He wāhanga nui te whakamātautau lupus anticoagulant (LA) o te whakamātautau taiwhanga mō ngā paturopi antiphospholipid, ā, kua tūtohutia kia whakamahia i roto i ngā āhuatanga haumanu maha, pērā i te tātaritanga taiwhanga o te mate antiphospholipid (APS) me te lupus erythematosus systemic (SLE), te aromatawai mōrearea o te venous thromboembolism (VTE), me te whakamārama mō te wā roa o te activated partial thromboplastin time (APTT) kāore i whakamāramahia. Ma tēnei tuhinga koe e āwhina kia mōhio ai ki te tikanga o te mate antiphospholipid (APS).
Ko te mate ārai-phospholipid (APS) he mate pūnaha ārai-tinana me ngā mate toto toto auau, te whakatahe ohorere auau, te thrombocytopenia, me ētahi atu hei whakaaturanga haumanu matua, me te haere tahi me te whānuitanga paturopi antiphospholipid pai (aPL) waenga me te teitei. I te nuinga o te wā ka wehea ki te APS tuatahi me te APS tuarua, ko te mea whakamutunga he tuarua ki ngā mate kiko honohono pēnei i te systemic lupus erythematosus (SLE) me te mate Sjögren. He uaua, he kanorau hoki ngā whakaaturanga haumanu o te APS, ā, ka pāngia ngā pūnaha katoa o te tinana, ko te whakaaturanga tino kitea ko te toto toto toto. Ko te pathogenesis o te APS ko te aPL e rere ana e herea ana ki ngā phospholipid mata pūtau me ngā pūmua here-phospholipid, ka whakahohe i ngā pūtau endothelial, PLT me te wBc, ka arahi ki ngā mate toto toto toto me ngā raruraru whānau, me te whakatairanga i te putanga mai o ētahi atu raruraru pūnaha ārai-tinana me te mumura. Ahakoa he pathogenic te aPL, ka puta noa te toto toto i ētahi wā, e tohu ana he mea nui ngā "pānga tuarua" poto pēnei i te mate, te mumura, te pokanga, te haputanga me ētahi atu mea whakaoho i roto i te tukanga o te toto toto.
Ina hoki, ehara i te mea onge te APS. Kua whakaatuhia e ngā rangahau e 25% o ngā tūroro e pāngia ana e te whiu kāore i whakamāramatia i raro i te 45 tau te pakeke he pai te aPL, 14% o ngā tūroro e pāngia ana e ngā mate toto toto uaua e hoki mai ana he pai te aPL, ā, 15% ki te 20% o ngā tūroro wahine e ngaro ana te haputanga e hoki mai ana he pai te aPL. Nā te kore o te māramatanga o ngā tākuta ki tēnei momo mate, ko te roanga o te wā tātaritanga o te APS he tata ki te 2.9 tau. He nui ake te kitea o te APS i roto i ngā wāhine, me te ōwehenga wahine: tāne he 9:1, ā, he nui ake te kitea i roto i ngā taiohi me ngā tāngata waenga-pakeke, engari 12.7% o ngā tūroro he >50 tau te pakeke.
1. Ngā takahanga toto totoka
Ko ngā whakaaturanga haumanu o te thrombosis vascular i roto i te APS e whakawhirinaki ana ki te momo, te wāhi me te rahi o ngā oko toto e pāngia ana, ā, ka taea te whakaatu hei oko toto kotahi, maha rānei e pāngia ana. Ko te thromboembolism venous (VTE) he mea noa ake i roto i te APS, ko te nuinga i roto i ngā uaua hohonu o ngā pito o raro. Ka taea hoki e ia te pā ki ngā sinuses venous intracranial, retina, subclavian, ate, whatukuhu, me te vena cava o runga me raro. Ko te thrombosis arterial APS (AT) he mea noa ake i roto i ngā uaua intracranial, ā, ka taea hoki e ia te pā ki ngā uaua renal, ngā uaua coronary, ngā uaua mesenteric, me ētahi atu. Hei tāpiri, tera pea ka pāngia ngā tūroro APS e te thrombosis microvascular i roto i te kiri, ngā kanohi, te ngākau, ngā pukahukahu, ngā whatukuhu me ētahi atu okana. I kitea e te tātaritanga meta he nui ake te mōrearea o te lupus anticoagulant (LA) positivity ki te thromboembolism i ngā antibodies antiphospholipid (acL); Kua whakaatuhia e ngā rangahau haumanu he nui te mōrearea o te thrombosis i roto i ngā tūroro APS me te aPL pai [arā, LA, aCL, glycoprotein I antibodies (αβGPI) positivity], tae atu ki te reiti thrombosis o te 44.2% i roto i ngā tau 10.
2. Te haputanga mate pāwera
He rite tonu te uaua o te pathophysiology o ngā whakaaturanga o te APS i roto i te whānautanga, ā, ka rerekē pea i runga i te wāhanga o te haputanga, ka hua ake he rerekētanga o ngā āhuatanga haumanu kua kitea. Ko te mumura, te whakahohenga o ngā tāpiringa, me te thrombosis o te whenua whānau katoa e kiia ana he āhuatanga mate o te APS o te whānautanga. Ko te haputanga mate i puta mai i te APS tētahi o ngā take torutoru ka taea te ārai me te rongoā, ā, mā te whakahaere tika ka taea te whakapai ake i ngā putanga o te haputanga. I kitea i roto i tētahi tātaritanga-maha i whakaputaina i te tau 2009 ko te aroaro o te LA me te aCL he hononga nui ki te matenga o te kukune i te >10 wiki o te haputanga; i kitea anō hoki i roto i tētahi arotake pūnaha me te tātaritanga-maha tata nei ko te pai o te LA he hononga tata ki te matenga o te kukune. I roto i ngā tūroro e mōhiotia ana he APS tō rātou, ko te mōrearea o te matenga o te kukune kei te 10% ki te 12% ahakoa te maimoatanga paerewa o te heparin me te aspirin iti-horopeta. Mō ngā tūroro APS he tohu kino o te preeclampsia, te ngoikore rānei o te whenua whānau, ko te aroaro o te LA me te aCL he hononga nui ki te preeclampsia; ko te materoto wawe tonu (<10 wiki o te haputanga) he raruraru whānau e whakaaro ana ki te tupono o te APS.
1. Te mate toto toto
Ko te thrombocytopenia tētahi o ngā whakaaturanga haumanu noa o ngā tūroro APS, me te auau o te 20% ~ 53%. I te nuinga o te wā, ko te SLE APS tuarua he ngawari ake te pāngia e te thrombocytopenia i te APS tuatahi. Ko te nui o te thrombocytopenia i roto i ngā tūroro APS he ngawari, he taurite rānei. Ko ngā pathogenesis pea ko te here tika o ngā aPL ki ngā platelets hei whakahohe me te kohikohi i ngā platelets, te kai o te microangiopathy thrombotic, te kai o te nui o te thrombosis, te pikinga o te pupuri i roto i te whēkau, me ngā urupare kino e pā ana ki ngā rongoā anticoagulant e tohuhia ana e te heparin. Nā te mea ka piki ake te mōrearea o te toto i te thrombocytopenia, he āwangawanga ā ngā tākuta mō te whakamahinga o te rongoā antithrombotic i roto i ngā tūroro APS me te thrombocytopenia, ā, e whakapono hē ana ka taea e te thrombocytopenia APS te whakaiti i te mōrearea o te hokinga mai o ngā huihuinga thrombotic i roto i ngā tūroro. Inaa, i te ritenga ke, kua whakaatuhia e ngā rangahau he nui ake te mōrearea o te hokinga mai o ngā huihuinga thrombotic i roto i ngā tūroro APS me te thrombocytopenia, nō reira me kaha ake te rongoā.
2. He mate onge, he mate morearea te CAPS, e tohuhia ana e te maha (≥3) ngā embolism vascular i roto i te tokoiti o ngā tūroro APS i roto i te wā poto (≤7 rā), he nui ngā titers, e pā ana ki ngā oko toto iti, me te whakaū histopathological o te thrombosis i roto i ngā oko toto iti. Ka mau tonu te APL pai i roto i ngā wiki 12, ka puta te ngoikore o ngā okana maha me te mōrearea o te mate, e mōhiotia ana ko te catastropholipid syndrome catastrophic. Ko tōna auau he tata ki te 1.0%, engari ko te reiti mate he teitei ki te 50% ~ 70%, he maha ngā wā ka puta mai i te whiu, te encephalopathy, te toto, te mate, me ētahi atu. Ko tōna pathogenesis pea ko te hanganga o te thrombotic storm me te inflammatory storm i roto i te wā poto.
Ko ngā aPL he kupu whānui mō tētahi rōpū o ngā autoantibodies me ngā phospholipids me/ranei ngā pūmua here phospholipid hei antigens ūnga. Ko ngā aPL ka kitea nuitia i roto i ngā tūroro he mate pūnaha ārai mate pērā i te APS, te SLE, me te mate Sjögren. Koinei ngā tohu taiwhanga tino kitea o te APS me ngā tohu mōrearea matua o ngā huihuinga toto toto me te haputanga pathological i roto i ngā tūroro APS. I roto i ēnei, ko te lupus anticoagulant (LA), ngā anticardiolipin antibodies (aCL), me ngā anti-β-glycoprotein I (αβGPⅠ), hei tohu taiwhanga i roto i te paerewa whakarōpūtanga APS, kua whakamahia whānuitia i roto i te mahi haumanu, ā, kua noho hei tētahi o ngā whakamātautau autoantibody tino kitea i roto i ngā taiwhanga haumanu.
Ki te whakatauritea ki ngā paturopi aCL me te anti-βGPⅠ, he kaha ake te hononga o te LA ki te thrombosis me te hāputanga pathological. He nui ake te mōrearea o te LA ki te thrombosis i te acL. Ā, he hononga tata ki te mate whānau i te wā e hapu ana >10 wiki. I roto i te poto, ko te LA pumau tonu te tohu kotahi tino whai hua mō te mōrearea thrombosis me te mate hāputanga.
He whakamātautau mahi a te LA e whakatau ana mēnā kei te tinana te LA i runga i te meka ka taea e te LA te whakaroa i te wā totoka o ngā ara rerekē e whakawhirinaki ana ki te phospholipid i roto i te vitro. Ko ngā tikanga kimi i te LA ko:
1. Whakamātautau tirotiro: tae atu ki te wā paitini nakahi kua diluted (dRVVT), te wā thromboplastin wāhanga whakahohe (APTT), te tikanga wā whakapūmau silica, te wā whakapūmau nakahi nunui me te wā whākōkī uaua nakahi. I tēnei wā, ko ngā aratohu kimi aPL o te ao pērā i te International Society on Thrombosis and Haemostasis (ISTH) me te Clinical Laboratory Standards Institute (CLSI) e taunaki ana kia kitea te LA mā roto i ngā ara whakapūmau e rua. I roto i ēnei, ko te dRVVT me te APTT ngā tikanga kimi e whakamahia whānuitia ana i te ao. Ko te tikanga ko te dRVVT te tikanga tuatahi e whakamahia ana, ā, ko te APTT tairongo ake (he iti ngā phospholipids, he silica rānei hei whakahohe) te tikanga tuarua.
2. Whakamātautau whakaranu: Ka whakaranua te plasma tūroro ki te plasma hauora (1:1) hei whakaū kāore te roa o te wā totohu i puta mai i te korenga o ngā āhuatanga totohu.
3. Whakamātautau whakaū: Ka whakarerekētia te kukū, te hanganga rānei o ngā phospholipid hei whakaū i te aroaro o te LA.
He mea tika kia mōhiotia ko te tauira tino pai mō te LA me kohi mai i ngā tūroro kāore anō kia whiwhi i te rongoā ārai toto, nā te mea ko ngā tūroro e rongoatia ana ki te warfarin, heparin, me ngā rongoā ārai toto hou (pērā i te rivaroxaban) tera pea ka puta he hua whakamātautau LA teka-pai; nō reira, me āta whakamārama i ngā hua whakamātautau LA o ngā tūroro e whiwhi ana i te rongoā ārai toto. Hei tāpiri, me āta whakamārama hoki i te whakamātautau LA i roto i te horopaki haumanu ohorere, nā te mea ka taea hoki e te pikinga ohorere o ngā taumata pūmua C-reactive te whakararu i ngā hua whakamātautau.
He mate pūnaha ārai mate te APS me ngā mate toto totoka auau, te whakatahe ohorere auau, te thrombocytopenia, me ētahi atu, hei tohu haumanu matua, me te titer waenga me te teitei pumau o ngā aPL.
Ko te APS tētahi o ngā tino take ka taea te rongoā mō te hāputanga pathological. Mā te whakahaere tika i te APS ka taea te whakapai ake i ngā putanga hāputanga.
I roto i ngā mahi haumanu, me whakauru hoki ki te APS ngā tūroro he tohu haumanu e pā ana ki te aPLs pērā i te livedo reticularis, te thrombocytopenia, me te mate takirere ngākau, tae atu ki te hunga e tutuki ana i ngā paearu whakarōpū haumanu, ā, he iti tonu ngā taitara o te aPLs. Ka tūpono hoki ēnei tūroro ki ngā mate toto toto me te hāputanga pathological.
Ko ngā whāinga matua mō te maimoatanga o te APS ko te aukati i te toto toto me te karo i te korenga o te haputanga.
Ngā Tohutoro
[1] Zhao Jiuliang, Shen Haili, Chai Kexia, me ētahi atu. Ngā aratohu mō te tātaritanga me te maimoatanga mō te mate antiphospholipid[J]. Chinese Journal of Internal Medicine
[2] Bu Jin, Liu Yuhong. Ngā whanaketanga i roto i te tātaritanga me te maimoatanga o te mate antiphospholipid[J]. Journal of Clinical Internal Medicine
[3] ARATOHU BSH Ngā aratohu mō te rangahau me te whakahaere i te mate antiphospholipid.
[4] Komiti Thrombosis me te Hemostasis o te Rōpū Hōhipera Rangahau Hainamana. He whakaae tahi mō te whakapūmautanga o te kitenga me te pūrongo i ngā rongoā ārai mate lupus [J].
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