Kuperewera kwa magazi m'thupi, mwa kutanthauzira, kumaphatikizapo kuchepa kwa maselo ofiira a magazi, kapena hemoglobini. Mawu akuti "hemolytic anemia" akuphatikizapo mitundu yosiyanasiyana ya kuchepa kwa magazi m'magazi kumene maselo ofiira amawonongeka msanga m'magazi. Mawu akuti "hemolysis" amatanthauza kuwonongeka kwa maselo ofiira a magazi (hemo = magazi; lysis = kuwonongeka).

Mafupa ali ndi mphamvu yosungira. Ndiko kuti, ikhoza kuonjezera kupanga kwake kwa maselo ofiira a magazi kufika pamlingo wina kuti apereke chiwonongeko chawo chowonjezeka. Nthawi zambiri, maselo ofiira amagazi amazungulira m'mitsempha kwa masiku pafupifupi 120. Kumapeto kwa moyo wawo, amawonongedwa ndi ndulu ndi chiwindi (onaninso pepala la Anemia - mwachidule). Kuwonongeka kofulumira kwa maselo ofiira a m’magazi ndi chinthu chofunika kwambiri popanga maselo ofiira a m’magazi, amene amapangidwa ndi impso, yotchedwa erythropoietin (EPO). Nthawi zina, mafupa amatha kupanga maselo ofiira ambiri monga momwe amawonongera mosadziwika bwino, kotero kuti mlingo wa hemoglobin sutsika. Tikulankhula za kulipidwa hemolysis, popanda magazi m'thupi. Izi ndi zofunika chifukwa pali zinthu zina zomwe zingapangitse kuti vutoli liwonongeke kukhala zinthu zomwe zingasokoneze kupanga EPO monga mimba, kulephera kwa impso, kuchepa kwa folic acid, kapena matenda aakulu.

Zimayambitsa

Kuperewera kwa magazi m'thupi la hemolytic nthawi zambiri kumadziwika kuti kumachitika chifukwa cha maselo ofiira a m'magazi omwe ali osadziwika bwino (intracorpuscular), kapena chinthu chomwe chili kunja kwa maselo ofiira a magazi (extracorpuscular). Kusiyanitsa kumapangidwanso pakati pa cholowa ndi kupeza hemolytic anemia.

Zoyambitsa zobadwa ndi intracorpuscular

• Hemoglobinopathies (mwachitsanzo, sickle cell anemia, etc.)
• Enzymopathies (mwachitsanzo, kuchepa kwa G6-PD)
• Matenda a Membrane ndi cytoskeletal (mwachitsanzo congenital spherocytosis)

Cholowa ndi extracorpuscular chifukwa

• Family hemolytic-uremic syndrome (atypical)

Chifukwa chopezeka komanso intracorpuscular

• Paroxysmal nocturnal hemoglobinuria

Chifukwa chopezedwa ndi extracorpuscular

• Kuwonongeka kwamakina (microangiopathy)
• Zothandizira poizoni
• Mankhwala
• matenda
• Immunological

Tiyeni tikambirane zitsanzo zingapo, popeza kuti n’zosatheka kuzifotokoza zonse mogwirizana ndi zimene zili m’bukuli.

Immunological hemolytic anemia:

Zochita za Autoimmune. Pachifukwa ichi, thupi, pazifukwa zosiyanasiyana, limapanga ma antibodies motsutsana ndi maselo ake ofiira a magazi: awa amatchedwa autoantibodies. Pali mitundu iwiri: omwe ali ndi autoantibodies otentha ndi omwe ali ndi autoantibodies ozizira, malingana ndi kutentha kwabwino kwa antibody ndi 37 ° C kapena 4 ° C. Kusiyana kumeneku n'kofunika chifukwa chithandizo chimasiyana kuchokera ku mawonekedwe kupita ku mawonekedwe.

-Autoantibodies otentha: amakhudza kwambiri akuluakulu ndipo amayambitsa kuchepa kwa magazi m'thupi komanso nthawi zina kwambiri. Amayimira 80% ya autoimmune hemolytic anemias. Mu theka la milandu, amatha kuyambitsidwa ndi mankhwala ena (alpha-methyldopa, L-dopa) kapena matenda ena (chotupa cha ovarian, lymphoproliferative syndrome, etc.). Izi zimatchedwa "sekondale" autoimmune hemolytic anemias, chifukwa amawoneka chifukwa cha matenda ena.

- Cold auto-antibodies: amalumikizidwa ndi magawo owopsa a kuwonongeka kwa maselo ofiira amwazi chifukwa cha kuzizira. Mu 30% ya milandu, tikulimbana ndi vuto lachiwiri la autoimmune lomwe limatha kufotokozedwa ndi matenda a virus kapena mycoplasma, kachilombo kamene kamakhala pakati pa ma virus ndi mabakiteriya.

Immunoallergic reactions. Ngati immunoallergic (non-autoimmune) mankhwala hemolysis, ma antibodies saukira maselo ofiira a magazi, koma mankhwala ena: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, ndi zina zotero.

Congenital hemolytic anemia:

Pali zigawo zitatu zofunika kwambiri m'maselo ofiira a magazi. Pali hemoglobini, membrane-cytoskeleton complex, ndi "makina" a enzymatic kuti azitha kugwira ntchito. Kusokonezeka kwa majini pazifukwa zitatuzi kungayambitse kuchepa kwa magazi m'thupi.

Kubadwa kwachilendo kwa nembanemba ya maselo ofiira a magazi. Chachikulu ndi congenital spherocytosis, chomwe chimatchedwa chifukwa cha mawonekedwe ozungulira omwe amadziwika ndi maselo ofiira a magazi ndipo amawapangitsa kukhala osalimba kwambiri. Zimakhala pafupipafupi: 1 mlandu mu 5000. Zolakwika zingapo za majini zimakhudzidwa, mawonekedwe apamwamba ndi autosomal amalamulira, koma mawonekedwe a recessive aliponso. Zitha kuyambitsa zovuta zina: ndulu, zilonda zam'miyendo.

Enzymopathies. Pali mitundu ingapo ya kuchepa kwa michere yomwe ingayambitse hemolytic anemia. Nthawi zambiri amatengera cholowa. Chodziwika kwambiri ndikusowa kwa puloteni yotchedwa "glucose-6-phosphate dehydrogenase", yomwe imayambitsa kuwonongeka msanga kwa maselo ofiira amwazi, kenako, hemolytic anemia.

Vuto la majini lomwe limakhudzidwa limalumikizidwa ndi X chromosome, motero, ndi amuna okha omwe angakhudzidwe. Azimayi amatha kunyamula vuto la majini ndi kupatsira ana awo. Kwa anthu omwe ali ndi vuto la enzyme iyi, kuchepa kwa magazi m'thupi la hemolytic kumachitika pambuyo pokumana ndi oxidizing agents.

Anthu omwe ali ndi vuto la G6PD amatha kukhala pachimake hemolysis akakumana ndi othandizira ena monga:

- kudya nyemba zamitundumitundu zotchedwa small-grain bean (addictive faba) kapena kukhudzana ndi mungu wochokera ku chomeracho (nyemba zamtunduwu zimagwiritsidwa ntchito podyetsa ziweto). Kulumikizana uku kumabweretsa pachimake hemolytic anemia yomwe imatchedwanso favism.

- kugwiritsa ntchito mankhwala ena: antimalarials, methyldopa (amachepetsa kuthamanga kwa magazi), sulfonamides (antibacterial), aspirin, non-steroidal anti-inflammatory drugs, quinidine, quinine, etc.

- kukhudzana ndi mankhwala ena monga mothballs.

- matenda ena.

Matendawa amapezeka kawirikawiri mwa anthu ochokera ku Mediterranean Basin (makamaka zilumba za Greek) komanso anthu akuda ku Africa ndi United States (kumene kufalikira kwake ndi 10% mpaka 14%). M’madera ena a dziko lapansi, 20 peresenti kapena kupitirirapo ya anthu ali nayo.

Hemoglobinopathies. Mawuwa amagwiritsidwa ntchito pofotokoza matenda obadwa nawo pomwe kupanga hemoglobin m'maselo ofiira am'magazi kumakhudzidwa. Sickle cell anemia (sickle cell anemia) ndi thalassemia ndi magulu awiri akuluakulu a hemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Matenda owopsa kwambiri ameneŵa amagwirizanitsidwa ndi kukhalapo kwa hemoglobini yachilendo yotchedwa hemoglobin S. Zimenezi zimasokoneza maselo ofiira a m’magazi ndi kuwapangitsa kukhala ngati kachigawo kakang’ono kapena kamkonyo (sickle cells), kuwonjezera pa kuwachititsa kufa. msanga. Onani tsamba la Sickle cell anemia.

Thalassemia. Chofala kwambiri m'mayiko ena a dziko lapansi, matenda aakuluwa amagwirizana ndi kusokonezeka kwa chibadwa komwe kumakhudza kupanga hemoglobini, pigment yamagazi m'maselo ofiira a magazi omwe amalola kunyamula mpweya kupita ku ziwalo. Maselo ofiira a m’magazi amene akhudzidwa ndi matendawa ndi osalimba ndipo amasweka msanga. Mawu akuti "thalassemia" amachokera ku mawu achi Greek akuti "thalassa", omwe amatanthauza "nyanja", monga momwe adawonekera koyamba mwa anthu ochokera kunyanja ya Mediterranean. Kuwonongeka kwa majini kumatha kukhudza malo awiri pakuphatikizika kwa hemoglobin: alpha chain kapena beta chain. Kutengera mtundu wa unyolo womwe wakhudzidwa, pali mitundu iwiri ya thalassemia: alpha-thalassemia ndi beta-thalassemia.

Zoyambitsa zina

Zimango zimayambitsa. Maselo ofiira amagazi amatha kuonongeka panthawi yamankhwala ena okhudzana ndi zida zamakina:

- ma prostheses (ma valve opangira mtima, etc.);

- kuyeretsa magazi a extracorporeal (hemodialysis);

- makina opangira okosijeni m'magazi (omwe amagwiritsidwa ntchito pa opaleshoni yamtima-mapapo), ndi zina zotero.

Nthawi zambiri, wothamanga wa marathon amatha kukhala ndi hemolysis chifukwa ma capillaries amapazi amaphwanyidwa mobwerezabwereza. Mkhalidwe umenewu wafotokozedwanso pambuyo pa magule ena amwambo aatali kwambiri, osavala mapazi.

Kuwonetsedwa ndi zinthu zapoizoni.

- Zida zamakampani kapena zapakhomo: aniline, arsenic hydrogen, nitrobenzene, naphthalene, paradichlorobenzene, etc.

- Nyama yapoizoni: kulumidwa ndi kangaude, kuluma kwa mavu, utsi wa njoka.

- Chomera chakupha: bowa wina.

Matenda.Kwambiri gastroenteritis chifukwa ndi coli, matenda oyambitsidwa ndi pneumococcus kapena staphylococcus, hepatitis, typhoid fever, malungo, ndi zina zotero. Malungo (kapena malungo) ndi omwe amachititsa kwambiri m'gululi. Malungo amayamba chifukwa cha tizilombo tomwe timamera m’maselo ofiira a magazi.

Hyperfunction ya ndulu. Ndi zachilendo kuti maselo ofiira a m'magazi awonongeke mu ndulu pambuyo pa ulendo wawo wa masiku 120, koma ngati chiwalochi chikugwira ntchito mopitirira muyeso, chiwonongekocho chimakhala chofulumira kwambiri ndipo hemolytic anemia imayamba.

Hhemoglobinuria paroxysmal usiku. Izi matenda amagwirizana ndi kukhalapo kwa hemoglobin mu mkodzo chifukwa kwambiri chiwonongeko cha maselo ofiira a magazi. Kukomoka usiku kumachitika chifukwa cha kupsinjika kwamtundu uliwonse, kukondoweza kwa chitetezo chamthupi kapena mankhwala ena. Nthawi zina matendawa amayambitsa kupweteka kwa msana komanso kusapeza bwino.

zotheka mavuto: thrombosis, m`mafupa hypoplasia, sekondale matenda.

• Zomwe zimagwirizanitsidwa ndi kuchepa kwa maselo ofiira a m'magazi: khungu lotuwa, kutopa, kufooka, chizungulire, kugunda kwa mtima, etc.
• Jaundice.
• Mkodzo wakuda.
• Kukula kwa ndulu.
• Zomwe zili zamtundu uliwonse wa anemia ya hemolytic. Onani "Kufotokozera Zachipatala".

Kwa kobadwa nako mitundu ya hemolytic anemia:

• Amene ali ndi mbiri ya banja.
• Anthu ochokera ku Mediterranean beseni, Africa, South ndi Southeast Asia ndi West Indies.

• Mwa anthu akusowa puloteni shuga-6-mankwala dehydrogenase: kukhudzana ndi oxidizing wothandizira (mankhwala ena, munda nyemba, etc.).
• Kwa mitundu ina ya hemolytic anemia:

  - Matenda ena: chiwindi, matenda a streptococcal kapena E. coli, matenda a autoimmune (monga lupus), chotupa cha m'chiberekero.

  - Mankhwala ena (antimalarials, penicillin, rifampicin, sulfonamides, etc.) kapena poizoni (aniline, arsenic hydrogen, etc.).

  - Zida zina zamakina zomwe zimagwiritsidwa ntchito pazamankhwala: mavavu opangira, zida zoyeretsera kapena kuthirira magazi.

  - Kupsinjika maganizo.

• Pakali pano, n’kosatheka kuletsa mitundu yotengera kubadwa kwa mwana kusiyapo kokha mwa kukaonana ndi mlangizi wa majini musanabereke mwana. Katswiriyo adzatha kudziwa kuopsa kobereka mwana yemwe ali ndi vuto la kuchepa kwa magazi m'thupi pamene (kapena onse awiri) mwa makolo omwe angakhalepo ali ndi mbiri ya banja (onaninso Sickle Cell Anemia kuti mudziwe zambiri za kuopsa kwa majini okhudzana ndi fomuyi. kuchepa kwa magazi m'thupi (hemolytic anemia).
• Ngati chinthu china chomwe chimayambitsa matendawa, chiyenera kupewedwa kuti tipewe kubwereza.
• Pamitundu yambiri ya kuchepa kwa magazi m'thupi, ndikofunikiranso kusamala ndi matenda ena.

Amasiyana malinga ndi mtundu wa hemolytic anemia.

• Chithandizo choyamba ndi chofunikira kwambiri potengera chithandizo chambiri kwa thupi komanso chifukwa chake ngati kuli kotheka
• Kuchuluka kwa folic acid kumasonyezedwa kwa odwala omwe ali ndi vuto la kuchepa kwa magazi m'thupi.
• Katemera wa matenda wamba ndi wofunikira kwa odwala omwe ali ndi vuto la chitetezo chamthupi, makamaka kwa anthu omwe ali ndi splenectomies (kuchotsa ndulu.⁶)
• Nthawi zina kuikidwa magazi kumasonyezedwa
• Splenectomy nthawi zina imaperekedwa⁷, makamaka kwa anthu omwe ali ndi cholowa cha spherocytosis, thalassemias omwe nthawi zambiri amafunika kuikidwa magazi komanso nthawi zina m'njira zina za kuchepa kwa magazi m'thupi. Zowonadi, makamaka mu ndulu ndi momwe maselo ofiira amawonongeka.
• Cortisone nthawi zina amaperekedwa kwa otentha antibody autoimmune anemia ndi kuganizira ozizira antibody anemia. Nthawi zina amagwiritsidwa ntchito paroxysmal nocturnal hemoglobinuria komanso makamaka thrombotic thrombocytopenic purpura. Mankhwala amphamvu a immunosuppressive, monga rituximab⁸, m'mitsempha ya immunoglobulins, azathioprine, cyclophosphamide, ndi cyclosporine angaganizidwe mu immunologic hemolytic anemias. Plasmapheresis nthawi zina imagwiritsidwa ntchito, makamaka pankhani ya thrombotic thrombocytopenic purpura.

Monga gawo lamachitidwe ake abwino, Passeportsanté.net ikukupemphani kuti mupeze malingaliro a akatswiri azaumoyo. Dr Dominic Larose, dokotala wazadzidzidzi, akukupatsani malingaliro ake pa kuchepa magazi m'thupi :

Thandizo lokhalo losavomerezeka lomwe limadziwika kuti sickle cell anemia. Onani tsamba ili kuti mumve zambiri.