Anemia, netsanangudzo, inosanganisira kudzikira kwemasero matsvuku eropa, kana kuti hemoglobin. Izwi rekuti "hemolytic anemia" rinosanganisira marudzi akasiyana eanemia apo masero matsvuku eropa anoparadzwa nguva isati yakwana muropa. Izwi rokuti "hemolysis" rinoreva kuparadzwa kwemasero matsvuku eropa (hemo = ropa; lysis = kuparadza).

Mwongo webhonzo une imwe nzvimbo yekuchengetedza. Ndiko kuti, inogona kuwedzera kugadzirwa kwayo kwemasero matsvuku eropa kune imwe nhanho kutsiva kuparadzwa kwavo kwakawedzera. Kazhinji, masero matsvuku eropa anotenderera mutsinga dzeropa kwemazuva anenge 120. Pakupera kwehupenyu hwavo, vanoparadzwa nespleen uye chiropa (onawo Anemia sheet - overview). Kukurumidza kuparadzwa kwemasero matsvuku eropa chinhu chinokosha chinokurudzira mukugadzirwa kwemasero matsvuku matsva, anoyananiswa nehomoni rinogadzirwa neitsvo, erythropoietin (EPO). Mune zvimwe zviitiko, mwongo unokwanisa kugadzira masero matsvuku eropa akawanda sehuwandu hwakaparadzwa zvisina kujairika, saka chiyero chehemoglobin hachidonhe. Tiri kutaura nezve kubhadharwa hemolysis, pasina anemia. Izvi zvakakosha nekuti pane zvimwe zvinhu zvinogona kuita kuti mamiriro ezvinhu aore kuita zvinhu zvinokanganisa kugadzirwa kweEPO senge nhumbu, kusasimba kweitsvo, kushomeka kwefolic acid, kana hutachiona hwakanyanya.

Zvinokonzera

Hemolytic anemia inowanzo rongwa maererano nekuti inokonzerwa nesero dzvuku reropa iro pacharo risiri kujairika (intracorpuscular), kana chinhu chiri kunze kwesero dzvuku reropa (extracorpuscular). Musiyano unoitwawo pakati penhaka uye yakawanikwa hemolytic anemia.

Hereditary uye intracorpuscular zvikonzero

• Hemoglobinopathies (semuenzaniso, sickle cell anemia, nezvimwewo)
• Enzymopathies (semuenzaniso G6-PD kushomeka)
• Membrane uye cytoskeletal abnormalities (semuenzaniso congenital spherocytosis)

Hereditary uye extracorpuscular chikonzero

• Familial hemolytic-uremic syndrome (atypical)

Yakawanikwa uye intracorpuscular chikonzero

• Paroxysmal nocturnal hemoglobinuria

Yakawanikwa uye extracorpuscular chikonzero

• Kuparadza kweMechanical (microangiopathy)
• Chepfu mishonga
• Pharmaceuticals
• zvirwere
• Immunological

Ngatikurukurei mienzaniso mishoma ipapo, sezvo zvisingabviri kuitsanangura yose muchirevo chechinyorwa chino.

Immunological hemolytic anemia:

Autoimmune reactions. Muchiitiko ichi, muviri, nokuda kwezvikonzero zvakasiyana-siyana, unobudisa masoja ekudzivirira zvirwere pamasero matsvuku eropa: aya anonzi autoantibodies. Kune marudzi maviri: avo vane autoantibodies inopisa uye avo vane autoantibodies inotonhora, zvichienderana nekuti tembiricha yakakwana yebasa rekurwisa antibody i37 ° C kana 4 ° C. Musiyano uyu wakakosha nekuti kurapwa kunosiyana kubva pachimiro kuenda kune chimiro.

-Autoantibodies anopisa: anonyanya kubata vanhu vakuru uye anokonzera chisingaperi uye dzimwe nguva chakanyanya hemolytic anemia. Vanomiririra 80% ye autoimmune hemolytic anemias. Muhafu yezviitiko, zvinogona kukonzerwa nemimwe mishonga (alpha-methyldopa, L-dopa) kana zvimwe zvirwere (ovarian tumor, lymphoproliferative syndrome, nezvimwewo). Izvi zvinonzi "secondary" autoimmune hemolytic anemias, sezvo inoratidzika semugumisiro wechimwe chirwere.

- Cold auto-antibodies: inosanganiswa neacute episode yekuparadza kwemasero matsvuku eropa kunokonzerwa nekutonhora. Muchikamu che30% chezviitiko, tiri kutarisana nechechipiri autoimmune reaction inogona kutsanangurwa nehutachiona hwehutachiona kana mycoplasma, microorganism yepakati pakati pehutachiona nehutachiona.

Immunoallergic reactions. Muchiitiko che immunoallergic (kwete-autoimmune) zvinodhaka hemolysis, masoja ekudzivirira chirwere haarwise masero matsvuku eropa, asi mamwe madhiragi: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, nezvimwe.

Congenital hemolytic anemia:

Pane zvinhu zvitatu zvakakosha mumasero matsvuku eropa. Kune hemoglobin, membrane-cytoskeleton complex, uye enzymatic "muchina" kuita kuti zvose zvishande. Genetic abnormalities mune chimwe chezvinhu zvitatu izvi zvinogona kukonzera hemolytic anemia.

Hereditary abnormalities ye membrane ye red blood cells. Iyo huru ndeye congenital spherocytosis, yakadanwa nekuda kwechimiro chedenderedzwa icho chinobva charatidza masero matsvuku eropa uye anoita kuti asanyanya kusimba. Inowanzoitika: 1 kesi muna 5000. Zvakawanda zvisizvo zvemajini zvinosanganisirwa, iyo classic fomu ndeye autosomal inotonga, asi recessive mafomu aripowo. Inogona kukonzera mamwe matambudziko: gallstones, maronda pamakumbo.

Enzymopathies. Kune akati wandei maitiro ekushaya enzyme anogona kukonzera hemolytic anemia. Vanowanzogara nhaka. Kunyanya kushaikwa mune enzyme inonzi "glucose-6-phosphate dehydrogenase", iyo inokonzera kuparadzwa kusati kwasvika kwemasero matsvuku eropa uye, zvino, hemolytic anemia.

Iyo genetic defect inobatanidzwa yakabatana neX chromosome, saka, varume chete ndivo vanogona kukanganiswa. Vakadzi vanogona kutakura chikanganiso chemajini vochipfuudza kuvana vavo. Muvanhu vane kushomeka kwe enzyme iyi, hemolytic anemia inowanzoitika zvichitevera kuratidzwa kune oxidizing agents.

Vanhu vane G6PD kushomeka vanogona kukura acute hemolysis kana vakafumurwa kune vamwe vamiririri vakaita se:

– kudyiwa kwemhando yebhinzi inonzi small-grain bean (addictive faba) kana kusangana nemukume wechirimwa ichocho (mhando yebhinzi iyi inoshandiswa pakudya kwezvipfuyo). Kubata uku kunoguma neacute hemolytic anemia inonziwo favism.

- kushandiswa kwemamwe mishonga: antimalarials, methyldopa (inoderedza BP), sulfonamides (antibacterial), aspirin, non-steroidal anti-inflammatory drugs, quinidine, quinine, nezvimwewo.

-kuratidzwa kune mamwe makemikari senge mothballs.

- zvimwe zvirwere.

Chirwere ichi chinowanzoonekwa kuvanhu vanobva kuMediterranean Basin (kunyanya kuGreece Islands) pamwe chete nevanhu vatema muAfrica neUnited States (uko kunowanikwa 10% kusvika 14%). Mune dzimwe nzvimbo dzenyika, 20% kana kupfuura yevagari vanayo.

Hemoglobinopathies. Izwi rinoshandiswa kurondedzera zvirwere zvemajini uko kugadzirwa kwehemoglobin mukati memasero matsvuku eropa kunokanganiswa. Sickle cell anemia (sickle cell anemia) uye thalassemia ndiwo mapoka maviri makuru ehemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Ichi chirwere chakakomba zvikuru chine chokuita nokuvapo kwe<em>hemoglobin isina kujairika inonzi hemoglobin S. Izvi zvinokanganisa masero matsvuku eropa uye zvinoapa chimiro che<em>crescent kana kuti scythe (sickle cells), kuwedzera pakukonzera kuti afe. nguva isati yakwana. Ona bepa reSickle cell anemia.

Thalassemia. Yakapararira kwazvo mune dzimwe nyika dzepasi, chirwere ichi chakakomba chakabatana neiyo genetic abnormality inokanganisa kugadzirwa kwehemoglobin, iyi pigment yeropa mumasero matsvuku eropa anobvumira kutakurwa kweokisijeni kune nhengo. Masero matsvuku eropa akabatwa haana simba uye anokurumidza kuputsika. Izwi rokuti "thalassemia" rinobva pashoko rechiGiriki rokuti "thalassa", rinoreva "gungwa", sezvarakatanga kuonekwa muvanhu vanobva kuMediterranean. Iyo genetic defect inogona kukanganisa nzvimbo mbiri mu synthesis yehemoglobin: iyo alpha chain kana beta cheni. Zvichienderana nerudzi rwecheni yakabatwa, kune marudzi maviri ethalassemia: alpha-thalassemia uye beta-thalassemia.

Zvimwe zvinokonzeresa

Mechanical zvinokonzera. Masero matsvuku eropa anogona kukuvadzwa panguva yemamwe marapirwo ane chekuita nemishini yemagetsi:

- prostheses (mavharuvhu ekugadzira emwoyo, nezvimwewo);

- extracorporeal kuchenesa ropa (hemodialysis);

- muchina wekuisa oksijeni muropa (unoshandiswa mukuvhiya mwoyo-mapapu), nezvimwe.

Kashoma, mumhanyi wemarathon anogona kuona mechanical hemolysis sezvo capillaries mutsoka anoramba achipwanyika. Mamiriro ezvinhu aya akatsanangurwa zvakare mushure memamwe matambiro akareba kwazvo, netsoka dzisina shangu.

Kusangana nezvinhu zvine chepfu.

- Zvigadzirwa zveindasitiri kana zvemumba zvine chepfu: aniline, arsenic hydrogen, nitrobenzene, naphthalene, paradichlorobenzene, nezvimwe.

- Mhuka ine muchetura: kurumwa nedandemutande, kurumwa newava, uturu hwenyoka.

- Dyara muchetura: mamwe fungi.

Zvirwere.Gastroenteritis yakaoma inokonzerwa ne Uye coli, zvirwere zvinokonzerwa nepneumococcus kana staphylococcus, hepatitis, typhoid fever, marariya, nezvimwewo. Malaria (kana marariya) ndiyo inonyanya kukosha muchikwata ichi. Malaria inokonzerwa neutachiona hunokura mukati memasero matsvuku eropa.

Hyperfunction yespleen. Zvakajairika kuti masero matsvuku eropa aparadzwe muberere mushure merwendo rwavo rwemazuva 120, asi kana nhengo iyi ikashanda zvakanyanya, kuparadzwa kunokurumidza uye hemolytic anemia inoitika.

Hhemoglobinuria paroxysmal nocturnal. Ichi chirwere chisingaperi chinosanganiswa nekuvapo kwe hemoglobin muweti nekuda kwekuparadzwa kwakanyanya kwemasero matsvuku eropa. Kubata kwehusiku kunokonzerwa nemhando ipi neipi yekushushikana, immune system stimulation kana mimwe mishonga. Dzimwe nguva chirwere chinokonzera marwadzo emusana uye kusagadzikana.

Zvinetso zvinogona kuitika: thrombosis, bone marrow hypoplasia, zvirwere zvechipiri.

• Izvo zvakabatanidzwa kune yakaderera mwero wemasero matsvuku eropa: ruvara rwakacheneruka, kuneta, kushaya simba, dzungu, kukurumidza kurova kwemoyo, nezvimwe.
• Jaundice.
• Weti yakasviba.
• Kuwedzera kwembeu.
• Izvo zvakananga kune imwe neimwe fomu yehemolytic anemia. Ona "Kutsanangurwa Kwezvekurapa".

Zvekuzvarwa mafomu ehemolytic anemia:

• Avo vane nhoroondo yemhuri.
• Vanhu vanobva kuMediterranean basin, Africa, South uye Southeast Asia uye West Indies.

• Mune vanhu vane kushomeka kweiyo enzyme glucose-6-phosphate dehydrogenase: kuratidzwa kune oxidizing agents (mimwe mishonga, bhinzi yemumunda, nezvimwewo).
• Kune mamwe marudzi ehemolytic anemia:

  - Zvimwe zvirwere: hepatitis, streptococcal utachiona kana E. coli, autoimmune disorders (yakadai se lupus), bundu reovarian.

  - Mimwe mishonga (antimalarials, penicillin, rifampicin, sulfonamides, nezvimwewo) kana mishonga ine chepfu (aniline, arsenic hydrogen, nezvimwewo).

  - Mimwe michina yemagetsi inoshandiswa mukurapa: mavharuvhu ekugadzira, zvishandiso zvekuchenesa kana kuisa mweya weropa.

  — Kushushikana.

• Parizvino, hazvibviri kudzivirira mafomu okugarwa nhaka kunze kwekunge wabvunza mupi wezano wemajini asati abata mwana. Nyanzvi ichakwanisa kuona njodzi dzekuzvara mwana ane hemolytic anemia kana (kana vese) vevabereki vangangove vaine nhoroondo yemhuri (onawo Sickle Cell Anemia kuti uwane rumwe ruzivo nezve njodzi dzemajini maererano nefomu iri. yehemolytic anemia).
• Kana chimwe chinhu chinokonzera chirwere chacho, chinofanira kudziviswa kudzivirira kudzokorora.
• Kune akawanda marudzi ehemolytic anemia, zvakakoshawo kuchengetedza kune humwe hutachiona.

Zvinosiyana zvichienderana nerudzi rwehemolytic anemia.

• Kurapa kunotanga uye kunonyanya kuenderana nerutsigiro rwese kune muviri uye chikonzero chinokonzera kana zvichibvira
• Kuwedzera kwe folic acid kunowanzo kuratidzwa kune varwere vane chisingaperi hemolytic anemia.
• Kudzivirira zvirwere zvakajairika kwakakosha kune avo varwere vane kukanganisa kudzivirira kwekudzivirira, kunyanya kune vanhu vane splenectomies (kubviswa kwepleen).⁶)
• Kuwedzerwa ropa pane dzimwe nguva kunoratidzirwa
• Splenectomy dzimwe nguva inokurudzirwa⁷, kunyanya muvanhu vane hereditary spherocytosis, thalassemias iyo inowanzoda kuwedzerwa ropa asiwo dzimwe nguva mune mamwe marudzi echisingaperi hemolytic anemia. Zvechokwadi, zvakanyanya muspleen umo masero matsvuku eropa anoparadzwa.
• Cortisone dzimwe nguva inonyorerwa kupisa antibody autoimmune anemia uye kufunga nezve inotonhora antibody anemia. Iyo dzimwe nguva inoshandiswa muzviitiko zveparoxysmal nocturnal hemoglobinuria uye kunyanya kune thrombotic thrombocytopenic purpura. Yakasimba immunosuppressive agents, senge rituximab⁸, intravenous immunoglobulins, azathioprine, cyclophosphamide, uye cyclosporine inogona kufungwa mune immunologic hemolytic anemias. Plasmapheresis dzimwe nguva inoshandiswa, kunyanya munyaya yeiyi thrombotic thrombocytopenic purpura.

Sechikamu chemabatiro ayo emhando yepamusoro, Passeportsanté.net inokukoka iwe kuti uwane maonero eanachiremba. Dr Dominic Larose, chiremba wechimbichimbi, anokupa maonero ake pane iyo hemolytic kupererwa neropa :

Mishonga isina kujairika chete yakaratidza nezve sickle cell anemia. Ona pepa iri kuti uwane rumwe ruzivo.