I-anemia, ngokwencazelo, ihilela ukwehla kwamangqamuzana egazi abomvu, noma amazinga e-hemoglobin. Igama elithi "hemolytic anemia" lihlanganisa izinhlobo ezahlukene ze-anemia lapho amangqamuzana egazi abomvu ebhujiswa ngaphambi kwesikhathi egazini. Igama elithi "hemolysis" lisho ukubhujiswa kwamaseli abomvu egazi (hemo = igazi; lysis = ukubhujiswa).

Umnkantsha unomthamo othile wokugodla. Lokho wukuthi, ingakhuphula ukukhiqizwa kwayo kwamaseli abomvu kufinyelele ezingeni elithile ukukhokhela ukubhujiswa kwawo okwandisiwe. Ngokuvamile, amangqamuzana abomvu egazi ajikeleza emithanjeni yegazi cishe izinsuku ezingu-120. Ekupheleni kwempilo yabo, babhujiswa ubende nesibindi (bheka nephepha le-Anemia - ukubuka konke). Ukucekelwa phansi okusheshayo kwamangqamuzana abomvu egazi kuyisikhuthazi esibalulekile ekukhiqizeni amangqamuzana egazi abomvu amasha, alanyaniswa yihomoni ekhiqizwa izinso, i-erythropoietin (EPO). Kwezinye izimo, umnkantsha uyakwazi ukukhiqiza amangqamuzana abomvu egazi amaningi njengenani elicekeleke phansi ngendlela engavamile, ukuze izinga le-hemoglobin lingehli. Sikhuluma nge-hemolysis enxeshezelwe, ngaphandle kwe-anemia. Lokhu kubalulekile ngoba kunezici ezithile ezingabangela isimo ukuba sibole sibe yizici ezizophazamisa ukukhiqizwa kwe-EPO njengokukhulelwa, ukwehluleka kwezinso, ukuntula i-folic acid, noma ukutheleleka okunamandla.

Izimbangela

I-hemolytic anemia ijwayele ukuhlukaniswa ngokuya ngokuthi ngabe idalwa yiseli elibomvu elijwayelekile ngokwalo (intracorpuscular), noma isici esingaphandle kweseli elibomvu (extracorpuscular). Umahluko wenziwa futhi phakathi kwe-hemolytic anemia ezuzwe njengefa.

Izimbangela zofuzo kanye ne-intracorpuscular

• I-Hemoglobinopathies (isb. i-sickle cell anemia, njll.)
• Ama-Enzymopathies (isib. Ukushoda kwe-G6-PD)
• Ukukhubazeka kwe-ulwelwesi kanye ne-cytoskeletal (isb. Congenital spherocytosis)

Imbangela yofuzo kanye ne-extracorpuscular

• I-familial hemolytic-uremic syndrome (i-atypical)

Imbangela etholiwe kanye ne-intracorpuscular

• I-Paroxysmal nocturnal hemoglobinuria

Imbangela etholiwe kanye ne-extracorpuscular

• Ukubhujiswa kwemishini (microangiopathy)
• Ama-ejenti anobuthi
• izidakamizwa
• Izifo
• Immunological

Ake sixoxe ngezibonelo ezimbalwa-ke, njengoba kungenakwenzeka ukuzichaza zonke kumongo walo mbhalo.

I-Immunological hemolytic anemia:

Ukusabela kwe-Autoimmune. Kulokhu, umzimba, ngenxa yezizathu ezihlukahlukene, ukhiqiza amasosha omzimba ngokumelene namangqamuzana awo abomvu egazi: lezi zibizwa ngokuthi ama-autoantibodies. Kunezinhlobo ezimbili: lezo ezinama-autoantibodies ashisayo kanye nalawo anamasosha omzimba abandayo, kuye ngokuthi izinga lokushisa elilungile lomsebenzi we-antibody lingu-37 ° C noma 4 ° C. Lo mehluko ubalulekile ngoba ukwelashwa kuyehluka kuye ngesimo.

- Ama-autoantibodies ashisayo: ikakhulukazi athinta abantu abadala futhi abangele i-anemia ye-hemolytic engapheli futhi kwesinye isikhathi enamandla. Zimelela ama-80% we-autoimmune hemolytic anemias. Engxenyeni yezimo, zingabangelwa izidakamizwa ezithile (i-alpha-methyldopa, i-L-dopa) noma izifo ezithile (i-ovarian tumor, i-lymphoproliferative syndrome, njll.). Lokhu kubizwa ngokuthi "isibili" i-autoimmune hemolytic anemias, njengoba ibonakala njengomphumela wesinye isifo.

-Amasosha omzimba abandayo: ahlotshaniswa neziqephu ezinzima zokubhujiswa kwamangqamuzana abomvu egazi okubangelwa amakhaza. Ezimweni ezingama-30%, sibhekene nokuphendula okuzenzakalelayo okuzenzakalelayo okungachazwa ukutheleleka ngegciwane noma i-mycoplasma, igciwane eliphakathi eliphakathi kwamagciwane nama-bacteria.

Ukusabela kwe-Immunoallergic. Esimeni se-immunoallergic (non-autoimmune) i-hemolysis yezidakamizwa, amasosha omzimba awahlaseli amangqamuzana egazi abomvu, kodwa izidakamizwa ezithile: i-penicillin, i-cefalotin, i-cephalosporins, i-rifampicin, i-phenacetin, i-quinine, njll.

I-Congenital hemolytic anemia:

Kunezingxenye ezintathu ezibalulekile kumangqamuzana abomvu egazi. Kukhona i- hemoglobin, i-membrane-cytoskeleton complex, “nomshini” one-enzymatic okwenza konke kusebenze. Ukungajwayelekile kwezakhi zofuzo kunoma iyiphi yalezi zici ezintathu kungabangela i-hemolytic anemia.

Ukungajwayelekile kwefa lolwelwesi lwamangqamuzana egazi abomvu. Eyinhloko i-congenital spherocytosis, eqanjwe kanjalo ngenxa yesimo esiyindilinga esiveza amangqamuzana egazi abomvu futhi awenza abe buthaka ngokukhethekile. Ivamisile uma kuqhathaniswa: ikesi elingu-1 kwabangu-5000. Kunezinto ezimbalwa ezingavamile zofuzo ezihilelekile, uhlobo lwakudala luwukubusa kwe-autosomal, kodwa amafomu okuphindaphinda akhona. Kungabangela izinkinga ezithile: i-gallstones, izilonda emilenzeni.

Ama-Enzymopathies. Kunezinhlobo eziningana zokuntuleka kwe-enzyme ezingabangela i-hemolytic anemia. Ngokuvamile ziyifa. Okuvame kakhulu ukuntuleka kwe-enzyme ebizwa ngokuthi "glucose-6-phosphate dehydrogenase", edala ukubhujiswa ngaphambi kwesikhathi kwamangqamuzana abomvu egazi futhi, kamuva, i-hemolytic anemia.

Isici sofuzo esihilelekile sixhunyaniswa ne-X chromosome, ngakho-ke, amadoda kuphela angathinteka. Abesifazane bangathwala isifo sofuzo futhi basidlulisele ezinganeni zabo. Kubantu abanalokhu kuntuleka kwe-enzyme, i-anemia ye-hemolytic ivame ukwenzeka ngemva kokuchayeka kuma-oxidizing agents.

Abantu abanokuntula kwe-G6PD bangahlakulela i-hemolysis eyingozi lapho bevezwe kuma-ejenti athile afana nalokhu:

- ukusetshenziswa kwezinhlobonhlobo zikabhontshisi okubizwa ngokuthi i-small-grain bean (umlutha faba) noma ukuvezwa impova evela kuleso sitshalo (lezi zinhlobo zobhontshisi zisetshenziselwa ukudla kwemfuyo). Lokhu kuthintana kubangela i-acute hemolytic anemia ebizwa nangokuthi i-favism.

- ukusetshenziswa kwezidakamizwa ezithile: i-antimalarials, i-methyldopa (eyehlisa umfutho wegazi), i-sulfonamides (ama-antibacterial), i-aspirin, i-non-steroidal anti-inflammatory drugs, i-quinidine, i-quinine, njll.

– ukuchayeka kumakhemikhali athile anjengama-mothballs.

– izifo ezithile.

Lesi sifo sivame ukutholakala kubantu abavela eMedithera Basin (ikakhulukazi eGreece Islands) kanye nakubantu abamnyama e-Afrika nase-United States (lapho ukutholakala kwaso kuyi-10% kuya ku-14%). Kwezinye izingxenye zomhlaba, abantu abangamaphesenti angu-20 noma ngaphezulu banayo.

Ama-hemoglobinopathies. Igama elisetshenziselwa ukuchaza izifo zofuzo lapho ukukhiqizwa kwe-hemoglobin ngaphakathi kwamangqamuzana abomvu egazi kuthinteka. I-Sickle cell anemia (i-sickle cell anemia) ne-thalassemia yizigaba ezimbili eziyinhloko ze-hemoglobinopathies.

I-Sickle cell anemia (i-sickle cell anemia)^4,5. Lesi sifo esibi kakhulu sixhumene nokuba khona kwe-hemoglobin engajwayelekile ebizwa ngokuthi i-hemoglobin S. Lokhu kuhlanekezela amangqamuzana abomvu egazi futhi kuwanikeze umumo wocezwana noma isikela (amangqamuzana amasikela), ngaphezu kokubabulala. ngaphambi kwesikhathi. Bheka ishidi elithi Sickle cell anemia.

Thalassemia. Kusabalele kakhulu emazweni athile omhlaba, lesi sifo esibi sihlotshaniswa nokungajwayelekile kwezakhi zofuzo okuthinta ukukhiqizwa kwe-hemoglobin, le pigment yegazi emangqamuzaneni abomvu egazi avumela ukuthuthwa komoyampilo ezithweni. Amangqamuzana abomvu egazi athintekile antekenteke futhi aphuka ngokushesha. Igama elithi "thalassemia" livela egameni lesiGreki elithi "thalassa", elisho "ulwandle", njengoba labonwa okokuqala kubantu baseMedithera. Isici sofuzo singathinta izindawo ezimbili ekuhlanganisweni kwe-hemoglobin: iketango le-alpha noma iketango le-beta. Ngokuya ngohlobo lweketango elithintekile, kunezinhlobo ezimbili ze-thalassemia: i-alpha-thalassemia ne-beta-thalassemia.

Ezinye izimbangela

Izimbangela zemishini. Amaseli abomvu egazi angonakala ngesikhathi sokwelashwa okuthile okuhlobene nemishini eyimishini:

- ama-prostheses (ama-valve okwenziwa kwenhliziyo, njll.);

- ukuhlanzwa kwegazi okungaphandle (hemodialysis);

- umshini wokufaka umoya-mpilo egazini (osetshenziswa ekuhlinzweni kwenhliziyo-amaphaphu), njll.

Akuvamile, umgijimi wemarathon angase abhekane ne-hemolysis ngomshini njengoba ama-capillaries ezinyaweni echotshozwa ngokuphindaphindiwe. Lesi simo sibuye sachazwa ngemuva kwemidanso ethile ethatha isikhathi eside, ngezinyawo ezingenalutho.

Ukuvezwa kwezinto ezinobuthi.

- Imikhiqizo yezimboni noma yasekhaya enobuthi: i-aniline, i-arsenic hydrogen, i-nitrobenzene, i-naphthalene, i-paradichlorobenzene, njll.

- Isilwane esinobuthi: ukulunywa isicabucabu, ukuntinyelwa ngumnyovu, ubuthi benyoka.

– Izitshalo ezinobuthi: isikhunta esithile.

Izifo.I-gastroenteritis enzima ebangelwa Futhi coli, izifo ezibangelwa i-pneumococcus noma i-staphylococcus, i-hepatitis, i-typhoid fever, i-malaria, njll. I-Malaria (noma i-malaria) iyimbangela ebaluleke kakhulu kulesi sigaba. Umalaleveva ubangelwa igciwane elikhula ngaphakathi kwamangqamuzana abomvu egazi.

Ukungasebenzi kahle kwe-spleen. Kuvamile ukuthi amangqamuzana egazi abomvu abhujiswe kubende ngemva kohambo lwawo lwezinsuku ezingu-120, kodwa uma lesi sitho sisebenza ngokweqile, ukubhujiswa kuyashesha kakhulu bese kulandela i-anemia ye-hemolytic.

Hi-emoglobinuria i-paroxysmal ebusuku. Lesi sifo esingapheli sihlotshaniswa nokuba khona kwe-hemoglobin emchameni ngenxa yokubhujiswa ngokweqile kwamangqamuzana abomvu egazi. Ukuquleka ebusuku kubangelwa noma yiluphi uhlobo lokucindezeleka, ukuvuselela amasosha omzimba noma imithi ethile. Ngezinye izikhathi lesi sifo sibangela ubuhlungu obuphansi kanye nokungahambi kahle.

Izinkinga ezingenzeka: i-thrombosis, i-bone marrow hypoplasia, izifo zesibili.

• Lokho kuxhunyaniswa nezinga eliphansi lamaseli abomvu: ukubala okukhanyayo, ukukhathala, ubuthakathaka, isiyezi, ukushaya kwenhliziyo okusheshayo, njll.
• Jaundice.
• Umchamo omnyama.
• Ukwanda kobende.
• Lezo eziqondile kuhlobo ngalunye lwe-anemia ye-hemolytic. Bona "Incazelo Yezokwelapha".

Ngezinhlobo zokuzalwa ze-hemolytic anemia:

• Labo abanomlando womndeni.
• Abantu abavela endaweni yaseMedithera, e-Afrika, eNingizimu naseNingizimu-mpumalanga ye-Asia nase-West Indies.

• Kubantu abanokuntuleka kwe-enzyme ye-glucose-6-phosphate dehydrogenase: ukuchayeka kuma-oxidizing agents (izidakamizwa ezithile, ubhontshisi wasensimini, njll.).
• Ezinye izinhlobo ze-anemia ye-hemolytic:

  Izifo ezithile: i-hepatitis, ukutheleleka kwe-streptococcal noma E. coli, ukuphazamiseka kwe-autoimmune (njenge-lupus), isimila sesibeletho.

  - Izidakamizwa ezithile (izidakamizwa ezilwa nomalaleveva, i-penicillin, i-rifampicin, i-sulfonamides, njll.) noma izinto ezinobuthi (i-aniline, i-arsenic hydrogen, njll.).

  - Imishini ethile eyimishini esetshenziswa kwezokwelapha: amavalvu okwenziwa, amathuluzi okuhlanza noma okufaka umoya-mpilo egazini.

  - Ukucindezeleka.

• Njengamanje, akunakwenzeka ukuvimbela amafomu ofuzo ngaphandle kokubonisana nomeluleki wezakhi zofuzo ngaphambi kokukhulelwa ingane. Uchwepheshe uzokwazi ukuthola ubungozi bokubeletha ingane ene-hemolytic anemia lapho (noma bobabili) babazali abangahle babe nomlando womndeni (bona futhi iSickle Cell Anemia ukuthola eminye imininingwane ngobungozi bezofuzo maqondana naleli fomu i-anemia ye-hemolytic).
• Uma into ethile ibhekene nalesi sifo, kufanele igwenywe ukuvikela ukuphindeka.
• Ezinhlotsheni eziningi ze-anemia ye-hemolytic, kubalulekile futhi ukuqapha izifo ezithile.

Ziyahlukahluka kuye ngohlobo lwe-hemolytic anemia.

• Ukwelashwa kuqala futhi okuyinhloko kusekelwe ekusekelweni okujwayelekile komzimba kanye nembangela eyinhloko lapho kungenzeka
• Isengezo se-folic acid ngokuvamile siboniswa ezigulini ezine-anemia engapheli ye-hemolytic.
• Ukugoma ezifweni ezivamile kubalulekile kulezo ziguli eziphazamise ukuzivikela komzimba, ikakhulukazi kubantu abane-splenectomies (ukususwa kwe-spleen).⁶)
• Ngezinye izikhathi kuboniswa ukumpontshelwa igazi
• Ngezinye izikhathi i-splenectomy iyaphakanyiswa⁷, ikakhulukazi kubantu abane-hereditary spherocytosis, i-thalassemia evame ukudinga ukumpontshelwa kodwa futhi ngezinye izikhathi nakwezinye izinhlobo ze-hemolytic anemia engapheli. Ngempela, ikakhulukazi ku-spleen lapho amangqamuzana egazi abomvu abhujiswa khona.
• I-Cortisone ngezinye izikhathi inqunyelwe i-antibody autoimmune anemia eshisayo futhi icabangele i-anemia ebandayo ye-antibody. Kwesinye isikhathi isetshenziswa ezimeni ze-paroxysmal nocturnal hemoglobinuria futhi ikakhulukazi i-thrombotic thrombocytopenic purpura. Ama-immunosuppressive aqinile, njenge-rituximab⁸, i-intravenous immunoglobulins, i-azathioprine, i-cyclophosphamide, ne-cyclosporine ingase icatshangelwe ku-immunologic hemolytic anemias. Ngezinye izikhathi i-Plasmapheresis isetshenziswa, ikakhulukazi endabeni yale thrombotic thrombocytopenic purpura.

Njengengxenye yendlela yekhwalithi yayo, iPasseportsanté.net ikumema ukuthi uthole umbono wochwepheshe bezempilo. UDkt Dominic Larose, udokotela ophuthumayo, ukunika umbono wakhe nge i-hemolytic anemia :

Okuwukuphela kwezindlela zokwelapha ezingajwayelekile ezihlonziwe ziphathelene ne-sickle cell anemia. Bheka leli shidi ukuze uthole imininingwane eyengeziwe.