Kuyesa kosakaniza: Plasma ya wodwala imasakanizidwa ndi plasma yathanzi (1:1) kuti zitsimikizire kuti nthawi yayitali yothira magazi si chifukwa cha kusowa kwa zinthu zothira magazi.
3. Kuyesa kutsimikizira: Kuchuluka kapena kapangidwe ka ma phospholipids kumasinthidwa kuti kutsimikizire kukhalapo kwa LA.
Ndikofunika kudziwa kuti chitsanzo choyenera cha LA chiyenera kutengedwa kuchokera kwa odwala omwe sanalandire chithandizo cha anticoagulant, chifukwa odwala omwe amalandira warfarin, heparin, ndi mankhwala atsopano oletsa magazi kuundana (monga rivaroxaban) akhoza kukhala ndi zotsatira zabodza za mayeso a LA; chifukwa chake, zotsatira za mayeso a LA a odwala omwe amalandira chithandizo cha anticoagulant ziyenera kutanthauziridwa mosamala. Kuphatikiza apo, mayeso a LA ayeneranso kutanthauziridwa mosamala m'malo azachipatala owopsa, chifukwa kukwera kwadzidzidzi kwa milingo ya mapuloteni a C-reactive kungasokonezenso zotsatira za mayeso.
APS ndi matenda odziteteza okha omwe amachititsa kuti magazi azituluka m'magazi, kuchotsa mimba mwadzidzidzi, thrombocytopenia, ndi zina zotero, monga zizindikiro zazikulu zachipatala, zomwe zimatsagana ndi kuchuluka kwa aPLs m'magazi.
APS ndi chimodzi mwa zifukwa zochepa zomwe zimayambitsa matenda a mimba zomwe zingathe kuchiritsidwa. Kusamalira bwino APS kungathandize kwambiri pakukula kwa mimba.
Mu ntchito zachipatala, APS iyeneranso kuphatikizapo odwala omwe ali ndi zizindikiro zokhudzana ndi aPL monga livedo reticularis, thrombocytopenia, ndi matenda a mtima, komanso omwe akukwaniritsa zofunikira zachipatala ndipo ali ndi ma titer otsika a aPL. Odwala oterewa alinso ndi chiopsezo cha zochitika za thrombosis ndi mimba yodwala.
Zolinga za chithandizo cha APS makamaka ndi kupewa thrombosis ndi kupewa kulephera kutenga mimba.
Zolemba
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, ndi ena. Malangizo ozindikira ndi kuchiza matenda a antiphospholipid [J]. Chinese Journal of Internal Medicine
[2] Bu Jin, Liu Yuhong. Kupita patsogolo pakupeza ndi kuchiza matenda a antiphospholipid [J]. Journal of Clinical Internal Medicine
[3] Malangizo a BSH okhudza kufufuza ndi kusamalira matenda a antiphospholipid.
[4] Komiti ya Thrombosis ndi Hemostasis ya Zipatala za Chinese Society of Research. Kugwirizana pa kukhazikitsa miyezo ya kuzindikira ndi kupereka malipoti a lupus anticoagulant [J].
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