ʻO ka hoʻāʻo lupus anticoagulant (LA) he ʻāpana koʻikoʻi ia o ka hoʻāʻo hale hana no nā antibodies antiphospholipid a ua ʻōlelo ʻia no ka hoʻohana ʻana i nā ʻano kūlana lapaʻau like ʻole, e like me ka hōʻoia ʻana o ka maʻi antiphospholipid (APS) a me ka systemic lupus erythematosus (SLE), ka loiloi pilikia o ka venous thromboembolism (VTE), a me ka wehewehe ʻana i ka manawa thromboplastin hapa i hoʻāla ʻole ʻia (APTT). E kōkua kēia ʻatikala iā ʻoe e kamaʻāina me ke ʻano o ka antiphospholipid syndrome (APS).
ʻO ka maʻi Antiphospholipid (APS) kahi maʻi autoimmune me nā hanana thrombotic vascular e hana hou ʻia, ka hoʻopau koke ʻana, ka thrombocytopenia, a me nā mea ʻē aʻe ma ke ʻano he mau hōʻike lapaʻau nui, me ka mau ʻana o ka medium a me ke kiʻekiʻe titer positive antiphospholipid antibody spectrum (aPLs). Hoʻokaʻawale pinepine ʻia i APS mua a me APS lua, ʻo ka mea hope loa he lua ia i nā maʻi pili e like me ka systemic lupus erythematosus (SLE) a me ka maʻi Sjögren. He paʻakikī a ʻokoʻa nā hōʻike lapaʻau o APS, a hiki ke hoʻopilikia ʻia nā ʻōnaehana āpau o ke kino, me ka hōʻike koʻikoʻi loa ʻo ka thrombosis vascular. ʻO ka pathogenesis o APS ʻo ia ka hoʻopili ʻana o ka aPL i nā phospholipids ʻili cell a me nā protein phospholipid-binding, e hoʻoulu ana i nā cell endothelial, PLTs a me wBc, e alakaʻi ana i nā hanana thrombotic vascular a me nā pilikia obstetric, a me ka hoʻolaha ʻana i ka hanana o nā pilikia autoimmune a me nā inflammatory. ʻOiai he pathogenic aPL, hana wale ka thrombosis i kekahi manawa, e hōʻike ana he mea nui nā "hahau lua" pōkole e like me ka maʻi, ka mumū, ke ʻoki ʻana, ka hāpai ʻana a me nā mea hoʻoulu ʻē aʻe i ke kaʻina hana o ka thrombosis.
ʻO ka ʻoiaʻiʻo, ʻaʻole ia he mea maʻamau ʻole ka APS. Ua hōʻike ʻia nā noiʻi he 25% o nā maʻi me ka hahau ʻana i wehewehe ʻole ʻia ma lalo o 45 mau makahiki he aPLs maikaʻi, 14% o nā maʻi me nā hanana thrombosis venous recurrent he aPLs maikaʻi, a he 15% a 20% o nā maʻi wahine me ka nalowale hāpai hou he aPLs maikaʻi. Ma muli o ka nele o ka hoʻomaopopo ʻana o nā kauka i kēia ʻano maʻi, ʻo ka awelika o ka manawa lohi o ka ʻike ʻana o APS ma kahi o 2.9 mau makahiki. ʻOi aku ka maʻamau o ka APS i nā wahine, me ka lakio wahine: kāne o 9:1, a ʻoi aku ka maʻamau i nā poʻe ʻōpio a me ka poʻe waena, akā he 12.7% o nā maʻi he >50 mau makahiki.
1. Nā hanana Thrombotic
ʻO nā hōʻike lapaʻau o ka thrombosis vascular i ka APS e pili ana i ke ʻano, kahi a me ka nui o nā kīʻaha koko i hoʻopilikia ʻia, a hiki ke hōʻike ʻia ma ke ʻano he mau kīʻaha koko hoʻokahi a nui paha i pili. ʻOi aku ka maʻamau o ka Venous thromboembolism (VTE) i ka APS, ʻoi aku ka maʻamau i nā veins hohonu o nā ʻaoʻao haʻahaʻa. Hiki iā ia ke hoʻopilikia i nā sinuses venous intracranial, retina, subclavian, ate, nā puʻupaʻa, a me nā vena cava kiʻekiʻe a me lalo. ʻO ka APS arterial thrombosis (AT) ka mea maʻamau i nā aʻa intracranial, a hiki ke hoʻopilikia i nā aʻa renal, nā aʻa coronary, nā aʻa mesenteric, a pēlā aku. Eia kekahi, hiki i nā maʻi APS ke loaʻa ka thrombosis microvascular i ka ʻili, nā maka, ka puʻuwai, nā māmā, nā puʻupaʻa a me nā ʻōpū ʻē aʻe. Ua ʻike ʻia ka meta-analysis he nui ka pilikia o ka lupus anticoagulant (LA) positivity i ka thromboembolism ma mua o nā antiphospholipid antibodies (acL); Ua hōʻike ʻia nā haʻawina lapaʻau e hōʻike ana nā poʻe maʻi APS me ka aPL maikaʻi [ie, LA, aCL, glycoprotein I antibodies (αβGPI) positivity] i kahi pilikia kiʻekiʻe o ka thrombosis, me ka helu thrombosis o 44.2% i loko o 10 mau makahiki.
2. Ka hāpai ʻana o ka maʻi
ʻO ke ʻano o ka pathophysiology o nā hōʻike obstetric o APS he paʻakikī like a hiki ke ʻokoʻa e like me ke kahua o ka hāpai ʻana, e hopena ana i ka heterogeneity o nā hiʻohiʻona lapaʻau i ʻike ʻia. ʻO ka mumū, ka hoʻoulu ʻana o ka complement, a me ka thrombosis placental ua manaʻo ʻia he mau mea pathogenic o ka obstetric APS. ʻO ka hāpai pathological i hoʻokumu ʻia e APS kekahi o nā kumu liʻiliʻi e hiki ke pale ʻia a mālama ʻia, a hiki i ka hoʻokele kūpono ke hoʻomaikaʻi maikaʻi i nā hopena hāpai. Ua ʻike ʻia kahi meta-analysis i paʻi ʻia ma 2009 ua pili nui ka loaʻa ʻana o LA a me aCL me ka make ʻana o ka fetal ma mua o 10 mau pule o ka hāpai ʻana; ua ʻike pū ʻia kahi loiloi ʻōnaehana hou a me ka meta-analysis ua pili pono ka LA positivity me ka make ʻana o ka fetal. I nā poʻe maʻi i ʻike ʻia he APS, ʻo ka pilikia o ka make ʻana o ka fetal he kiʻekiʻe mau ia e like me 10% a 12% me ka mālama maʻamau o ka heparin a me ka aspirin haʻahaʻa. No nā poʻe maʻi APS me nā hōʻailona koʻikoʻi o ka preeclampsia a i ʻole ka lawa ʻole o ka placental, pili nui ka loaʻa ʻana o LA a me aCL me ka preeclampsia; ʻo ka hāʻule mua ʻana (<10 mau pule o ka hāpai ʻana) he pilikia obstetric e noʻonoʻo pinepine ana i ka hiki ke loaʻa iā APS.
1.Thrombocytopenia
ʻO ka Thrombocytopenia kekahi o nā hōʻike lapaʻau maʻamau o nā maʻi APS, me ka nui o 20% ~ 53%. ʻO ka maʻamau, ʻoi aku ka maʻalahi o ka SLE secondary APS i ka thrombocytopenia ma mua o ka APS mua. ʻO ke kiʻekiʻe o ka thrombocytopenia i nā maʻi APS he akahai a waena paha. ʻO ka pathogenesis hiki ke komo pū me nā aPL e hoʻopaʻa pololei ana i nā platelets e hoʻāla a hōʻuluʻulu i nā platelets, ka ʻai ʻana o ka microangiopathy thrombotic, ka ʻai ʻana i ka nui o ka thrombosis, ka hoʻonui ʻia ʻana o ka paʻa ʻana i loko o ka spleen, a me nā hopena maikaʻi ʻole e pili ana i nā lāʻau anticoagulant i hōʻike ʻia e heparin. No ka mea hiki i ka thrombocytopenia ke hoʻonui i ka pilikia o ke kahe ʻana o ke koko, hopohopo nā kauka lapaʻau e pili ana i ka hoʻohana ʻana i ka lāʻau antithrombotic i nā maʻi APS me ka thrombocytopenia, a manaʻoʻiʻo hewa hoʻi e hiki i ka APS thrombocytopenia ke hōʻemi i ka pilikia o ka hoʻi hou ʻana o nā hanana thrombotic i nā maʻi. ʻO ka ʻoiaʻiʻo, ma ke ʻano ʻē, ua hōʻike nā haʻawina ua hoʻonui nui ʻia ka pilikia o ka hoʻi hou ʻana o nā hanana thrombotic i nā maʻi APS me ka thrombocytopenia, no laila pono e mālama ʻia me ka ikaika.
2. He maʻi laha ʻole ʻo CAPS, he maʻi hoʻoweliweli ola i hōʻike ʻia e nā embolism vascular he nui (≥3) i loko o kahi helu liʻiliʻi o nā maʻi APS i loko o kahi manawa pōkole (≤7 mau lā), maʻamau me nā titers kiʻekiʻe, e hoʻopilikia ana i nā kīʻaha koko liʻiliʻi, a me ka hōʻoia histopathological o ka thrombosis i loko o nā kīʻaha koko liʻiliʻi. Hoʻomau ka APL positivity i loko o 12 mau pule, e hoʻoulu ai i ka hāʻule ʻana o nā ʻōpū he nui a me ka pilikia o ka make, i ʻike ʻia ʻo catastropholipid syndrome catastrophic. ʻO kona hanana ma kahi o 1.0%, akā ʻo ka nui o ka make he 50% ~ 70%, pinepine ma muli o ka hahau ʻana, encephalopathy, hemorrhage, maʻi, a pēlā aku. ʻO kona pathogenesis hiki ke hoʻokumu ʻia ʻo ka ʻino thrombotic a me ka ʻino inflammatory i loko o kahi manawa pōkole.
ʻO aPLs kahi huaʻōlelo laulā no kahi hui o nā autoantibodies me nā phospholipids a/a i ʻole nā protein phospholipid-binding e like me nā antigens target. Loaʻa nui nā aPL i nā poʻe maʻi me nā maʻi autoimmune e like me APS, SLE, a me Sjögren's syndrome. ʻO lākou nā māka hoʻokolohua ʻano nui o APS a me nā mea wānana pilikia nui o nā hanana thrombotic a me ka hāpai pathological i nā poʻe maʻi APS. I waena o lākou, ʻo ka lupus anticoagulant (LA), nā antibodies anticardiolipin (aCL), a me nā antibodies anti-β-glycoprotein I (αβGPⅠ), ma ke ʻano he mau hōʻailona hoʻokolohua ma ke kūlana hoʻonohonoho APS, ua hoʻohana nui ʻia i ka hana lapaʻau a ua lilo i hoʻokahi o nā hoʻokolohua autoantibody maʻamau i nā keʻena hoʻokolohua lapaʻau.
Ke hoʻohālikelike ʻia me nā antibodies aCL a me anti-βGPⅠ, ʻoi aku ka ikaika o ka pilina o LA me ka thrombosis a me ka hāpai pathological. ʻOi aku ka nui o ka pilikia o ka thrombosis o LA ma mua o ka acL. A pili loa ia i ka hāʻule ʻana o ka hāpai ʻana ma mua o 10 mau pule. I ka pōkole, ʻo ka LA maikaʻi mau ka mea wānana hoʻokahi kūpono loa o ka pilikia thrombotic a me ka maʻi hāpai.
He hoʻāʻo hana ʻo LA e hoʻoholo ai inā loaʻa i ke kino ka LA ma muli o ka hiki i ka LA ke hoʻolōʻihi i ka manawa coagulation o nā ala like ʻole e pili ana i ka phospholipid in vitro. ʻO nā ʻano ʻike o LA:
1. Hoʻāʻo nānā: me ka manawa venom viper diluted (dRVVT), ka manawa thromboplastin hapa i hoʻāla ʻia (APTT), ke ʻano manawa coagulation silica, ka manawa coagulation nahesa nui a me ka manawa enzyme vein snake. I kēia manawa, ʻōlelo nā alakaʻi ʻike aPLs honua e like me ka International Society on Thrombosis and Haemostasis (ISTH) a me ka Clinical Laboratory Standards Institute (CLSI) e ʻike ʻia ʻo LA e ʻelua mau ala coagulation like ʻole. I waena o lākou, ʻo dRVVT a me APTT nā ʻano ʻike i hoʻohana nui ʻia ma ka honua holoʻokoʻa. ʻO ka maʻamau, hoʻohana ʻia ʻo dRVVT ma ke ʻano he koho mua, a ʻo ka APTT ʻoi aku ka naʻau (low phospholipids a silica paha ma ke ʻano he activator) e hoʻohana ʻia ma ke ʻano he ʻano ʻelua.
2. Hoʻāʻo hoʻohuihui: Hoʻohuihui ʻia ka plasma maʻi me ka plasma olakino (1:1) e hōʻoia i ka manawa coagulation lōʻihi ʻaʻole ma muli o ka nele o nā mea coagulation.
3. Hoʻāʻo hōʻoia: Hoʻololi ʻia ka nui a i ʻole ka haku mele ʻana o nā phospholipids e hōʻoia i ke alo o LA.
He mea kūpono ke hoʻomaopopo ʻana he pono e hōʻiliʻili ʻia ka hāpana kūpono no LA mai nā poʻe maʻi i loaʻa ʻole ka lāʻau anticoagulant, no ka mea, ʻo nā poʻe maʻi i mālama ʻia me warfarin, heparin, a me nā anticoagulants waha hou (e like me rivaroxaban) hiki ke loaʻa nā hopena hoʻāʻo LA wahaheʻe; no laila, pono e wehewehe pono ʻia nā hopena hoʻāʻo LA o nā poʻe maʻi e loaʻa ana ka lāʻau anticoagulant. Eia kekahi, pono e wehewehe pono ʻia ka hoʻāʻo LA ma ke ʻano lapaʻau koʻikoʻi, no ka mea, ʻo ka piʻi ʻana o nā pae protein C-reactive hiki ke hoʻopilikia i nā hopena hoʻāʻo.
He maʻi autoimmune ka APS me nā hanana thrombotic vascular hou, ka hoʻopau hāpai hou ʻana, ka thrombocytopenia, a me nā mea ʻē aʻe ma ke ʻano he mau hōʻike lapaʻau nui, i hele pū ʻia me nā titers waena a kiʻekiʻe mau o aPLs.
ʻO APS kekahi o nā kumu liʻiliʻi hiki ke mālama ʻia o ka hāpai keiki pathological. ʻO ka mālama pono ʻana i ka APS hiki ke hoʻomaikaʻi maikaʻi i nā hopena hāpai.
Ma ka hana lapaʻau, pono e hoʻokomo pū ʻia ka APS i nā maʻi me nā hōʻike lapaʻau e pili ana i ka aPLs e like me ka livedo reticularis, thrombocytopenia, a me ka maʻi valve puʻuwai, a me ka poʻe e hoʻokō ana i nā pae hoʻohālikelike lapaʻau a he mau titers haʻahaʻa mau o aPLs. Loaʻa pū i kēlā mau maʻi ka pilikia o nā hanana thrombotic a me ka hāpai pathological.
ʻO nā pahuhopu lapaʻau o APS ka pale ʻana i ka thrombosis a me ka pale ʻana i ka hāʻule ʻana o ka hāpai ʻana.
Nā Kuhikuhi
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, et al. Nā alakaʻi no ka ʻike ʻana a me ka mālama ʻana no ka maʻi antiphospholipid[J]. Chinese Journal of Internal Medicine
[2] Bu Jin, Liu Yuhong. Nā holomua i ka ʻike a me ka mālama ʻana i ka maʻi antiphospholipid[J]. Journal of Clinical Internal Medicine
[3] ALAKAʻI BSH Nā alakaʻi no ka noiʻi ʻana a me ka mālama ʻana i ka maʻi antiphospholipid.
[4] Kōmike Thrombosis a me Hemostasis o ka Hui Kina o nā Halemai Noiʻi. ʻO ka ʻaelike ma ke kūlana maʻamau o ka ʻike ʻana a me ka hōʻike ʻana i ka lupus anticoagulant [J].
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