Anaemia, site na nkọwa ya, gụnyere mbelata nke mkpụrụ ndụ ọbara uhie, ma ọ bụ ọkwa hemoglobin. Okwu a bụ "hemolytic anaemia" na-agụnye ụdị anaemia dị iche iche ebe a na-ebibi mkpụrụ ndụ ọbara uhie n'oge akaghi aka n'ime ọbara. Okwu a bụ "hemolysis" pụtara mbibi nke mkpụrụ ndụ ọbara uhie (hemo = ọbara; lysis = mbibi).

Ụmị ọkpụkpụ nwere ikike idobere ụfọdụ. Ya bụ, ọ nwere ike ịbawanye mmepụta nke mkpụrụ ndụ ọbara uhie ruo n'ókè ụfọdụ iji kwụọ ụgwọ mbibi ha na-abawanye. Dị ka ọ na-adịkarị, mkpụrụ ndụ ọbara uhie na-ekesa n'ime arịa ọbara ruo ihe dị ka ụbọchị 120. N'ọgwụgwụ nke ndụ ha, splin na imeju na-ebibi ha (leekwa akwụkwọ Anaemia - nkọwa). Nbibi ngwa ngwa nke mkpụrụ ndụ ọbara uhie bụ ihe mkpali dị mkpa na mmepụta nke mkpụrụ ndụ ọbara uhie ọhụrụ, nke a na-ebute site na hormone nke akụrụ na-emepụta, erythropoietin (EPO). N'ọnọdụ ụfọdụ, ụmị ọkpụkpụ na-enwe ike ịmịpụta ọtụtụ mkpụrụ ndụ ọbara uhie dị ka ọnụọgụ nke na-emebi emebi, yabụ ọkwa hemoglobin adịghị adalata. Anyị na-ekwu maka hemolysis akwụ ụgwọ, na-enweghị anaemia. Nke a dị mkpa n'ihi na e nwere ihe ụfọdụ nwere ike ime ka ọnọdụ ahụ gbanwee n'ime ihe ndị ga-egbochi mmepụta EPO dị ka afọ ime, ọdịda akụrụ, ụkọ folic acid, ma ọ bụ nnukwu ọrịa.

Eme

A na-ekewakarị anaemia hemolytic dabere ma ọ bụ mkpụrụ ndụ ọbara uhie kpatara ya bụ ihe na-adịghị mma (intracorpuscular), ma ọ bụ ihe dịpụrụ adịpụ na sel ọbara uhie (extracorpuscular). E mekwara ihe dị iche n'etiti anaemia hemolytic nke enwetara na nke enwetara.

Ihe nketa na intracorpuscular kpatara

• Hemoglobinopathies (dịka sickle cell anaemia, wdg)
• Enzymopathies (dịka ụkọ G6-PD)
• Membrane na cytoskeletal abormalities (dịka congenital spherocytosis)

Ihe nketa na extracorpuscular kpatara

• Ezinụlọ hemolytic-uremic syndrome (nke a na-ahụkarị)

Enwetara na intracorpuscular kpatara

• Paroxysmal nocturnal hemoglobinuria

Nweta na extracorpuscular kpatara

• Mechanical mbibi (microangiopathy)
• Ndị na-egbu egbu
• ọgwụ
• -efe efe
• Immunological

Ka anyị tụlee ihe atụ ole na ole mgbe ahụ, ebe ọ bụ na ọ gaghị ekwe omume ịkọwa ha nile n'ọnọdụ nke akwụkwọ a.

Ọrịa hemolytic anaemia:

Mmeghachi omume autoimmune. N'okwu a, ahụ, n'ihi ihe dị iche iche, na-emepụta ọgwụ mgbochi megide mkpụrụ ndụ ọbara uhie nke ya: ndị a na-akpọ autoantibodies. Enwere ụdị abụọ: ndị nwere autoantibodies na-ekpo ọkụ na ndị nwere oyi autoantibodies, dabere na okpomọkụ kachasị mma maka ọrụ mgbochi bụ 37 Celsius C ma ọ bụ 4 Celsius C. Nke a dị iche iche dị mkpa n'ihi na ọgwụgwọ dịgasị iche site n'ụdị ruo n'ụdị.

- autoantibodies na-ekpo ọkụ: na-emetụta ndị okenye ma na-ebute anaemia na-adịghị ala ala na mgbe ụfọdụ nnukwu hemolytic. Ha na-anọchi anya 80% nke autoimmune hemolytic anaemia. N'ime ọkara nke ikpe ahụ, enwere ike ịkpalite ha site na ọgwụ ụfọdụ (alpha-methyldopa, L-dopa) ma ọ bụ ọrịa ụfọdụ ( tumor ovarian, lymphoproliferative syndrome, wdg). A na-akpọ nke a dị ka anaemias autoimmune hemolytic "nke abụọ", ebe ọ bụ na ha na-apụta n'ihi ọrịa ọzọ.

– oyi auto-ọgwụ mgbochi ọrịa: na-ejikọta ya na nnukwu ngosipụta nke mbibi nke mkpụrụ ndụ ọbara uhie nke oyi na-akpata. N'ime 30% nke ikpe, anyị na-emeso mmeghachi omume autoimmune nke abụọ nke enwere ike ịkọwa ya site na nje virus ma ọ bụ mycoplasma, microorganism intermediate n'etiti nje na nje bacteria.

Mmeghachi omume immunoallergic. N'ihe banyere hemolysis ọgwụ immunoallergic (na-abụghị autoimmune), ọgwụ mgbochi ọrịa anaghị alụso mkpụrụ ndụ ọbara uhie ọgụ, mana ụfọdụ ọgwụ: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, wdg.

Ọrịa hemolytic anaemia na-ebute:

Enwere ihe atọ dị mkpa na mkpụrụ ndụ ọbara uhie. E nwere hemoglobin, akpụkpọ ahụ-cytoskeleton mgbagwoju, na enzymatic "igwe" na-eme ka ọ niile na-arụ ọrụ. Ọdịiche nke mkpụrụ ndụ ihe nketa na nke ọ bụla n'ime ihe atọ a nwere ike ibute anaemia hemolytic.

Ihe nketa nketa nke akpụkpọ ahụ nke mkpụrụ ndụ ọbara uhie. Nke bụ isi bụ congenital spherocytosis, nke a na-akpọ ya n'ihi ọdịdị okirikiri nke na-akọwapụta mkpụrụ ndụ ọbara uhie na nke na-eme ka ha na-esighi ike. Ọ na-adịtụ ugboro ugboro: 1 ikpe na 5000. Ọtụtụ mkpụrụ ndụ ihe nketa abnormalities na-agụnye, kpochapụwo ụdị bụ autosomal achị, ma recessive iche-dịkwa. Ọ nwere ike ịkpata nsogbu ụfọdụ: nkume gallstone, ọnya na ụkwụ.

Enzymopathies. Enwere ọtụtụ ụdị ụkọ enzyme nke nwere ike ibute anaemia hemolytic. Ha na-abụkarị ihe nketa. Ihe na-emekarị bụ ụkọ nke enzyme a na-akpọ "glucose-6-phosphate dehydrogenase", nke na-ebute mbibi nke mkpụrụ ndụ ọbara uhie n'oge na-adịghị anya na, emesịa, anaemia hemolytic.

A na-ejikọta ntụpọ mkpụrụ ndụ ihe nketa nke metụtara X chromosome, ya mere, ọ bụ naanị ụmụ nwoke nwere ike imetụta. Ụmụ nwanyị nwere ike iburu ntụpọ mkpụrụ ndụ ihe nketa ma nyefee ya ụmụ ha. N'ime ndị nwere ụkọ enzyme a, anaemia hemolytic na-emekarị mgbe ikpughe na ihe ndị na-egbu egbu.

Ndị nwere ụkọ G6PD nwere ike ịmalite hemolysis buru ibu mgbe ekpughere ya na ụfọdụ ndị ọrụ dịka:

- oriri nke ụdị agwa dị iche iche a na-akpọ obere ọka (obere ọka).faba riri ahụ) ma ọ bụ ikpughe pollen sitere na osisi ahụ (a na-eji ụdị agwa dị iche iche eme nri anụ ụlọ). Mmekọrịta a na-ebute nnukwu anaemia hemolytic nke a na-akpọ favism.

- iji ọgwụ ụfọdụ: antimalarials, methyldopa (na-ebelata ọbara mgbali), sulfonamides (antibacterial), aspirin, ọgwụ mgbochi mkpali na-abụghị steroidal, quinidine, quinine, wdg.

– ikpughe ụfọdụ kemịkalụ dị ka mothball.

– ụfọdụ ọrịa.

A na-achọpụtakarị ọrịa a na ndị si n'Oké Osimiri Mediterenian (karịsịa Agwaetiti Greek) yana ndị ojii nọ n'Africa na United States (ebe ọ bụ 10% ruo 14%). N'akụkụ ụfọdụ nke ụwa, 20% ma ọ bụ karịa nke ndị bi na ya nwere ya.

Hemoglobinopathies. Okwu eji kọwaa ọrịa mkpụrụ ndụ ihe nketa ebe a na-emetụta mmepụta hemoglobin n'ime mkpụrụ ndụ ọbara uhie. Sickle cell anaemia (sickle cell anaemia) na thalassemia bụ akụkụ abụọ bụ isi nke hemoglobinopathies.

Sickle cell anaemia (sickle cell anaemia)^4,5. Ọrịa a dị oke njọ jikọtara ya na ọnụnọ hemoglobin na-adịghị mma nke a na-akpọ hemoglobin S. Nke a na-agbagọ mkpụrụ ndụ ọbara uhie ma na-enye ha ọdịdị nke ọnwa ma ọ bụ scythe (sickle cell), na mgbakwunye na ime ka ha nwụọ. oge akaghi aka. Lee mpempe akwụkwọ Sickle cell anaemia.

Thalassaemia. Gburugburu ebe niile na mba ụfọdụ nke ụwa, ọrịa a siri ike jikọtara ya na mkpụrụ ndụ ihe nketa na-emetụta mmepụta hemoglobin, ụcha ọbara a na mkpụrụ ndụ ọbara uhie nke na-enye ohere ibuga oxygen na akụkụ ahụ. Mkpụrụ ndụ ọbara uhie nke metụtara na-esighi ike ma gbarie ngwa ngwa. Okwu a bu "thalassemia" sitere na okwu Grik "thalassa", nke putara "osimiri", dika eweputara ya na mbu na ndi mmadu sitere na basin Mediterranean. Nrụrụ mkpụrụ ndụ ihe nketa nwere ike imetụta ebe abụọ na njikọ nke hemoglobin: agbụ alfa ma ọ bụ agbụ beta. Dabere n'ụdị yinye emetụtara, enwere ụdị abụọ nke thalassemia: alfa-thalassemia na beta-thalassemia.

Ihe ndị ọzọ na-akpata ya

Mechanical ihe kpatara ya. Enwere ike imebi mkpụrụ ndụ ọbara uhie n'oge ọgwụgwọ ụfọdụ metụtara ngwaọrụ arụ ọrụ:

- prostheses (valvụ arụ ọrụ maka obi, wdg);

- extracorporeal ọbara ọcha (hemodialysis);

- igwe na-eme ka ikuku oxygen na-eme ka ọbara (nke a na-eji n'ịwa ahụ obi-akpa ume), wdg.

Ọ dị obere, onye na-agba ọsọ marathon nwere ike ịnweta hemolysis n'ibu dịka a na-etipịa capillaries n'ụkwụ ya ugboro ugboro. A kọwawokwa ọnọdụ a mgbe ụfọdụ ịgba egwu okpukpe dị ogologo ogologo oge, n'ụkwụ efu.

Ikpughe ihe ndị na-egbu egbu.

- Ngwaahịa ụlọ ọrụ ma ọ bụ ihe na-egbu egbu: aniline, hydrogen arsenic, nitrobenzene, naphthalene, paradichlorobenzene, wdg.

– Anụ na-egbu egbu: ududo, nsị, nsị agwọ.

– Osisi nsi: ụfọdụ fungi.

Ọrịa.Ọrịa gastroenteritis siri ike kpatara Na coli, ọrịa na-ebute site na pneumococcus ma ọ bụ staphylococcus, ịba ọcha n'anya, typhoid fever, ịba, wdg. ịba (ma ọ bụ ịba) bụ ihe kacha akpata na ngalaba a. Ọrịa ịba bụ nje nje na-eto n'ime mkpụrụ ndụ ọbara uhie na-akpata.

Hyperfunction nke splin. Ọ bụ ihe kwesịrị ekwesị ka ebibie mkpụrụ ndụ ọbara uhie na splin ka ha gachara njem ụbọchị 120, mana ọ bụrụ na akụkụ ahụ na-arụ ọrụ nke ukwuu, mbibi ahụ na-adị ngwa ngwa, anaemia hemolytic na-amalite.

Hhemoglobinuria paroxysmal nocturnal. A na-ejikọta ọrịa a na-adịghị ala ala na ọnụnọ hemoglobin na mmamịrị n'ihi oke mbibi nke mkpụrụ ndụ ọbara uhie. Ihe ọdịdọ nke abalị na-ebute site n'ụdị nrụgide ọ bụla, mkpali usoro ahụ ji alụso ọrịa ọgụ ma ọ bụ ọgwụ ụfọdụ. Mgbe ụfọdụ, ọrịa ahụ na-ebute mgbu azụ na ahụ erughị ala.

Nsogbu ndị nwere ike ime: thrombosis, ụmị ọkpụkpụ hypoplasia, ọrịa nke abụọ.

• Ndị jikọtara ya na obere ọbara ọbara uhie: agba agba agba, ike ọgwụgwụ, adịghị ike, isi ọwụwa, obi mgbawa ngwa ngwa, wdg.
• Jaundice.
• Miri mamiri.
• Ịbawanye splin.
• Ndị ahụ akọwapụtara maka ụdị ọ bụla nke anaemia hemolytic. Hụ "Nkọwa ahụike".

Maka usoro ọmụmụ nke anaemia hemolytic:

• Ndị nwere akụkọ ezinụlọ.
• Ndị si n'osimiri Mediterranean, Africa, South na Southeast Asia na West Indies.

• N'ime ndị nwere ụkọ nke enzyme glucose-6-phosphate dehydrogenase: ikpughe na ndị na-eme ihe na-egbu egbu (ụfọdụ ọgwụ, ahịhịa ubi, wdg).
• Maka ụdị ndị ọzọ nke anaemia hemolytic:

  - Ọrịa ụfọdụ: ịba ọcha n'anya, ọrịa streptococcal ma ọ bụ E. coli, ọrịa autoimmune (dị ka lupus), akpụ ovarian.

  - Ụfọdụ ọgwụ (antimalarials, penicillin, rifampicin, sulfonamides, wdg) ma ọ bụ ihe na-egbu egbu (aniline, arsenic hydrogen, wdg).

  - Ụfọdụ ngwaọrụ eji arụ ọrụ na ọgwụ: valves artificial, ngwaọrụ maka ime ka ọ dị ọcha ma ọ bụ ikuku oxygen.

  - Nchegbu.

• Ka ọ dị ugbu a, ọ gaghị ekwe omume igbochi ụdị ihe nketa ma e wezụga site n'ịgakwuru onye ndụmọdụ gbasara mkpụrụ ndụ ihe nketa tupu a mụọ nwa. Ọkachamara ahụ ga-enwe ike ịchọpụta ihe egwu dị n'ịmụ nwa nwere anaemia hemolytic ma ọ bụ (ma ọ bụ abụọ) nke nne na nna nwere ike nwere akụkọ ihe mere eme ezinụlọ (leekwa Sickle Cell Anemia maka nkọwa ndị ọzọ gbasara ihe egwu mkpụrụ ndụ ihe nketa gbasara ụdị a. ọrịa anaemia hemolytic).
• Ọ bụrụ na otu ihe kpatara ọrịa ahụ, a ga-ezere ya iji gbochie nlọghachi azụ.
• Maka ọtụtụ ụdị anaemia hemolytic, ọ dịkwa mkpa ịchebe ọrịa ụfọdụ.

Ha dịgasị iche dabere n'ụdị anaemia hemolytic.

• Ọgwụgwọ bụ nke mbụ dabere na nkwado zuru oke maka ahụ yana ihe kpatara ya mgbe enwere ike
• A na-egosikarị mgbakwunye folic acid maka ndị ọrịa nwere anaemia hemolytic na-adịghị ala ala.
• Ịgba ọgwụ mgbochi ọrịa na-efe efe dị mkpa maka ndị ọrịa ahụ nwere ihe nchebe nchebe, karịsịa na ndị nwere splenectomies (iwepụ splin.⁶)
• A na-egosi mmịnye ọbara mgbe ụfọdụ
• A na-atụ aro splenectomy mgbe ụfọdụ⁷, karịsịa na ndị nwere spherocytosis eketa, thalassemias nke na-achọkarị mmịnye ọbara kamakwa mgbe ụfọdụ n'ụdị ndị ọzọ nke anaemia hemolytic na-adịghị ala ala. N'ezie, ọ bụ n'ụba ka a na-ebibi mkpụrụ ndụ ọbara uhie.
• A na-enye Cortisone mgbe ụfọdụ maka anaemia autoimmune antibody na-ekpo ọkụ yana ịtụle maka anaemia mgbochi oyi. A na-eji ya mgbe ụfọdụ n'ihe gbasara haemoglobinuria nocturnal paroxysmal na karịsịa maka thrombocytopenic purpura thrombotic. Ihe mgbochi immunosuppressive siri ike, dị ka rituximab⁸, Immunoglobulin intravenous, azathioprine, cyclophosphamide, na cyclosporine nwere ike na-atụle na immunologic hemolytic anaemias. A na-eji Plasmapheresis eme ihe mgbe ụfọdụ, ọkachasị n'ihe gbasara thrombocytopenic purpura a.

Dịka akụkụ nke usoro ịdị mma ya, Passeportsanté.net na -akpọ gị oku ka ị chọpụta echiche nke ọkachamara ahụike. Dr Dominic Larose, dibịa mberede, na -enye gị echiche ya na hemolytic anaemia :

Naanị ọgwụgwọ a na-adịghị ahụkebe achọpụtara gbasara ọrịa sickle cell anaemia. Lee mpempe akwụkwọ a maka nkọwa ndị ọzọ.