ʻO ka anemia, ma ka wehewehe ʻana, pili i ka emi ʻana o nā keʻena koko ʻulaʻula, a i ʻole ka pae hemoglobin. ʻO ka huaʻōlelo "hemolytic anemia" e pili ana i nā ʻano anemia like ʻole kahi i luku ʻia ai nā ʻulaʻula ʻulaʻula i loko o ke koko. ʻO ka huaʻōlelo "hemolysis" ʻo ia ka luku ʻana i nā ʻulaʻula ʻulaʻula (hemo = koko; lysis = luku).

Loaʻa i ka ʻiʻo iwi kekahi mana mālama. ʻO ia hoʻi, hiki iā ia ke hoʻonui i kāna hana ʻana i nā ʻulaʻula ʻulaʻula i kahi pae e uku ai i kā lākou luku nui ʻana. ʻO ka maʻamau, kaʻapuni nā ʻulaʻula i loko o nā kīʻaha koko no kahi o 120 mau lā. I ka pau ʻana o ko lākou ola ʻana, luku ʻia lākou e ka spleen a me ke akepaʻa (e ʻike pū i ka pepa Anemia - ʻike nui). ʻO ka luku ʻia ʻana o nā keʻena koko ʻulaʻula he mea hoʻoikaika nui ia i ka hana ʻana o nā keʻena koko ʻulaʻula hou, i hoʻopili ʻia e kahi hormone i hana ʻia e nā puʻupaʻa, erythropoietin (EPO). I kekahi mau hihia, hiki i ka iwi iwi ke hoʻohua i ka nui o ke koko ʻulaʻula e like me ka nui i luku ʻia, no laila ʻaʻole e hāʻule ka pae hemoglobin. Ke kamaʻilio nei mākou e pili ana i ka hemolysis uku, me ka ʻole o ka anemia. He mea koʻikoʻi kēia no ka mea aia kekahi mau mea e hiki ai ke hoʻoheheʻe ʻia ke kūlana i nā mea e keʻakeʻa ai i ka hana EPO e like me ka hāpai ʻana, ka hāʻule ʻana o ke kīkī, ka nele o ka waikawa folic, a i ʻole ka maʻi ʻeha.

Nā kumu

Hoʻokaʻawale ʻia ka anemia hemolytic e like me ke kumu o ke koko ʻulaʻula ʻulaʻula nona iho (intracorpuscular), a i ʻole kahi mea i waho o ke koko ʻulaʻula (extracorpuscular). Hoʻokaʻawale ʻia hoʻi ma waena o ka hereditary a me ka anemia hemolytic i loaʻa.

Nā kumu hoʻoilina a me intracorpuscular

• Hemoglobinopathies (e like me sickle cell anemia, etc.)
• Enzymopathies (e laʻa me ka hemahema o G6-PD)
• Nā mea ʻino o ka membrane a me ka cytoskeletal (e laʻa me ka congenital spherocytosis)

Ke kumu hoʻoilina a extracorpuscular

• ʻO ka maʻi hemolytic-uremic ʻohana (atypical)

Loaʻa a intracorpuscular kumu

• ʻO ka hemoglobinuria paroxysmal nocturnal

Loaʻa a extracorpuscular kumu

• Ka luku ʻana i ka mīkini (microangiopathy)
• Nā mea ʻawaʻawa
• nā lāʻauʻona
• Infections
• Immunological

E kūkākūkā kākou i kekahi mau laʻana, no ka mea, ʻaʻole hiki ke wehewehe i nā mea a pau i loko o ka pōʻaiapili o kēia palapala.

ʻO ka anemia hemolytic immunological:

Nā hopena autoimmune. I kēia hihia, ke kino, no nā kumu like ʻole, hoʻopuka i nā antibodies e kūʻē i kona mau koko ʻulaʻula ponoʻī: ua kapa ʻia kēia mau autoantibodies. ʻElua ʻano: ʻo ka poʻe me nā autoantibodies wela a me nā autoantibodies anu, e hilinaʻi ana inā ʻo 37 ° C a i ʻole 4 ° C ka mahana kūpono no ka hana antibody. He mea nui kēia ʻokoʻa no ka mea ʻokoʻa ka mālama ʻana mai ke ʻano a i ke ʻano.

- ʻO nā autoantibodies wela: pili nui i nā pākeke a hoʻoulu i ka anemia hemolytic koʻikoʻi a i kekahi manawa. Hōʻike lākou i ka 80% o nā anemia hemolytic autoimmune. I ka hapalua o nā hihia, hiki ke hoʻoulu ʻia e kekahi mau lāʻau (alpha-methyldopa, L-dopa) a i ʻole kekahi mau maʻi (ovarian tumor, lymphoproliferative syndrome, etc.). Ua kapa ʻia kēia ma ke ʻano he "secondary" autoimmune hemolytic anemias, ʻoiai ua ʻike ʻia lākou ma muli o kahi maʻi ʻē aʻe.

- Cold auto-antibodies: pili pū me nā ʻāpana koʻikoʻi o ka luku ʻana i nā keʻena koko ʻulaʻula ma muli o ke anu. Ma 30% o nā hihia, ke hana nei mākou i kahi hopena autoimmune lua i hiki ke wehewehe ʻia e kahi maʻi viral a i ʻole mycoplasma, kahi microorganism waena ma waena o nā maʻi a me nā maʻi bacteria.

Nā hopena immunoallergic. I ka hihia o ka hemolysis lāʻau immunoallergic (non-autoimmune), ʻaʻole hoʻouka nā antibodies i nā ʻulaʻula ʻulaʻula, akā kekahi mau lāʻau lapaʻau: penicillin, cefalotin, cephalosporins, rifampicin, phenacetin, quinine, etc.

ʻO ka anemia hemolytic congenital:

ʻEkolu mau mea pono i loko o ke koko ʻulaʻula. Aia ka hemoglobin, ka membrane-cytoskeleton complex, a me ka "mīkini" enzymatic e hana i nā mea a pau. Hiki ke hoʻoulu ʻia ka maʻi anemia hemolytic i nā ʻano like ʻole o kēia mau mea ʻekolu.

ʻO nā maʻi kūlohelohe o ka membrane o ke koko ʻulaʻula. ʻO ka mea nui, ʻo ia ka congenital spherocytosis, i kapa ʻia ma muli o ke ʻano spherical e hōʻike ana i nā ʻāpana koko ʻulaʻula a ʻo ia ka mea e palupalu loa ai. He mea maʻamau ia: 1 hihia ma 5000. Ua pili kekahi mau genetic abnormalities, ʻo ke ʻano maʻamau he autosomal dominate, akā aia nā ʻano recessive. Hiki iā ia ke hoʻopilikia i kekahi mau pilikia: gallstones, ʻeha ma nā wāwae.

Nā Enzymopathies. Nui nā ʻano hemahema o ka enzyme e hiki ai ke hoʻoulu i ka anemia hemolytic. He hoʻoilina maʻamau lākou. ʻO ka mea maʻamau he hemahema i loko o kahi enzyme i kapa ʻia ʻo "glucose-6-phosphate dehydrogenase", ka mea e hoʻopau koke ai i nā keʻena koko ʻulaʻula a, ma hope, hemolytic anemia.

Hoʻopili ʻia ka hemahema genetic i ka chromosome X, no laila, hiki i nā kāne ke hoʻopilikia. Hiki i nā wāhine ke lawe i ke kīnā genetic a hoʻoili i kā lākou mau keiki. I loko o ka poʻe me kēia hemahema enzyme, ʻike pinepine ʻia ka anemia hemolytic ma hope o ka ʻike ʻana i nā mea hoʻoneʻe.

Hiki i ka poʻe me ka G6PD deficiency ke hoʻomohala i ka hemolysis acute ke ʻike ʻia i kekahi mau mea e like me:

- ka ʻai ʻana i nā ʻano pīni i kapa ʻia ʻo small-grain bean (addictive faba) a i ʻole ka ʻike ʻana i ka pollen mai kēlā meakanu (ua hoʻohana ʻia kēia ʻano pīni no ka hānai holoholona). ʻO kēia ka hopena i ka anemia hemolytic acute i kapa ʻia ʻo favism.

- ka hoʻohana ʻana i kekahi mau lāʻau: antimalarials, methyldopa (hoʻohaʻahaʻa i ke koko), sulfonamides (antibacterials), aspirin, non-steroidal anti-inflammatory drugs, quinidine, quinine, etc.

- ka ʻike ʻana i kekahi mau mea kemika e like me ka mothballs.

- kekahi mau maʻi.

ʻIke pinepine ʻia kēia maʻi i nā poʻe mai ka Mediterranean Basin (ʻo ia hoʻi nā mokupuni Helene) a me nā poʻe ʻeleʻele ma ʻApelika a me ʻAmelika Hui Pū ʻIa (kahi o 10% a 14%). Ma kekahi mau wahi o ka honua, 20% a oi aku paha o ka heluna kanaka.

Hemoglobinopathies. ʻO ka huaʻōlelo i hoʻohana ʻia no ka wehewehe ʻana i nā maʻi genetic kahi e hoʻopili ʻia ai ka hana ʻana o ka hemoglobin i loko o ke koko ʻulaʻula. ʻO ka maʻi maʻi maʻi maʻi (sickle cell anemia) a me thalassemia nā ʻāpana nui ʻelua o ka hemoglobinopathies.

Sickle cell anemia (sickle cell anemia)^4,5. Hoʻopili ʻia kēia maʻi koʻikoʻi me ka loaʻa ʻana o kahi hemoglobin ʻanoʻole i kapa ʻia ʻo hemoglobin S. Hoʻololi kēia i nā keʻena koko ʻulaʻula a hāʻawi iā lākou i ke ʻano o ka crescent a i ʻole ka scythe (sickle cell), ma waho aʻe o ka make ʻana iā lākou. ma mua o ka manawa. E ʻike i ka pepa Sickle cell anemia.

Kahalaima. Laha loa i kekahi mau ʻāina o ka honua, pili kēia maʻi koʻikoʻi i kahi maʻi ʻino e pili ana i ka hana ʻana o ka hemoglobin, kēia puaʻa koko i loko o nā ʻulaʻula ʻulaʻula e hiki ai ke lawe i ka oxygen i nā kino. ʻO nā keʻena koko ʻulaʻula i hoʻopilikia ʻia he palupalu a haki koke. ʻO ka huaʻōlelo "thalassemia" mai ka huaʻōlelo Helene "thalassa", ʻo ia hoʻi ka "kai", e like me ka mea i ʻike mua ʻia i nā poʻe mai ke kai Mediterranean. Hiki ke hoʻopilikia i ka genetic defect i nā wahi ʻelua i ka synthesis o ka hemoglobin: ke kaulahao alpha a i ʻole ke kaulahao beta. Ma muli o ke ʻano o ke kaulahao i hoʻopili ʻia, ʻelua ʻano o ka thalassemia: alpha-thalassemia a me beta-thalassemia.

ʻO nā kumu ʻē aʻe

Nā kumu mīkini. Hiki ke hōʻino ʻia ke koko ʻulaʻula i ka wā o kekahi mau lāʻau lapaʻau e pili ana i nā mea mīkini:

- nā prostheses (nā kiwi hana no ka puʻuwai, a me nā mea ʻē aʻe);

- hoʻomaʻemaʻe koko extracorporeal (hemodialysis);

- mīkini e oxygenate i ke koko (hoʻohana ʻia i ka ʻoki ʻana i ka naʻau-māmā), etc.

ʻAʻole hiki i ka mea holo marathon ke ʻike i ka hemolysis mechanical i ka wā e ʻeha pinepine ʻia nā capillaries i nā wāwae. Ua wehewehe ʻia kēia kūlana ma hope o kekahi mau hula hoʻomana lōʻihi loa, ma nā wāwae wāwae ʻole.

Ka ʻike ʻana i nā mea ʻawaʻawa.

- Nā huahana ʻona a i ʻole ka home: aniline, arsenic hydrogen, nitrobenzene, naphthalene, paradichlorobenzene, etc.

- Holoholona ʻona: nahu ʻia ka spider, ka nahesa, ka nahesa.

- Mea kanu ʻona: kekahi mau haʻi.

Hoʻopilikia.ʻO ka gastroenteritis koʻikoʻi ma muli o A me ka coli, nā maʻi i hoʻokumu ʻia e ka pneumococcus a i ʻole staphylococcus, hepatitis, typhoid fever, malaria, etc. ʻO ka maʻi maʻi (a i ʻole ka maʻi maʻi) ke kumu nui o kēia māhele. Hoʻokumu ʻia ka maʻi maʻi e kahi parasite e ulu ana i loko o ke koko ʻulaʻula.

Hyperfunction o ka spleen. He mea maʻamau ka luku ʻia ʻana o ke koko ʻulaʻula i loko o ka spleen ma hope o ko lākou huakaʻi 120 lā, akā inā hana nui kēia okana, wikiwiki loa ka luku ʻana a hiki mai ka anemia hemolytic.

Hʻemoglobinuria paroxysmal nocturnal. Hoʻopili ʻia kēia maʻi maʻi me ka loaʻa ʻana o ka hemoglobin i loko o ka mimi ma muli o ka luku nui ʻana o nā ʻulaʻula ʻulaʻula. Hoʻokumu ʻia ka hopu ʻana i ka pō e kekahi ʻano o ke koʻikoʻi, ka hoʻoulu ʻana i ka ʻōnaehana immune a i ʻole kekahi mau lāʻau lapaʻau. I kekahi manawa, hoʻoulu ka maʻi i ka ʻeha hope a me ka ʻeha.

Hiki i nā pilikia: thrombosis, iwi iwi hypoplasia, maʻi lua.

• ʻO nā mea i hoʻopili ʻia i kahi pae haʻahaʻa o nā keʻena koko ʻulaʻula: ʻeleʻele ka ʻili, ka luhi, ka nāwaliwali, ka dizziness, ka puʻuwai wikiwiki, etc.
• Jaundice
• Mimi ʻeleʻele.
• Ka hoʻonui ʻana o ka ʻāʻī.
• ʻO nā mea kiko'ī i kēlā me kēiaʻano o ka anemia hemolytic. E nānā i ka "Ho'ākāka Lapaʻau".

No nā ʻano congenital o ka anemia hemolytic:

• Ka poe me ka moolelo ohana.
• ʻO nā kānaka mai ke kai Mediterranean, ʻApelika, Hema a me Asia Hema a me West Indies.

• I ka poʻe me ka hemahema o ka enzyme glucose-6-phosphate dehydrogenase: ka ʻike ʻana i nā mea oxidizing (kekahi mau lāʻau lapaʻau, field bean, etc.).
• No nā ʻano ʻē aʻe o ka anemia hemolytic:

  - ʻO kekahi mau maʻi: he maʻi hepatitis, streptococcal maʻi a i ʻole E. coli, nā maʻi autoimmune (e like me ka lupus), ka maʻi ovarian.

  - Kekahi mau lāʻau (antimalarials, penicillin, rifampicin, sulfonamides, etc.) a i ʻole nā ​​mea ʻawaʻawa (aniline, arsenic hydrogen, etc.).

  - ʻO kekahi mau mea mīkini i hoʻohana ʻia i ka lāʻau lapaʻau: nā kiwi hana, nā mea hana no ka hoʻomaʻemaʻe a i ʻole ka oxygenating i ke koko.

  — Ke kaumaha.

• I kēia manawa, ʻaʻole hiki ke pale i nā ʻano hoʻoilina ke ʻole ma ke kūkākūkā ʻana i kahi kākā'ōlelo genetic ma mua o ka hāpai ʻana i kahi keiki. Hiki i ka loea ke hoʻoholo i nā pilikia o ka hānau ʻana i kahi keiki me ka anemia hemolytic inā loaʻa kekahi (a i ʻole ʻelua) o nā mākua i hiki ke loaʻa ka moʻolelo ʻohana (e ʻike pū i ka Sickle Cell Anemia no nā kikoʻī hou aku e pili ana i nā pilikia genetic e pili ana i kēia palapala. o ka anemia hemolytic).
• Inā he mea kiko'ī ke kuleana o ka maʻi, pono e paleʻia e pale i ka hoʻi hou.
• No nā ʻano maʻi hemolytic he nui, he mea nui nō hoʻi e kiaʻi i kekahi mau maʻi.

Hoʻololi lākou ma muli o ke ʻano o ka anemia hemolytic.

• Hoʻokumu mua ʻia ka mālama ʻana ma muli o ke kākoʻo maʻamau i ke kino a me ke kumu kumu ke hiki
• Hōʻike pinepine ʻia kahi mea hoʻohui o ka folic acid no nā poʻe maʻi me ka anemia hemolytic mau loa.
• He mea koʻikoʻi ka hoʻopaʻa ʻana i nā maʻi maʻamau no kēlā poʻe maʻi i hōʻemi i ka pale ʻana i ka pale ʻana, ʻoi aku hoʻi i nā poʻe me ka splenectomies (ka wehe ʻana i ka spleen.⁶)
• Hōʻike ʻia ke kahe koko i kekahi manawa
• Manaʻo ʻia ʻo Splenectomy i kekahi manawa⁷, ʻoi aku ka nui o nā poʻe me ka hereditary spherocytosis, thalassemias e koi pinepine ai i ka hoʻololi ʻana akā i kekahi manawa ma nā ʻano ʻē aʻe o ka anemia hemolytic mau loa. ʻOiaʻiʻo, ʻo ka hapa nui o ka spleen e luku ʻia ai nā ʻulaʻula.
• Hoʻonohonoho ʻia ʻo Cortisone i kekahi manawa no ka anemia autoimmune antibody wela a no ka noʻonoʻo ʻana no ka anemia antibody anu. Hoʻohana ʻia ia i kekahi manawa i nā hihia o ka hemoglobinuria nocturnal paroxysmal a ma ke ʻano no ka thrombotic thrombocytopenic purpura. ʻO nā mea hana immunosuppressive ikaika, e like me rituximab⁸, hiki ke noʻonoʻo ʻia nā immunoglobulins intravenous, azathioprine, cyclophosphamide, a me cyclosporine i ka immunologic hemolytic anemias. Hoʻohana ʻia ka Plasmapheresis i kekahi manawa, i ka hihia o kēia thrombotic thrombocytopenic purpura.

Ma ke ʻano he ʻano kūpono, hoʻokipa aku ʻo Passeportsanté.net iā ʻoe e ʻike i ka manaʻo o kahi ʻoihana olakino. Hāʻawi ʻo Dr Dominic Larose iā ʻoe i kona manaʻo no ka anemia hemolytic :

ʻO nā lāʻau lapaʻau maʻamau wale nō i ʻike ʻia e pili ana i ka maʻi sickle cell anemia. E ʻike i kēia pepa no nā kikoʻī hou aku.