Khaello ea mali, ho ea ka tlhaloso, e akarelletsa ho fokotseha ha lisele tse khubelu tsa mali, kapa boemo ba hemoglobin. Poleloana "hemolytic anemia" e akaretsa mefuta e fapaneng ea phokolo ea mali moo lisele tse khubelu tsa mali li senyehang pele ho nako maling. Lentsoe "hemolysis" le bolela ho senyeha ha lisele tse khubelu tsa mali (hemo = mali; lysis = timetso).

Moko oa masapo o na le sebaka se itseng sa polokelo. Ke hore, e ka eketsa tlhahiso ea eona ea lisele tse khubelu tsa mali ho isa boemong bo itseng ho lefella tšenyo ea tsona e eketsehileng. Ka tloaelo, lisele tse khubelu tsa mali li potoloha methapong ea mali ka matsatsi a ka bang 120. Qetellong ea bophelo ba bona, ba senngoa ke spleen le sebete (sheba le Anemia sheet - kakaretso). Ho senyeha ho potlakileng ha lisele tse khubelu tsa mali ke tšusumetso ea bohlokoa tlhahisong ea lisele tse khubelu tsa mali, tse kenngoeng ke homone e hlahisoang ke liphio, erythropoietin (EPO). Maemong a mang, moko oa masapo o khona ho hlahisa lisele tse khubelu tsa mali tse ngata ho feta tekanyo e senyehileng ka mokhoa o sa tloaelehang, kahoo tekanyo ea hemoglobin ha e theohe. Re bua ka compensed hemolysis, ntle le khaello ea mali. Sena ke sa bohlokoa hobane ho na le lintlha tse itseng tse ka etsang hore boemo bo bole hore e be lintlha tse tla sitisa tlhahiso ea EPO e kang ho ima, ho hloleha ha liphio, ho haella ha folic acid, kapa tšoaetso e matla.

Lisosa

Hemolytic anemia ka kakaretso e hlophisoa ho latela hore na e bakoa ke sele e khubelu ea mali eo ka boeona e sa tloaelehang (intracorpuscular), kapa ntho e kantle ho sele e khubelu ea mali (extracorpuscular). Phapang e boetse e etsoa lipakeng tsa lefutso le lefu la haemolytic anemia.

Lisosa tsa lefutso le intracorpuscular

• Hemoglobinopathies (mohlala, sickle cell anemia, joalo-joalo)
• Enzymopathies (mohlala, khaello ea G6-PD)
• Ho se tloaelehe ha Membrane le cytoskeletal (mohlala, congenital spherocytosis)

Lebaka la lefutso le extracorpuscular

• Lefu la lelapa la hemolytic-uremic (atypical)

E fumanoeng le sesosa sa intracorpuscular

• Paroxysmal nocturnal hemoglobinuria

Lebaka le fumanweng le extracorpuscular

• Ho senyeha ha mechine (microangiopathy)
• Lintho tse chefo
• Meriana
• ditshwaetso
• Immunological

Joale, a re tšohleng mehlala e seng mekae, kaha ho ke ke ha khoneha ho e hlalosa kaofela ho latela moelelo oa tokomane ena.

Immunological hemolytic anemia:

Boitšoaro ba Autoimmune. Tabeng ena, 'mele, ka mabaka a sa tšoaneng, o hlahisa li-antibodies khahlanong le lisele tse khubelu tsa mali: tsena li bitsoa li-autoantibodies. Ho na le mefuta e 'meli: ba nang le li-autoantibodies tse chesang le tse nang le li-autoantibodies tse batang, ho itšetlehile ka hore na mocheso o nepahetseng bakeng sa ts'ebetso ea antibody ke 37 ° C kapa 4 ° C. Phapang ena ke ea bohlokoa hobane phekolo e fapana ho ea ka sebōpeho.

- Li-autoantibodies tse chesang: li ama batho ba baholo haholo 'me li baka phokolo ea mali e sa foleng le ka linako tse ling e matla haholo. Li emela karolo ea 80 lekholong ea anemia ea autoimmune hemolytic. Halofo ea linyeoe, li ka hlahisoa ke lithethefatsi tse itseng (alpha-methyldopa, L-dopa) kapa mafu a itseng ( hlahala ea mahe a bomme, lefu la lymphoproliferative, joalo-joalo). Sena se bitsoa "secondary" autoimmune hemolytic anemia, kaha li hlaha ka lebaka la lefu le leng.

- Li-antibodies tse batang: li amahanngoa le liketsahalo tse matla tsa timetso ea lisele tse khubelu tsa mali tse bakoang ke serame. Maemong a 30%, re sebetsana le karabelo ea bobeli ea autoimmune e ka hlalosoang ke tšoaetso ea kokoana-hloko kapa mycoplasma, likokoana-hloko tse pakeng tsa likokoana-hloko le libaktheria.

Matšoao a immunoallergic. Tabeng ea immunoallergic (non-autoimmune) lithethefatsi hemolysis, li-antibodies ha li hlase lisele tse khubelu tsa mali, empa lithethefatsi tse ling: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, joalo-joalo.

Congenital hemolytic anemia:

Ho na le likarolo tse tharo tsa bohlokoa liseleng tse khubelu tsa mali. Ho na le hemoglobin, membrane-cytoskeleton complex, le “mochini” oa enzymatic o etsang hore lintho tsohle li sebetse. Ho se tloaelehe ha liphatsa tsa lefutso ho efe kapa efe ea lintlha tsena tse tharo ho ka baka phokolo ea mali ea hemolytic.

Matšoao a lefutso a lera la lisele tse khubelu tsa mali. E ka sehloohong ke congenital spherocytosis, e rehelletsoeng joalo ka lebaka la sebopeho sa spherical se tšoaeang lisele tse khubelu tsa mali le tse etsang hore li senyehe haholo. E batla e le khafetsa: nyeoe e le 'ngoe ho 1. Ho na le mefokolo e mengata ea liphatsa tsa lefutso, mofuta oa khale ke autosomal dominate, empa mefuta e mengata e teng hape. E ka baka mathata a itseng: gallstones, liso maotong.

Enzymopathies. Ho na le mefuta e 'maloa ea khaello ea enzyme e ka bakang khaello ea mali ea hemolytic. Ka kakaretso ke lefutso. Ntho e tloaelehileng haholo ke khaello ea enzyme e bitsoang "glucose-6-phosphate dehydrogenase", e bakang timetso ea lisele tse khubelu tsa mali pele ho nako, ka mor'a moo, hemolytic anemia.

Bothata ba liphatsa tsa lefutso bo amehang bo amana le X chromosome, ka hona, ke banna feela ba ka amehang. Basali ba ka jara bofokoli boo ba liphatsa tsa lefutso ’me ba bo fetisetsa ho bana ba bona. Ho batho ba nang le khaello ea enzyme ena, phokolo ea mali ea hemolytic hangata e hlaha kamora ho pepesehela li-oxidizing agents.

Batho ba nang le khaello ea G6PD ba ka hlaolela hemolysis e matla ha ba pepesehetse mahlahana a itseng a kang:

- tšebeliso ea mefuta e fapaneng ea linaoa tse bitsoang li-small-grain bean (faba e lemaletseng) kapa ho pepesehela peō e phofshoana e tsoang semeleng seo (mofuta ona oa linaoa o sebelisoa ho fepa liphoofolo). Ho kopana hona ho fella ka khaello ea mali e matla ea hemolytic eo hape e bitsoang favism.

- tšebeliso ea lithethefatsi tse itseng: antimalarials, methyldopa (fokotsa khatello ea mali), sulfonamides (antibacterial), aspirin, non-steroidal anti-inflammatory lithethefatsi, quinidine, quinine, joalo-joalo.

- ho pepesehela lik'hemik'hale tse itseng joalo ka mothballs.

– mafu a itseng.

Hangata lefu lena le fumanoa ho batho ba tsoang Mediterranean Basin (haholo-holo Lihlekehlekeng tsa Greece) hammoho le batho ba batšo ba Afrika le United States (moo ho ata ha bona ho 10% ho 14%). Likarolong tse ling tsa lefatše, 20% kapa ho feta ea baahi ba na le eona.

Hemoglobinopathies. Lentsoe le sebelisetsoang ho hlalosa mafu a lefutso moo tlhahiso ea hemoglobin ka har'a lisele tse khubelu tsa mali e amehang. Sickle cell anemia (sickle cell anemia) le thalassemia ke lihlopha tse peli tse kholo tsa hemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Boloetse bona bo batlang bo le kotsi bo amahanngoa le ho ba teng ha hemoglobin e sa tloaelehang e bitsoang hemoglobin S. Sena se khopamisa lisele tse khubelu tsa mali ’me se li fa sebōpeho sa crescent kapa scythe (sickle cell), ho phaella ho li bakang ho shoa. pele ho nako. Sheba leqephe la Sickle cell anemia.

Thalassemia. E atile haholo linaheng tse ling tsa lefats'e, lefu lena le tebileng le amahanngoa le ho se tloaelehe ha liphatsa tsa lefutso tse amang tlhahiso ea hemoglobin, pigment ena ea mali liseleng tse khubelu tsa mali tse lumellang ho tsamaisoa ha oksijene lithong tsa 'mele. Lisele tse khubelu tsa mali tse amehileng lia fokola 'me li robeha kapele. Lentsoe "thalassemia" le tsoa lentsoeng la Segerike "thalassa", le bolelang "leoatle", joalokaha le ne le bonoa ka lekhetlo la pele ho batho ba tsoang libakeng tsa Mediterranean. Bofokoli ba liphatsa tsa lefutso bo ka ama libaka tse peli ka har'a motsoako oa hemoglobin: ketane ea alpha kapa ketane ea beta. Ho itšetlehile ka mofuta oa ketane e amehang, ho na le mefuta e 'meli ea thalassemia: alpha-thalassemia le beta-thalassemia.

Lisosa tse ling

Lisosa tsa mechine. Lisele tse khubelu tsa mali li ka senyeha nakong ea kalafo e itseng e amanang le lisebelisoa tsa mochini:

- li-prostheses (li-valve tsa maiketsetso tsa pelo, joalo-joalo);

- tlhoekiso ea mali ea extracorporeal (hemodialysis);

- mochini o kenyang oksijene maling (o sebelisoang ho buuoa ka pelo le matšoafo), joalo-joalo.

Hangata, semathi sa marathone se ka 'na sa fumana hemolysis ea mechine ha li-capillaries maotong li ntse li pshatleha khafetsa. Boemo bona bo boetse bo hlalositsoe ka mor'a metjeko e itseng ea nako e telele haholo ea moetlo, ka maoto a sa roala letho.

Ho pepesehela lintho tse chefo.

- Lihlahisoa tse chefo tsa indasteri kapa tsa malapeng: aniline, arsenic hydrogen, nitrobenzene, naphthalene, paradichlorobenzene, joalo-joalo.

– Phoofolo e chefo: ho longoa ke sekho, ho longoa ke bobi, mahloko a noha.

- Semela se chefo: li-fungus tse itseng.

Tšoaetso.Koko e matla e bakoang ke Le coli, mafu a bakoang ke pneumococcus kapa staphylococcus, hepatitis, feberu ea mala, malaria, joalo-joalo Malaria (kapa malaria) ke sesosa sa bohlokoa ka ho fetisisa sehlopheng sena. Malaria e bakoa ke kokoana-hloko e hōlang ka har'a lisele tse khubelu tsa mali.

Hyperfunction ea spleen. Ke ntho e tloaelehileng hore lisele tse khubelu tsa mali li senyehe ka har'a spleen ka mor'a leeto la matsatsi a 120, empa haeba setho sena se sebetsa ka mokhoa o feteletseng, timetso e potlakile haholo 'me mali a haemolytic a hlaha.

Hhemoglobinuria paroxysmal bosiu. Lefu lena le sa foleng le amahanngoa le boteng ba haemoglobin ka moroto ka lebaka la timetso e feteletseng ea lisele tse khubelu tsa mali. Ho tsieleha ha bosiu ho bakoa ke mofuta ofe kapa ofe oa khatello ea kelello, ho susumetsa sesole sa 'mele kapa meriana e itseng. Ka linako tse ling lefu lena le baka bohloko bo ka tlase ba mokokotlo le ho se thabise.

Mathata a ka bang teng: thrombosis, hypoplasia ea moko oa masapo, tšoaetso ea bobeli.

• Tse amahanngoang le boemo bo tlaase ba lisele tse khubelu tsa mali: lebala le lerootho, mokhathala, bofokoli, ho tsekela, ho otla ha pelo ka potlako, joalo-joalo.
• Jaundice.
• Moroto o lefifi.
• Ho atolosoa ha spleen.
• Tse tobileng mofuta o mong le o mong oa phokolo ea mali ea hemolytic. Sheba "Tlhaloso ea Bongaka".

Bakeng sa mefuta ea congenital ea anemia ea hemolytic:

• Ba nang le nalane ea lelapa.
• Batho ba tsoang libakeng tsa Mediterranean, Afrika, Boroa le Boroa-bochabela Asia le West Indies.

• Ho batho ba nang le khaello ea enzyme glucose-6-phosphate dehydrogenase: ho pepesehela mahlahana a oxidizing (lithethefatsi tse itseng, linaoa tsa masimong, joalo-joalo).
• Bakeng sa mefuta e meng ea anemia ea hemolytic:

  - Mafu a mang: lefu la sebete, tšoaetso ea streptococcal kapa E. coli, mathata a autoimmune (joaloka lupus), hlahala ea mahe a bomme.

  - Lithethefatsi tse ling (li-antimalarial, penicillin, rifampicin, sulfonamides, joalo-joalo) kapa lintho tse chefo (aniline, arsenic hydrogen, joalo-joalo).

  - Lisebelisoa tse ling tse sebelisoang ho tsa bongaka: li-valve tsa maiketsetso, lisebelisoa tsa ho hloekisa kapa ho kenya mali maling.

  - Khatello ea maikutlo.

• Hona joale, ho ke ke ha khoneha ho thibela mefuta ea lefutso ntle le ho buisana le moeletsi oa liphatsa tsa lefutso pele u emola ngoana. Setsebi se tla khona ho fumana likotsi tsa ho tsoala ngoana ea nang le mali a haemolytic ha e mong (kapa ka bobeli) ba bao e ka bang batsoali ba e-na le nalane ea lelapa (sheba le Sickle Cell Anemia bakeng sa lintlha tse ling mabapi le likotsi tsa lefutso mabapi le foromo ena. khaello ea mali ea haemolytic).
• Haeba ntho e itseng e ikarabella bakeng sa lefu lena, e lokela ho qojoa ho thibela ho pheta-pheta.
• Bakeng sa mefuta e mengata ea phokolo ea mali ea haemolytic, ho bohlokoa hape ho itebela khahlanong le mafu a itseng.

Li fapane ho latela mofuta oa phokolo ea mali ea hemolytic.

• Kalafo ke ea pele le e ka sehloohong e thehiloeng ho tšehetso e akaretsang ho 'mele le sesosa se ka sehloohong ha ho khoneha
• Tlatsetso ea folic acid hangata e bontšoa ho bakuli ba nang le anemia e sa foleng ea hemolytic.
• Ho thibela mafu a tloaelehileng ho bohlokoa ho bakuli ba nang le ts'ireletso ea 'mele ea ho itšireletsa mafung, haholo-holo ho batho ba nang le splenectomies (ho tlosoa ha spleen).⁶)
• Ka linako tse ling tšelo ea mali e bontšoa
• Ka linako tse ling ho etsoa tlhahiso ea ho tlosa splenectomy⁷, haholo-holo ho batho ba nang le lefutso la spherocytosis, thalassemias tseo hangata li hlokang tšelo ea mali empa hape ka linako tse ling le mefuteng e meng ea phokolo ea mali e sa foleng ea hemolytic. Ka sebele, ke karolo e khōlō ea spleen moo lisele tse khubelu tsa mali li senyehang.
• Cortisone ka linako tse ling e laeloa bakeng sa hot antibody autoimmune anemia le ho nahana ka phokolo ea mali e batang. Ka linako tse ling e sebelisoa maemong a paroxysmal nocturnal hemoglobinuria mme haholoholo bakeng sa thrombotic thrombocytopenic purpura. Lisebelisoa tse matla tsa immunosuppressive, joalo ka rituximab⁸, li-immunoglobulins tse kenang methapong, azathioprine, cyclophosphamide, le cyclosporine li ka ’na tsa nkoa e le immunologic hemolytic anemias. Ka linako tse ling plasmapheresis e sebelisoa, haholo-holo tabeng ea thrombotic thrombocytopenic purpura.

E le karolo ea mokhoa oa eona oa boleng, Passeportsanté.net eu mema hore u fumane maikutlo a setsebi sa bophelo bo botle. Ngaka Dominic Larose, ngaka ea maemo a tšohanyetso, ou fa maikutlo a hae mabapi le haemolytic khaello ea mali :

Liphekolo tse sa tloaelehang feela tse boletsoeng li amana le sickle cell anemia. Sheba leqephe lena bakeng sa lintlha tse ling.