ʻO Paroxysmal hemoglobinuria nocturnal (PNH)

ʻO Paroxysmal nocturnal hemoglobinuria kahi maʻi laha ʻole kahi e wāwahi ai nā hunaola ʻulaʻula ma mua o ka maʻamau.

Loaʻa i nā poʻe me kēia maʻi nā pūnana koko e nalo ana i kahi gen i kapa ʻia ʻo PIG-A. ʻAe kēia gen i kahi mea i kapa ʻia glycosyl-phosphatidylinositol (GPI) e kōkua i kekahi mau protein i pili i nā hunaola.

Me ka ʻole o PIG-A, ʻaʻole hiki i nā protein koʻikoʻi ke hoʻohui i ka ʻili pūnaewele a pale i ka pūnaewele mai nā mea i loko o ke koko i kapa ʻia he kōkua. A ʻo kahi hopena, haki ke koko ʻulaʻula i ka wanaʻao. Hoʻopuka nā hunaola ʻulaʻula i ka hemoglobin i loko o ke koko, a hiki ke hele i loko o ka mimi. Hiki i kēia ke hana i kēlā me kēia manawa, akā ʻike ʻia paha i ka pō a i ke kakahiaka nui paha.

Hiki i ka maʻi ke hoʻopili i nā poʻe o nā makahiki. Pili paha ia me ka anemia aplastic, myelodysplastic syndrome, a i ʻole leukemia myelogenous huehue.

ʻAʻole ʻike ʻia nā mea weliweli, koe wale no ka anemia aplastic mua.

Pākuʻi ʻia paha nā ʻōuli:

• Abdominal eha
• ʻEha kua
• Huʻi koko, hana paha i loko o kekahi poʻe
• Mimi ʻeleʻele, hele mai a hele
• Māʻeha a hoʻokahe wale paha
• ʻEha poʻo
• Pokole o ka hanu
• Nawaliwali, luhi
• Pallor
• ʻEha ʻeha
• Pilikia i ka ale ʻana

ʻO ka helu ʻulaʻula a me keʻokeʻo o ke koko a haʻahaʻa paha ka helu platelet.

Hōʻailona ka mimi ʻulaʻula a ʻeleʻele paha i ka haki o nā hunaola ʻulaʻula a ke hoʻokuʻu ʻia nei ka hemoglobin i ke kahe o ke kino a i ka hope loa i ka mimi.

Nā hoʻokolohua e hana ʻia ai e kuhikuhi i kēia ʻano me:

• Helu piha koko (CBC)
• Hōʻike Coombs
• Kahe ana ka cytometry e ana i kekahi mau protein
• Hōʻike Ham (acid hemolysin)
• Serum hemoglobin a me haptoglobin
• Hōʻike hemolisis Sucrose
• Urinalysis
• Mimi hemosiderin, urobilinogen, hemoglobin
• Hoʻāʻo LDH
• Helu helu Retikulosit

ʻO nā steroid a i ʻole nā ​​lāʻau ʻē aʻe e kāohi i ka ʻōnaehana pale e kōkua paha i ka lohi o ka haki ʻana o nā hunaola ʻulaʻula. Pono paha ke kuhi koko. Hāʻawi ʻia ka hao mea hoʻohui a me ka waikawa folic. Pono pū ʻia nā mea lahilahi o ke koko e pale aku i nā clots mai ka hana ʻana.

ʻO Soliris (eculizumab) kahi lāʻau i hoʻohana ʻia e mālama iā PNH. Pale ia i ka haki ʻana o nā hunaola ʻulaʻula.

Hiki ke hoʻōla i ka iwi iwi i kēia maʻi. Hiki iā ia ke ho'ōki i ka pilikia o ka hoʻomohalaʻana i ka PNH i ka poʻe me ka anemia aplastic.

Pono nā kānaka āpau me PNH e loaʻa nā lāʻau āpau e kūʻē i kekahi ʻano bacteria i mea e pale aku ai i ka maʻi. E noi i kāu mea mālama ola i nā mea kūpono iā ʻoe.

Hoʻololi ka hopena. Ola ka hapanui o nā kānaka ma mua o 10 mau makahiki ma hope o kā lākou hōʻoia. Hiki i ka make ke hopena mai nā pilikia e like me ka hoʻokumu ʻana o ke koko (thrombosis) a i ʻole ke kahe ʻana o ke koko.

I nā hihia maʻamau, hoʻemi paha nā hunaola ʻino i ka manawa.

Komo pū nā pilikia:

• Leukemia myelogenous huikau
• Anemia anplastic
• Huʻi koko
• Make
• Hemolytic anemia
• Anemia hemahema hao
• ʻO Myelodysplasia

Kāhea i kāu mea hāʻawi inā ʻike ʻoe i ke koko i loko o kou mimi, inā hōʻeha nā hōʻailona a hoʻomaikaʻi ʻole paha me ka lāʻau, a inā ulu nā ʻōuli hou.

ʻAʻohe ala i ʻike ʻia e pale ai i kēia maʻi.

PNH

• Nā pūnaewele koko

ʻO Brodsky RA. ʻO Paroxysmal hemoglobinuria nocturnal. I: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Nā Kumumanaʻo a me ka Hoʻomeheu. ʻEpekema 7 Piladelapia, PA: Elsevier; 2018: chap 31.

ʻO Michel M. Autoimmune a me nā anemias hemolytic intravascular. I: Goldman L, Schafer AI, eds. ʻO ka lāʻau lapaʻau Goldman-Cecil. 26th ed. Piladelapia, PA: Elsevier; 2020: chap 151.