Lefu la Antiphospholipid - APS

Antiphospholipid syndrome (APS) ke boloetse bo ikemetseng bo kenyang mali khafetsa (thromboses)Ha u e-na le boemo bona, sesole sa hau sa 'mele se etsa liprotheine tse sa tloaelehang tse hlaselang lisele tsa mali le lera la methapo ea mali. Boteng ba lisireletsi tsena bo ka baka mathata ka phallo ea mali mme ha lebisa masoleng a kotsi methapong ea mali 'meleng oohle.

Lebaka le tobileng la APS ha le tsejoe. Liphetoho tse ling tsa liphatsa tsa lefutso le lintho tse ling (joalo ka ts'oaetso) li ka baka bothata ho hlaha.

Hangata e fumanoa ho batho ba nang le mafu a mang a ikemetseng, joalo ka systemic lupus erythematosus (SLE). Boemo bona ke basali ba tloaelehileng ho feta banna, Hangata bo fumanoa ho basali ba nang le nalane ea ho senyeheloa hangata.

Batho ba bang ba na le lithibela-mafu tse boletsoeng ka holimo, empa ha ba na APS. Lisosa tse ling li ka etsa hore batho bana ba be le mali, ho kenyelletsa:

• Ho tsuba
• Phomolo ea nako e telele ea bethe
• Boimana
• Phekolo ea Hormone kapa lipilisi tsa thibelo ea bokhachane
• Kankere
• Lefu la liphio

O kanna oa se be le matšoao, leha o na le lithibela-mafu. Matšoao a ka hlahang a kenyelletsa:

• Mali a koahetsoe ke maoto, matsoho kapa matšoafo. Masoapo a ka ba methapong kapa methapong ea methapo.
• Ho senyeheloa hangata kapa ho tsoaloa.
• Rash, ho batho ba bang.

Maemong a sa tloaelehang, maqhubu a hlaha ka tšohanyetso methapong e mengata ka matsatsi a mangata. Sena se bitsoa lefu le kotsi la anti-phospholipid syndrome (CAPS). E ka lebisa ho stroke le ho koaloa hoa liphio, sebete le litho tse ling 'meleng oohle le ho ruruha ha maoto le matsoho.

Liteko tsa lithibela-mafu tsa lupus le antiphospholipid li ka etsoa ha:

• Ho ba le mali a sa lebelloang hoa etsahala, joalo ka ho bacha kapa ba se nang mabaka a mang a kotsi a ho koala mali.
• Mosali o na le nalane ea ho lahleheloa ke bokhachane khafetsa.

Liteko tsa lupus anticoagulant ke liteko tsa ho koala mali. Li-antibodies tsa antiphospholipid (aPL) li etsa hore tlhahlobo e be e sa tloaelehang ka laboratoring.

Mefuta ea liteko tsa ho koala e ka kenyelletsa:

• Nako e sebelisitsoeng ea karoloana ea thromboplastin (aPTT)
• Nako ea mahloko ea Russell
• Teko ea thibelo ea Thromboplastin

Ho tla etsoa liteko tsa lithibela-mafu tsa antiphospholipid (aPL). Li kenyeletsa:

• Liteko tsa Anticardiolipin antibody
• Masole a mmele ho beta-2-glypoprotein I (Beta2-GPI)

Mofani oa hao oa tlhokomelo ea bophelo o tla hlahloba lefu la antiphospholipid antibody syndrome (APS) haeba u e-na le teko e nepahetseng bakeng sa aPL kapa lupus anticoagulant, le ketsahalo e le 'ngoe kapa ho feta tse latelang:

• Leqhoa la mali
• Ho senyeheloa hangata

Liteko tse ntle li hloka ho netefatsoa kamora libeke tse 12. Haeba u na le tlhahlobo e ntle ntle le likarolo tse ling tsa lefu lena, u ke ke ua fumana lefu la APS.

Kalafo ea APS e reretsoe ho thibela mathata a tsoang maling a macha a bopang kapa a mang a teng hore a hole. U tla hloka mofuta o itseng oa moriana o fokotsang mali. Haeba le uena u na le lefu le ikemetseng, joalo ka lupus, u tla hloka ho boloka boemo boo le bona bo laoloa.

Kalafo e nepahetseng e tla itšetleha ka hore na boemo ba hau bo matla hakae le mathata ao e a bakang.

ANTIPHOSPHOLIPID ANTIBODY SYNDROME (APS)

Ka kakaretso, o tla hloka kalafo ka tšesaane ea mali nako e telele haeba o na le APS. Kalafo ea pele e ka ba heparin. Meriana ena e fanoa ka ente.

Maemong a mangata, warfarin (Coumadin), e fanoang ka molomo, e ea qalisoa. Ho hlokahala hore hangata ho hlokomela boemo anticoagulation. Hangata sena se etsoa ka tlhahlobo ea INR.

Haeba u na le APS 'me u ima, u tla hloka ho lateloa haufi-ufi ke mofani ea nang le tsebo ea boemo bona. U ke ke ua nka warfarin nakong ea bokhachane, empa ho fapana le moo u tla fuoa li-heparin shots.

Haeba u na le SLE le APS, mofani oa hau oa marang-rang o tla u khothaletsa hore u noe hydroxychloroquine.

Hajoale, mefuta e meng ea meriana e fokotsang mali ha e khothaletsoe.

CATASTROPHIC ANTIPHOSPHOLIPID SYNDROME (CAPS) LITLHAKU TSE KHOLO

Kalafo ea CAPS e kenyelletsang motsoako oa kalafo ea li-anticoagulation, tekanyetso e phahameng ea li-corticosteroids, le phapanyetsano ea plasma e atlehile ho batho ba bangata. Ka linako tse ling IVIG, rituximab kapa eculizumab e boetse e sebelisetsoa linyeoe tse matla.

Teko e ntle ea LUPUS ANTICOAGULANT KAPA APL

Ha o na ho hloka kalafo haeba o sena matšoao, ho senyeheloa ke bokhachane, kapa ha o e-so ka o ba le leqhubu la mali.

Nka mehato e latelang ho thusa ho thibela mali ho hlaha:

• Qoba lipilisi tse ngata tsa thibelo ea bokhachane kapa kalafo ea li-hormone bakeng sa ho ilela khoeli (basali).
• U SE KE UA tsuba kapa ua sebelisa lihlahisoa tse ling tsa koae.
• Ema 'me u tsamaee nakong ea lifofane tse telele kapa ka linako tse ling ha u tlameha ho lula kapa ho paqama nako e telele.
• Tsamaisa maqaqailana hodimo le tlase ha o sa kgone ho potoloha.

U tla fuoa meriana e fokotsang mali (joalo ka heparin le warfarin) ho thusa ho thibela ho koaloa ha mali:

• Ka mor'a ho buuoa
• Kamora ho robeha ha masapo
• Ka mofetše o mafolofolo
• Ha o hloka ho lula kapa ho robala nako e telele, joalo ka nakong ea ho lula sepetlele kapa ho hlaphoheloa hae

U kanna ua hloka ho noa mali a tšesaane bakeng sa libeke tse 3 ho isa ho tse 4 kamora ho etsoa opereishene ho theola menyetla ea ho hoama ha mali.

Ntle le kalafo, batho ba nang le APS ba tla pheta ho hoama. Boholo ba nako, sephetho se setle ka kalafo e nepahetseng, e kenyeletsang kalafo ea nako e telele ea anticoagulation. Batho ba bang ba ka ba le maqeba a mali ao ho leng thata ho a laola ho sa tsotelehe kalafo. Sena se ka lebisa ho CAPS, e ka bang kotsing ea bophelo.

Bitsa mofani oa hau haeba u bona matšoao a mali, joalo ka:

• Ho ruruha kapa bokgubedu maotong
• Phefumoloho e khutšoanyane
• Bohloko, ho ba shohlo, 'mala oa letlalo le phatsima letsohong kapa leotong

Hape bua le mofani oa hau haeba u pheta ho lahleheloa ke bokhachane khafetsa (ho senyeheloa ke mpa).

Lithibela-mafu Anticardiolipin; Hughes lefu

• Sesosa sa lupus erythematosus sefahlehong
• Ho koala mali

Amigo MC, Khamashta MA. Antiphospholipid syndrome: pathogenesis, tlhahlobo ea mafu le tsamaiso. Ho: Hochberg MC, Gravallese EM, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, bahlophisi. Rheumatology. La 7th. Philadelphia, PA: Elsevier; 2019: khaolo ea 148.

Cervera R, Rodríguez-Pintó I, Colafrancesco S, le al. 14th International Congress mabapi le Antiphospholipid Antibodies Task Force e tlaleha ka koluoa ​​ea antiphospholipid syndrome. Iketsetse Auto. 2014; 13 (7): 699-707. PMID: 24657970 www.ncbi.nlm.nih.gov/pubmed/24657970.

Dufrost V, Risse J, Wahl D, Zuily S. Direct anticoagulants ea molomo e sebelisoa ho lefu la antiphospholipid: na meriana ee ke mokhoa o sebetsang le o bolokehileng oa warfarin? Tlhahlobo e hlophisitsoeng ea lingoliloeng: karabelo ho maikutlo. Cur ea Rheumatol Rep. 2017; 19 (8): 52. PMID: 28741234 www.ncbi.nlm.nih.gov/pubmed/28741234.

Erkan D, Salmone JE, Lockshin MD. Lefu la anti-phospholipid. Ka: Firestein GS, Budd RC, Gabriel SE, McInnes IB, O'Dell JR, bahlophisi. Kelley le Firestein's Bookbook ea Rheumatology. 10th ed. Philadelphia, PA: Elsevier; 2017: khaolo ea 82.

Webosaete ea National Heart, Lung le Blood Institute. Lefu la Antiphospholipid antibody. www.nhlbi.nih.gov/health-topics/antiphospholipid-antibody-syndrome. E fihlile ka la 5 Phuptjane, 2019.