Anemia, raws li kev txhais, suav nrog kev txo qis hauv cov qe ntshav liab, lossis qib hemoglobin. Lo lus "hemolytic anemia" suav nrog ntau hom kab mob ntshav qab zib uas cov qe ntshav liab raug rhuav tshem ntxov hauv cov ntshav. Lo lus "hemolysis" txhais tau tias kev puas tsuaj ntawm cov qe ntshav liab (hemo = ntshav; lysis = kev puas tsuaj).

Cov pob txha pob txha muaj peev xwm cia. Ntawd yog, nws tuaj yeem ua rau nws cov qe ntshav liab nce mus rau ib theem los them rau lawv qhov kev puas tsuaj ntau ntxiv. Feem ntau, cov qe ntshav liab ncig hauv cov hlab ntsha li 120 hnub. Thaum kawg ntawm lawv lub neej, lawv raug rhuav tshem los ntawm tus po thiab lub siab (saib daim ntawv Anemia - txheej txheem cej luam). Kev puas tsuaj sai ntawm cov qe ntshav liab yog qhov tseem ceeb hauv kev tsim cov qe ntshav liab tshiab, uas yog kho los ntawm cov tshuaj hormones tsim los ntawm ob lub raum, erythropoietin (EPO). Qee zaum, cov pob txha pob txha muaj peev xwm tsim tau ntau cov qe ntshav liab raws li tus nqi uas txawv txav, yog li qib hemoglobin tsis poob. Peb tab tom tham txog kev them nyiaj hemolysis, tsis muaj ntshav qab zib. Qhov no yog qhov tseem ceeb vim tias muaj qee yam uas tuaj yeem ua rau qhov xwm txheej tsis zoo rau hauv cov khoom uas yuav cuam tshuam rau EPO ntau lawm xws li cev xeeb tub, raum tsis ua haujlwm, folic acid tsis txaus, lossis mob hnyav.

Ua rau

Hemolytic anemia feem ntau yog cais raws li seb nws yog tshwm sim los ntawm cov qe ntshav liab uas nws tus kheej txawv txav (intracorpuscular), los yog ib qho tseem ceeb uas yog sab nraud rau cov qe ntshav liab (extracorpuscular). Ib qho kev sib txawv kuj yog ua los ntawm kev sib txawv ntawm cov keeb kwm thiab tau txais hemolytic anemia.

Cov kab mob thiab intracorpuscular ua rau

• Hemoglobinopathies (xws li mob qog nqaij hlav anemia, thiab lwm yam)
• Enzymopathies (xws li G6-PD deficiency)
• Membrane thiab cytoskeletal abnormalities (xws li congenital spherocytosis)

Hereditary thiab extracorpuscular ua rau

• Tsev neeg hemolytic-uremic syndrome (atypical)

Tau thiab intracorpuscular ua rau

• Paroxysmal nocturnal hemoglobinuria

Tau thiab extracorpuscular ua rau

• Mechanical rhuav tshem (microangiopathy)
• Cov tshuaj lom neeg
• pharmaceuticals
• Cov kab mob
• Kev Siv Tshuaj Tiv Thaiv

Cia peb tham txog ob peb yam piv txwv tom qab ntawd, vim nws tsis tuaj yeem piav qhia lawv txhua tus hauv cov ntsiab lus ntawm daim ntawv no.

Immunological hemolytic anemias:

Cov tshuaj tiv thaiv autoimmune. Hauv qhov no, lub cev, rau ntau yam laj thawj, tsim cov tshuaj tiv thaiv tiv thaiv nws tus kheej cov qe ntshav liab: cov no hu ua autoantibodies. Muaj ob hom: cov neeg uas muaj autoantibodies kub thiab cov autoantibodies txias, nyob ntawm seb qhov zoo tshaj plaws kub rau cov tshuaj tiv thaiv kab mob yog 37 ° C los yog 4 ° C. Qhov kev sib txawv no yog qhov tseem ceeb vim tias kev kho mob txawv ntawm daim ntawv.

- Kub autoantibodies: feem ntau cuam tshuam rau cov neeg laus thiab ua rau mob ntev thiab qee zaum hnyav hemolytic anemia. Lawv sawv cev rau 80% ntawm autoimmune hemolytic anemias. Hauv ib nrab ntawm cov neeg mob, lawv tuaj yeem tshwm sim los ntawm qee yam tshuaj (alpha-methyldopa, L-dopa) lossis qee yam kab mob (cov qog ntawm zes qe menyuam, lymphoproliferative syndrome, thiab lwm yam). Qhov no yog hu ua "secondary" autoimmune hemolytic anemias, vim lawv tshwm sim los ntawm lwm yam kab mob.

- Cold auto-antibodies: cuam tshuam nrog kev mob hnyav ntawm kev puas tsuaj ntawm cov qe ntshav liab los ntawm kev mob khaub thuas. Hauv 30% ntawm cov neeg mob, peb tab tom cuam tshuam nrog cov tshuaj tiv thaiv kab mob autoimmune thib ob uas tuaj yeem piav qhia los ntawm tus kab mob kis los yog mycoplasma, cov kab mob nruab nrab ntawm cov kab mob thiab cov kab mob.

Cov tshuaj tiv thaiv immunoallergic. Nyob rau hauv cov ntaub ntawv ntawm immunoallergic (tsis yog-autoimmune) tshuaj hemolysis, cov tshuaj tiv thaiv tsis tua cov qe ntshav liab, tab sis qee yam tshuaj: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, thiab lwm yam.

Congenital hemolytic anemias:

Muaj peb yam tseem ceeb hauv cov qe ntshav liab. Muaj hemoglobin, lub membrane-cytoskeleton complex, thiab enzymatic "tshuab" ua kom nws tag nrho cov hauj lwm. Cov caj ces txawv txav ntawm ib qho ntawm peb yam no tuaj yeem ua rau hemolytic anemia.

Hereditary abnormalities ntawm daim nyias nyias ntawm cov ntshav liab. Lub ntsiab yog congenital spherocytosis, yog li lub npe vim hais tias ntawm spherical zoo uas ces characterizes cov qe ntshav liab thiab uas ua rau lawv tsis yooj yim. Nws yog qhov nquag: 1 rooj plaub hauv 5000. Muaj ntau yam kev txawv txav ntawm caj ces muaj feem cuam tshuam, daim ntawv classic yog autosomal dominate, tab sis cov ntaub ntawv recessive kuj muaj. Nws tuaj yeem ua rau qee yam teeb meem: gallstones, rwj ntawm ob txhais ceg.

Enzymopathies. Muaj ntau hom enzyme deficiency uas tuaj yeem ua rau hemolytic anemia. Lawv feem ntau yog los ntawm noob neej. Feem ntau yog qhov tsis txaus ntawm cov enzyme hu ua "glucose-6-phosphate dehydrogenase", uas ua rau kev puas tsuaj ntxov ntxov ntawm cov qe ntshav liab thiab, tom qab, hemolytic anemia.

Cov kab mob caj ces uas cuam tshuam yog txuas nrog X chromosome, yog li ntawd, tsuas yog txiv neej tuaj yeem cuam tshuam. Cov poj niam tuaj yeem nqa cov kab mob caj ces thiab kis mus rau lawv cov menyuam. Hauv cov neeg uas muaj qhov tsis txaus enzyme no, hemolytic anemia feem ntau tshwm sim tom qab raug oxidizing tus neeg sawv cev.

Cov neeg uas tsis muaj G6PD tuaj yeem tsim mob hemolysis thaum raug rau qee tus neeg ua haujlwm xws li:

- noj ntau yam ntawm taum hu ua me-grain taum (quav fab) los yog raug paj ntoos los ntawm cov nroj tsuag (qhov no ntau yam ntawm taum yog siv rau tsiaj txhu noj). Qhov kev sib cuag no ua rau mob hemolytic anemia tseem hu ua favism.

- kev siv qee yam tshuaj: tshuaj tiv thaiv kab mob, methyldopa (tso ntshav siab), sulfonamides (antibacterials), tshuaj aspirin, tshuaj tsis-steroidal anti-inflammatory, quinidine, quinine, thiab lwm yam.

– raug rau tej yam tshuaj xws li mothballs.

– tej yam kab mob.

Tus kab mob no feem ntau kuaj pom hauv cov neeg los ntawm Mediterranean Basin (tshwj xeeb yog Greek Islands) nrog rau cov neeg dub hauv Africa thiab Tebchaws Meskas (qhov twg nws qhov muaj feem ntau yog 10% txog 14%). Hauv qee qhov chaw hauv ntiaj teb, 20% lossis ntau dua ntawm cov pejxeem muaj nws.

Hemoglobinopathies. Lo lus siv los piav txog cov kab mob caj ces uas tsim cov hemoglobin hauv cov qe ntshav liab cuam tshuam. Sickle cell anemia (sickle cell anemia) thiab thalassemia yog ob yam tseem ceeb ntawm hemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Cov kab mob loj no txuas nrog rau qhov muaj qhov txawv txav ntawm hemoglobin hu ua hemoglobin S. Qhov no ua rau cov qe ntshav liab distors thiab ua rau lawv zoo li lub crescent lossis scythe (sickle cells), ntxiv rau ua rau lawv tuag. ntxov ntxov. Saib daim ntawv Sickle cell anemia.

Thalassemia. Kev nthuav dav heev hauv qee lub tebchaws hauv ntiaj teb, tus kab mob loj no tau txuas nrog cov caj ces txawv txav uas cuam tshuam rau kev tsim cov hemoglobin, cov ntshav pigment hauv cov qe ntshav liab uas tso cai rau kev thauj cov pa oxygen mus rau hauv nruab nrog cev. Cov qe ntshav liab cuam tshuam tsis yooj yim thiab tawg sai sai. Lo lus "thalassemia" los ntawm Greek lo lus "thalassa", uas txhais tau tias "hiav txwv", raws li nws tau xub pom nyob rau hauv cov neeg los ntawm lub Mediterranean phiab. Cov kab mob caj ces tuaj yeem cuam tshuam rau ob qhov chaw hauv kev sib txuas ntawm hemoglobin: alpha saw lossis beta saw. Nyob ntawm seb hom saw cuam tshuam, muaj ob hom thalassemia: alpha-thalassemia thiab beta-thalassemia.

Lwm yam ua

Mechanical ua rau. Cov qe ntshav liab tuaj yeem raug puas ntsoog thaum muaj qee yam kev kho mob ntsig txog cov cuab yeej siv tshuab:

- prostheses (artificial li qub rau lub plawv, thiab lwm yam);

- cov ntshav purification (hemodialysis);

- tshuab kom oxygenate cov ntshav (siv hauv kev phais plawv- ntsws), thiab lwm yam.

Tsis tshua muaj, tus neeg khiav dej num marathon tuaj yeem ntsib cov neeg kho tshuab hemolysis vim tias cov hlab ntsha hauv ko taw tau rov ua dua. Qhov xwm txheej no kuj tau piav qhia tom qab qee qhov kev seev cev ntev heev, ntawm ko taw liab qab.

Raug rau cov ntsiab lus lom.

- Cov khoom lag luam lossis cov tshuaj lom neeg hauv tsev: aniline, arsenic hydrogen, nitrobenzene, naphthalene, paradichlorobenzene, thiab lwm yam.

- Tsiaj txhu: kab laug sab tom, nkuaj, nab venom.

- Cog tshuaj lom: tej yam fungi.

Kab mob.Mob plab gastroenteritis tshwm sim los ntawm Thiab coli, kab mob tshwm sim los ntawm pneumococcus los yog staphylococcus, kab mob siab, typhoid fever, malaria, thiab lwm yam. Malaria (los yog malaria) yog qhov tseem ceeb tshaj plaws ua rau hauv pawg no. Malaria yog tshwm sim los ntawm cov kab mob cab uas loj hlob hauv cov qe ntshav liab.

Hyperfunction ntawm tus po. Nws yog ib txwm ua rau cov qe ntshav liab raug rhuav tshem hauv tus po tom qab lawv txoj kev taug kev 120-hnub, tab sis yog tias lub cev no ua haujlwm ntau dhau, kev puas tsuaj sai dhau thiab hemolytic anemia tshwm sim.

Hntshav qab zib paroxysmal nocturnal. Cov kab mob ntev no cuam tshuam nrog kev muaj hemoglobin hauv cov zis vim kev puas tsuaj ntau ntawm cov qe ntshav liab. Kev qaug dab peg hmo ntuj yog tshwm sim los ntawm txhua yam kev ntxhov siab, kev tiv thaiv kab mob los yog qee yam tshuaj. Qee zaum tus kab mob ua rau mob nraub qaum thiab tsis xis nyob.

Cov teeb meem tshwm sim: thrombosis, pob txha pob txha hypoplasia, kab mob thib ob.

• Cov uas txuas rau cov qe ntshav liab qis: daj ntseg, qaug zog, qaug zog, kiv taub hau, lub plawv dhia ceev, thiab lwm yam.
• Qhov muag daj.
• Tsaus zis.
• Kev loj hlob ntawm tus po.
• Cov uas tshwj xeeb rau txhua hom hemolytic anemia. Saib “Kev piav qhia kho mob”.

Rau congenital daim ntawv ntawm hemolytic anemia:

• Cov uas muaj keeb kwm ntawm tsev neeg.
• Cov neeg los ntawm Mediterranean phiab, Africa, South thiab Southeast Asia thiab West Indies.

• Hauv cov neeg uas muaj qhov tsis txaus ntawm enzyme glucose-6-phosphate dehydrogenase: raug oxidizing cov neeg ua haujlwm (qee yam tshuaj, taum teb, thiab lwm yam).
• Rau lwm hom hemolytic anemia:

  - Qee yam kab mob: kab mob siab, kab mob streptococcal lossis E. coli, kab mob autoimmune (xws li lupus), qog ntawm zes qe menyuam.

  - Qee yam tshuaj (antimalarials, penicillin, rifampicin, sulfonamides, thiab lwm yam) lossis tshuaj lom (aniline, arsenic hydrogen, thiab lwm yam).

  - Tej yam khoom siv kho tshuab siv hauv cov tshuaj: cov khoom siv li qub, cov cuab yeej siv rau kev ua kom huv lossis oxygenating cov ntshav.

  — Kev nyuaj siab.

• Tam sim no, nws tsis tuaj yeem tiv thaiv cov ntaub ntawv keeb kwm tshwj tsis yog los ntawm kev sab laj tus kws pab tswv yim caj ces ua ntej xeeb menyuam. Tus kws kho mob tshwj xeeb yuav tuaj yeem txiav txim siab txog kev pheej hmoo ntawm kev yug me nyuam nrog hemolytic anemia thaum twg (lossis ob leeg) ntawm cov niam txiv muaj peev xwm muaj keeb kwm hauv tsev neeg (saib Sickle Cell Anemia kom paub meej ntxiv txog cov kev pheej hmoo ntawm caj ces hais txog daim ntawv no. ntawm hemolytic anemia).
• Yog tias ib yam khoom tshwj xeeb muaj lub luag haujlwm rau tus kab mob, nws yuav tsum tau zam kom tsis txhob rov tshwm sim dua.
• Rau ntau hom hemolytic anemia, nws tseem ceeb heev kom tiv thaiv qee yam kab mob.

Lawv txawv nyob ntawm seb hom hemolytic anemia.

• Kev kho mob yog thawj zaug thiab qhov tseem ceeb raws li kev txhawb nqa dav dav rau lub cev thiab lub hauv paus ua rau thaum ua tau
• Ib qho ntxiv ntawm folic acid feem ntau qhia rau cov neeg mob uas mob hemolytic anemia.
• Kev txhaj tshuaj tiv thaiv kab mob sib kis yog qhov tseem ceeb rau cov neeg mob uas tsis muaj kev tiv thaiv kab mob, tshwj xeeb tshaj yog rau cov neeg uas muaj tus kab mob splenectomies (tshem tawm tus po⁶)
• Qee zaum kev hloov ntshav tau qhia
• Qee zaum Splenectomy tau qhia⁷, tshwj xeeb tshaj yog nyob rau hauv cov neeg uas muaj kab mob sib kis, thalassemias uas feem ntau xav tau kev tso ntshav tab sis qee zaum kuj muaj lwm hom mob hemolytic anemia. Tseeb tiag, nws yog qhov loj hauv tus po uas cov qe ntshav liab raug rhuav tshem.
• Cortisone qee zaum raug muab tshuaj rau cov tshuaj tiv thaiv kab mob autoimmune anemia thiab xav txog kev mob khaub thuas antibody anemia. Nws yog qee zaum siv rau hauv cov xwm txheej ntawm paroxysmal nocturnal hemoglobinuria thiab tshwj xeeb tshaj yog rau thrombotic thrombocytopenic purpura. Cov tshuaj tiv thaiv kab mob muaj zog, xws li rituximab⁸, intravenous immunoglobulins, azathioprine, cyclophosphamide, thiab cyclosporine tej zaum yuav raug txiav txim siab hauv cov kab mob hemolytic anemias. Plasmapheresis qee zaum siv, tshwj xeeb tshaj yog nyob rau hauv cov ntaub ntawv ntawm no thrombotic thrombocytopenic purpura.

Raws li ib feem ntawm nws txoj hauv kev zoo, Passeportsanté.net caw koj kom pom qhov kev xav ntawm kws tshaj lij kev noj qab haus huv. Dr Dominic Larose, kws kho mob xwm txheej ceev, muab nws lub tswv yim rau koj hemolytic anemia :

Tsuas yog cov kev kho mob tsis sib xws tau txheeb xyuas txog kev mob qog nqaij hlav hauv hlwb. Saib daim ntawv no kom paub meej ntxiv.