Dhiig-yaraantu, qeexitaan ahaan, waxay ku lug leedahay hoos u dhaca unugyada dhiigga cas, ama heerarka hemoglobin. Erayga "hemolytic anemia" wuxuu ka kooban yahay noocyo kala duwan oo dhiig-yaraan ah halkaasoo unugyada dhiigga cas ay si degdeg ah ugu burburaan dhiigga. Erayga "hemolysis" macneheedu waa burburinta unugyada dhiigga cas (hemo = dhiig; lysis = burburin).

Dhuuxa lafta ayaa leh awood kayd ah oo gaar ah. Taasi waa, waxay kordhin kartaa wax soo saarkeeda unugyada dhiigga cas ilaa heer gaar ah si ay u magdhowdo burburkooda kordhay. Sida caadiga ah, unugyada dhiigga cas waxay ku wareegaan xididdada dhiigga ilaa 120 maalmood. Dhamaadka noloshooda, waxaa baabi'iya beeryarada iyo beerka (sidoo kale eeg xaashida Anemia- dulmarka). Burburinta degdega ah ee unugyada dhiigga cas waa kicinta muhiimka ah ee soo saarista unugyada dhiigga cas ee cusub, kaas oo ay dhexdhexaadiyaan hormoon ay soo saaraan kelyaha, erythropoietin (EPO). Xaaladaha qaarkood, dhuuxa lafta ayaa awood u leh inay soo saaraan inta ugu badan ee unugyada dhiigga cas ee caddadka si aan caadi ahayn u burburay, markaa heerka hemoglobin hoos uma dhaco. Waxaan ka hadleynaa hemolysis magdhow ah, oo aan lahayn dhiig-yaraan. Tani waa muhiim sababtoo ah waxaa jira arrimo gaar ah oo keeni kara in xaaladdu ay u kala jajabto arrimo faragelin doona wax soo saarka EPO sida uurka, kelyaha oo shaqeyn, yaraanta folic acid, ama caabuq degdeg ah.

Sababaha

Hemolytic anemia guud ahaan waxaa loo kala saaraa iyadoo loo eegayo haddii ay keento unug cas cas oo laftiisa aan caadi ahayn (intracorpuscular), ama arrin ka baxsan unugyada dhiigga cas (extracorpuscular). Waxa kale oo la kala soocay kuwa la iska dhaxlo iyo kuwa hemolytic anemia.

Sababaha dhaxalka ah iyo kuwa gudaha ku jira

• Hemoglobinopathies (sida sickle cell anemia, iwm.)
• Enzymopathies (tusaale G6-PD yaraan)
• Xubinta iyo cytoskeletal aan caadi ahayn (tusaale spherocytosis lagu dhasho)

Sababta dhaxalka ah iyo kuwa ka baxsan jidhka

• Qoyska hemolytic-uremic syndrome (mid caadi ah)

Sababta la helay iyo intrakursicular

• Paroxysmal habeennimo hemoglobinuria

Sababta la helay iyo kuwa ka baxsan jidhka

• Burburinta makaanikada (microangiopathy)
• Wakiilada sunta ah
• Daawooyinka
• Infections
• Immunological

Aynu haddaba ka hadalno dhawr tusaale, mar haddii aanay suurtogal ahayn in dhammaantood lagu sifeeyo nuxurka qoraalkan.

Dhiig-yaraanta difaaca jirka ee hemolytic:

Dareen-celinta difaaca jirka. Xaaladdan oo kale, jidhku, sababo kala duwan, wuxuu soo saaraa unugyada difaaca jirka ee ka soo horjeeda unugyada dhiigga cas: kuwan waxaa loo yaqaannaa autoantibodies. Waxaa jira laba nooc: kuwa leh autoantibodies kulul iyo kuwa qaba autoantibodies qabow, iyadoo ku xiran haddii heerkulka ugu fiican ee dhaqdhaqaaqa antibody uu yahay 37 ° C ama 4 ° C. Farqigaani waa muhiim sababtoo ah daaweyntu way ku kala duwan tahay qaab ilaa qaab.

– Autoantibodies kulul: badiyaa waxay saameeyaan dadka qaangaarka ah waxayna sababaan dhiig-yaraan joogto ah oo marmarka qaarkood aad u daran. Waxay ka dhigan yihiin 80% dhiig-yaraanta hemolytic-ka ee difaaca jirka. Kiisaska kala badh ka mid ah, waxaa kicin kara daawooyinka qaarkood (alpha-methyldopa, L-dopa) ama cudurrada qaarkood (burnada ugxan-sidaha, lymfoproliferative syndrome, iwm.). Tan waxaa loo tixraacaa sida "labaad" autoimmune hemolytic anemias, maadaama ay u muuqdaan natiijada cudur kale.

- Unugyada dhiigga cas ee qabow ee ka-hortagga jirka: waxay la xiriiraan marxalado ba'an oo burburinaya unugyada dhiigga cas ee uu keeno hargabku. 30% kiisaska, waxaan la tacaaleynaa falcelinta difaaca jirka ee labaad taasoo lagu macnayn karo infekshanka fayraska ama mycoplasma, microorganism dhexdhexaad ah oo u dhexeeya fayraska iyo bakteeriyada.

Dareen-celinta difaaca jirka. Haddii ay dhacdo hemolysis (non-autoimmune) daawada hemolysis, unugyada difaacayaashu ma weeraraan unugyada dhiigga cas, laakiin daawooyinka qaarkood: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, iwm.

Dhiig-yaraanta hemolytic ee lagu dhasho:

Waxaa jira saddex qaybood oo muhiim ah oo ku jira unugyada dhiigga cas. Waxaa jira hemoglobin, kakanaanta xuubka-cytoskeleton, iyo "mashiinada" enzymatic si ay u wada shaqeeyaan. Isbeddellada hidde-sideyaasha mid ka mid ah saddexdan arrimood waxay sababi karaan dhiig-yaraan hemolytic.

Waxyaalaha aan caadiga ahayn ee la iska dhaxlo ee xuubka unugyada dhiigga cas. Midda ugu weyn waa spherocytosis lagu dhasho, oo loogu magac daray sababtoo ah qaabka koofiyada oo markaas tilmaamaya unugyada dhiigga cas oo ka dhigaya kuwo si gaar ah u jilicsan. Waa mid soo noqnoqda: 1 kiis sannadkii 5000. Dhowr cillado hidde-side ah ayaa ku lug leh, qaabka caadiga ah waa autosomal Dominate, laakiin foomamka dib-u-celinta ayaa sidoo kale jira. Waxay keeni kartaa dhibaatooyin gaar ah: dhagxaanta xameetida, boogaha lugaha.

Enzymopathies. Waxaa jira dhowr nooc oo yaraanta ensaymka oo keeni karta dhiig-yarida hemolytic. Guud ahaan waa dhaxal. Midda ugu badan waa yaraanta enzyme loo yaqaan "glucose-6-phosphate dehydrogenase", kaas oo sababa burburin degdeg ah unugyada dhiigga cas iyo, ka dib, dhiig-yaraanta hemolytic.

Ciladda hidde-sidaha ee ku lug leh waxay ku xiran tahay koromosoomka X, sidaas darteed, ragga kaliya ayaa saameyn kara. Dumarku waxay qaadi karaan cilladda hidde-sidaha waxayna u gudbin karaan carruurtooda. Dadka qaba yaraanta ensaymes-ka, dhiig-yaraanta hemolytic waxay inta badan ku dhacdaa ka dib soo-gaadhista walxaha oksaydhka.

Dadka qaba yaraanta G6PD waxay yeelan karaan hemolysis ba'an marka ay la kulmaan wakiilada qaarkood sida:

- isticmaalka noocyada kala duwan ee digirta loo yaqaan digirta yar-yar (faba la qabatimo) ama soo-gaadhista manka kasoo baxa geedkaas (Digirtan kala duwan waxa loo isticmaalaa quudinta xoolaha). Xidhiidhkani waxa uu keenaa dhiig-yaraan ba'an oo hemolytic ah oo loo yaqaan favism.

- isticmaalka dawooyinka qaarkood: antimalarials, methyldopa (cadaadiska dhiigga hoos u dhigta), sulfonamides (antibacterial), aspirin, daawooyinka aan steroid ahayn ee anti-bararka, quinidine, quinine, iwm.

- soo-gaadhista kiimikooyinka qaarkood sida baalallayda.

- caabuqyada qaarkood.

Cudurkan waxaa inta badan lagu ogaadaa dadka ka yimid badda Mediterranean-ka (gaar ahaan jasiiradaha Giriiga) iyo sidoo kale dadka madow ee Afrika iyo Maraykanka (halkaas oo faafitaankiisu yahay 10% ilaa 14%). Meelo ka mid ah adduunka, 20% ama in ka badan oo dadweynaha ah ayaa qaba.

Hemoglobinopathies. Erayga loo isticmaalo in lagu qeexo cudurrada hidda-socodka ee ay saamayso soo saarista haemoglobin ee gudaha unugyada dhiigga cas. Sickle cell anemia (sickle cell anemia) iyo thalassaemia waa labada qaybood ee ugu waaweyn ee hemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Cudurkan halista ah waxa uu ku xidhan yahay joogitaanka haemoglobin aan caadi ahayn oo loo yaqaan hemoglobin S. Tani waxay qalloocinaysaa unugyada dhiigga cas waxayna siinaysaa qaabka bilaha ama matag (unugyada sickle), oo ay u dheer tahay inay u dhintaan. dhicis. Fiiri xaashida Sickle cell anemia.

Thalassaemia. Aad ugu baahay waddamo ka mid ah aduunka, cudurkan halista ah waxa uu ku xidhan yahay cillad hidde-sideyaal ah oo saamaysa soo saarista haemoglobin, midabkan dhiiga ee ku jira unugyada cas cas, taas oo u ogolaanaysa in ogsajiinta loo qaado xubnaha. Unugyada dhiigga cas ee ay saameeyeen waa jilicsan yihiin oo si degdeg ah u jajaban yihiin. Ereyga "thalassaemia" wuxuu ka yimid ereyga Giriigga "thalassa", oo macneheedu yahay "bad", sida markii ugu horreysay lagu arkay dadka ka soo jeeda badda Mediterranean. Ciladda hidde-sidaha waxay saameyn kartaa laba meelood oo ka mid ah isku-darka haemoglobin: silsiladda alfa ama silsiladda beta. Iyadoo ku xiran nooca silsiladda ay saameysay, waxaa jira laba nooc oo thalassaemia ah: alfa-thalassaemia iyo beta-thalassaemia.

Sababaha kale

Sababaha makaanikada. Unugyada dhiigga cas waa ay dhaawacmi karaan inta lagu jiro daawaynta qaarkood ee la xidhiidha qalabka farsamada:

- qalabyada (qalabka macmalka ah ee wadnaha, iwm.);

- sifaynta dhiigga ka baxsan jidhka (hemodialysis);

- mishiinka si oksijiinta dhiigga loogu shubo (oo loo isticmaalo qalliinka wadnaha-sanbabada), iwm.

Marar dhif ah, orodyahanka marathon-ka ayaa laga yaabaa inuu la kulmo dhiigbaxa farsamada maadaama xididada cagaha ay si isdaba joog ah u jajabaan. Xaaladdan ayaa sidoo kale lagu sifeeyay ka dib qaar ka mid ah qoob-ka-ciyaar dhaqameed oo aad u daba dheeraaday, oo cagaha qaawan.

Soo-gaadhista walxaha sunta ah.

- Alaabooyinka sunta warshadaha ama kuwa gudaha: aniline, hydrogen arsenic, nitrobenzene, naphthalene, paradichlorobenzene, iwm.

– Xayawaanka sunta ah: qaniinyada caarada, qaniinyada wasakhda, sunta abeesada.

– Geedo sun ah: fangaska qaarkood.

Infekshannada.Gastroenteritis daran oo uu keeno Iyo coli, caabuqyada ay keento pneumococcus ama staphylococcus, cagaarshow, qandhada tiifowga, duumada, iwm. Duumada (ama malaariyada) ayaa ah sababta ugu muhiimsan ee qaybtan. Duumada waxaa sababa dulin ka soo baxa gudaha unugyada dhiigga cas.

Hawlkarnimada beeryarada. Waa wax iska caadi ah in unugyada cas cas ay ku burburaan beeryarada ka dib safarkooda 120-ka maalmood ah, laakiin haddii xubintani ay si xad dhaaf ah u shaqeyso, burburku aad buu u degdegayaa waxaana ka dhasha dhiig-yarida hemolytic.

Hhemoglobinuria paroxysmal habeennimo. Cudurkan dabadheeraadku wuxuu la xidhiidha joogitaanka haemoglobin ee kaadida sababtoo ah burburka xad dhaafka ah ee unugyada dhiigga cas. Suuxdinaha habeenkii waxaa keena nooc kasta oo walbahaar, kicinta habka difaaca jirka ama daawooyinka qaarkood. Mararka qaarkood cudurku wuxuu keenaa xanuunka dhabarka hoose iyo raaxo la'aan.

Dhibaatooyinka suurtagalka ah: thrombosis, hypoplasia dhuuxa lafta, caabuqyada labaad.

• Kuwa ku xidhan heerka hooseeya ee unugyada dhiigga cas: midabka midabka, daal, daciifnimo, dawakhaad, garaaca wadnaha oo degdeg ah, iwm.
• Jaundice.
• Kaadi madow.
• Balaadhinta beeryarada.
• Kuwa u gaarka ah nooc kasta oo ah dhiig-yarida hemolytic. Fiiri "Sharaxaad Caafimaad".

Qaababka lagu dhasho ee hemolytic anemia:

• Kuwa leh taariikh qoys.
• Dadka ka yimid badda Mediterranean-ka, Afrika, Koonfur iyo Koonfur-bari Aasiya iyo Galbeedka Hindiya.

• Dadka qaba yaraanta enzyme gulukoos-6-fosfate dehydrogenase: soo-gaadhista walxaha oxidizing (daawooyinka qaarkood, digirta beerta, iwm.).
• Noocyada kale ee dhiig-yaraanta hemolytic:

  - Cudurada qaarkood: cagaarshow, caabuq streptococcal ama E. coli, xanuunada difaaca jirka (sida lupus), burada ugxansidaha.

  - Daawooyinka qaarkood (antimalarials, penicillin, rifampicin, sulfonamides, iwm) ama walxaha sunta ah (aniline, hydrogen arsenic, iwm.).

  - Aaladaha farsamada ee loo isticmaalo daawadda: alwaaxyada macmalka ah, qalabka lagu sifeeyo ama ogsijiinta dhiiga.

  - Cadaadiska

• Waqtigan xaadirka ah, suurtagal maaha in laga hortago qaababka dhaxalka ah marka laga reebo la-talinta la-taliyaha hidaha ka hor intaanay uur qaadin ilmaha. Khabiirku waxa uu awood u yeelan doonaa in uu go'aamiyo khatarta uu leeyahay ilmo qaba dhiig-yaraan dhiig-yaraan ah marka midkood (ama labadaba) waalidka suurtagalka ah ay leeyihiin taariikh qoys (sidoo kale Sickle Cell Anemia si aad u hesho faahfaahin dheeraad ah oo ku saabsan khatarta hiddesideyaasha ee la xiriirta foomkan dhiig-yaraanta hemolytic).
• Haddii walax gaar ah ay mas'uul ka tahay cudurka, waa in laga fogaado si looga hortago soo noqoshada.
• Noocyo badan oo dhiig-yaraan ah oo hemolytic ah, sidoo kale waa muhiim in laga ilaaliyo caabuqyada qaarkood.

Way kala duwan yihiin iyadoo ku xiran nooca dhiig-yaraanta hemolytic.

• Daawaynta ayaa marka hore ku salaysan taageerada guud ee jidhka iyo sababta salka u ah marka ay suurtogal tahay
• Kaabista folic acid ayaa guud ahaan loo tilmaamay bukaanada qaba dhiig-yarida hemolytic ee raaga.
• Tallaalka ka hortagga caabuqyada caadiga ah ayaa muhiim u ah bukaannada difaaca difaaca daciifka ah, gaar ahaan dadka qaba splenectomies (saarista beeryarada).⁶)
• Dhiig ku shubista ayaa mararka qaarkood la tilmaamaa
• Splenectomy ayaa mararka qaarkood la soo jeediyaa⁷, gaar ahaan dadka qaba spherocytosis dhaxalka ah, thalassaemias oo inta badan u baahan in lagu shubo laakiin sidoo kale mararka qaarkood noocyada kale ee dhiig-yaraanta hemolytic. Runtii, waxay inta badan ku jirtaa beeryarada in unugyada dhiigga cas ay burburaan.
• Cortisone waxaa mararka qaarkood loo qoraa antibody-ga kulul ee autoimmune anemia iyo in laga fiirsado qabow ka hortagga dhiig-yaraanta. Waxaa mararka qaarkood loo isticmaalaa xaaladaha haemoglobinuria ee paroxysmal nocturnal iyo gaar ahaan thrombocytopenic purpura. Daawooyinka difaaca jirka ee xooggan, sida rituximab⁸, Immunoglobulins xididada, azathioprine, cyclophosphamide, iyo cyclosporine ayaa laga yaabaa in loo tixgeliyo in immunologic hemolytic anemias. Plasmapheresis ayaa mararka qaarkood loo isticmaalaa, gaar ahaan kiiska thrombocytopenic purpura.

Iyada oo qayb ka ah qaabkeeda tayada leh, Passeportsanté.net waxay kugu martiqaadeysaa inaad ogaato fikradda xirfadle caafimaad. Dr Dominic Larose, oo ah dhakhtar degdeg ah, ayaa ku siinaya ra'yigiisa dhiig-yaraan hemolytic :

Daawaynta kaliya ee aan caadiga ahayn ayaa lagu aqoonsaday inay khuseeyaan sickle cell anemia. Faahfaahin dheeraad ah ka eeg xaashidan.