ʻO Paroxysmal nocturnal hemoglobinuria: he aha ia a pehea e hana ai ka hōʻoia

Anter

• Nā hōʻailona nui
• Pehea e hana ʻia ai ke kuhi
• Pehea e mālama ai

ʻO Paroxysmal nocturnal hemoglobinuria, ʻike ʻia ʻo PNH, kahi maʻi laha ʻole o ke kumu kūpuna, ʻano ʻia e nā loli i ka membrane o nā hunaola ʻulaʻula, e alakaʻi ana i kona luku ʻia ʻana a me ka hoʻopau ʻana i nā ʻāpana o nā hunaola ʻulaʻula i ka mimi, a no laila ua manaʻo ʻia he hemolytic mau. anemia

ʻO ka huaʻōlelo nocturne e pili ana i ka wā o ka lā ke nānā ʻia ka helu kiʻekiʻe loa o ka luku ʻia o ke koko ʻulaʻula i ka poʻe me kēia maʻi, akā ua hōʻike ʻia nā noiʻi ʻana he hemolysis, ʻo ia hoʻi ka luku ʻia ʻana o nā hunaola ʻulaʻula, i kēlā me kēia manawa o ka lā i nā kānaka i loaʻa i ka maʻi. hemoglobinuria.

ʻAʻohe lāʻau o PNH, akā hiki ke hana i ka lapaʻau ma o ka hoʻoneʻe ʻana i ka iwi iwi a me ka hoʻohana ʻana iā Eculizumab, ʻo ia ka lāʻau lapaʻau kikoʻī no ka mālama ʻana i kēia maʻi. E aʻo hou aʻe e pili ana iā Eculizumab.

Nā hōʻailona nui

ʻO nā ʻōuli nui o nocturnal paroxysmal hemoglobinuria:

• ʻO ka mimi ʻeleʻele ʻeleʻele loa, ma muli o ke kiʻekiʻe o ka nui o nā hunaola ʻulaʻula i ka mimi;
• Nāwaliwali;
• Somnolence;
• Lauoho nawaliwali a me nā kui;
• Lohi;
• ʻEha ʻeha;
• Nā maʻi pinepine;
• Manaʻo maʻi;
• Abdominal eha;
• ʻO Jaundice;
• ʻO ke kīnā ʻole o erectile kāne;
• Hoemi i ka hana hakuʻala.

ʻO ka poʻe me ka hemoglobinuria o ka paroxysmal nocturnal i loaʻa ka manawa kūpono o ka thrombosis ma muli o nā loli i ke kaʻina o ke kō ʻana o ke koko.

Pehea e hana ʻia ai ke kuhi

Hoʻomaopopo ʻia ke kuhi o nocturnal paroxysmal hemoglobinuria ma o kekahi mau hoʻokolohua, e like me:

• Heluhelu koko, i ka poʻe me PNH, hōʻike ʻia ka pancytopenia, i kūlike i ka hoʻoliʻiliʻi o nā ʻāpana koko āpau - ʻike pehea e wehewehe ai i ka helu o ke koko;
• Nā hana o bilirubin manuahi, ka mea i hoʻonui ʻia;
• Ka ʻike a me ka hana ʻana, ma o ka cytometry kahe, o ka Nā antigens CD55 a me CD59, ʻo ia nā protein i loko o ka membrane o nā hunaola ʻulaʻula a, i ke kumu o hemoglobinuria, e hoʻemi ʻia a ʻaʻole paha.

Ma waho aʻe o kēia mau hoʻāʻo, hiki i ka hematologist ke noi i nā hoʻokolohua kōkua, e like me ka hōʻike sukrosa a me ka hōʻike HAM, i kōkua i ke kuhi ʻana i ka hemoglobinuria o ka paroxysmal nocturnal. ʻO ka maʻamau maʻamau ka laha ma waena o 40 a me 50 mau makahiki a ʻo ke ola o ke kanaka ma kahi o 10 a 15 mau makahiki.

Pehea e mālama ai

ʻO ka mālama ʻana i ka hemoglobinuria paroxysmal nocturnal hiki ke hana ʻia me ke transplantation o nā cell stem hematopoietic allogeneic a me ka lāʻau Eculizumab (Soliris) 300mg i kēlā me kēia 15 lā. Hiki ke hoʻolako ʻia kēia lāʻau lapaʻau e SUS ma o ka hana kū kānāwai.

Paipai pū ʻia ka mea hoʻohui hao me ka waikawa folic, a he mea pono e mālama pono i ka nutritional a me ka hematological.