Paroxysmal usiku wotchedwa hemoglobinuria (PNH)

Paroxysmal nocturnal hemoglobinuria ndi matenda osowa pomwe ma cell ofiira amafa msanga kuposa nthawi zonse.

Anthu omwe ali ndi matendawa ali ndi maselo amwazi omwe akusowa jini wotchedwa PIG-A. Jini imeneyi imalola chinthu chotchedwa glycosyl-phosphatidylinositol (GPI) kuthandiza mapuloteni ena kumamatira kumaselo.

Popanda PIG-A, mapuloteni ofunikira sangathe kulumikizana ndi selo ndikuteteza khungu ku zinthu zamagazi zotchedwa complement. Zotsatira zake, maselo ofiira amafa msanga kwambiri. Maselo ofiirawo amatulutsa hemoglobin m'magazi, omwe amatha kulowa mkodzo. Izi zitha kuchitika nthawi iliyonse, koma zimachitika usiku kapena m'mawa kwambiri.

Matendawa amatha kukhudza anthu amisinkhu iliyonse. Itha kukhala yokhudzana ndi kuchepa kwa magazi m'thupi, matenda a myelodysplastic, kapena khansa ya m'magazi.

Zowopsa, kupatula kuchepa kwa magazi m'thupi koyambirira, sizidziwika.

Zizindikiro zimaphatikizapo:

• Kupweteka m'mimba
• Ululu wammbuyo
• Kuundana kwamagazi, kumatha kupanga anthu ena
• Mkodzo wamdima, umabwera ndikupita
• Kuvulaza kosavuta kapena kutuluka magazi
• Mutu
• Kupuma pang'ono
• Kufooka, kutopa
• Pallor
• Kupweteka pachifuwa
• Zovuta kumeza

Kuwerengera kwa maselo ofiira ndi oyera magazi kuwerengera kumatha kukhala kotsika.

Mkodzo wofiira kapena wofiirira umawonetsa kuwonongeka kwa maselo ofiira amwaziwo ndikuti hemoglobin imatulutsidwa m'magazi amthupi ndikumapeto pake mkodzo.

Kuyesa komwe kungachitike kuti mupeze vutoli ndi monga:

• Kuwerengera kwathunthu kwa magazi (CBC)
• Mayeso a Coombs
• Kuyenda kwa cytometry kuyeza mapuloteni ena
• Mayeso a Ham (acid hemolysin)
• Seramu hemoglobin ndi haptoglobin
• Mayeso a Sucrose hemolysis
• Kupenda kwamadzi
• Mkodzo hemosiderin, urobilinogen, hemoglobin
• Mayeso a LDH
• Kuwerengera kwa reticulocyte

Steroids kapena mankhwala ena omwe amaletsa chitetezo cha mthupi angathandize kuchepetsa kuwonongeka kwa maselo ofiira amwazi. Kuthiriridwa magazi kungafune. Chitsulo chowonjezera ndi folic acid amaperekedwa. Ochepetsa magazi amafunikiranso kuti magazi asapangike.

Soliris (eculizumab) ndi mankhwala omwe amagwiritsidwa ntchito pochiza PNH. Imaletsa kuwonongeka kwa maselo ofiira amwazi.

Kuika mafuta m'mafupa kumatha kuchiza matendawa. Zingathenso kuletsa chiopsezo chokhala ndi PNH mwa anthu omwe ali ndi kuchepa kwa magazi m'thupi.

Anthu onse omwe ali ndi PNH ayenera kulandira katemera ku mitundu ina ya mabakiteriya kuti ateteze matenda. Funsani wothandizira zaumoyo wanu omwe ali oyenera kwa inu.

Zotsatira zimasiyanasiyana. Anthu ambiri amakhala ndi moyo zaka zopitilira 10 atawazindikira. Imfa imatha chifukwa cha zovuta monga kupangika kwa magazi (thrombosis) kapena kutuluka magazi.

Nthawi zambiri, maselo osadziwika amatha kutsika pakapita nthawi.

Zovuta zingaphatikizepo:

• Pachimake myelogenous khansa ya m'magazi
• Kuchepa kwa magazi m'thupi
• Kuundana kwamagazi
• Imfa
• Kuchepa kwa magazi m'thupi
• Kusowa kwa magazi m'thupi kwachitsulo
• Myelodysplasia

Itanani omwe akukuthandizani mukapeza magazi mumkodzo wanu, ngati zizindikiro zikukulirakulira kapena sizikusintha ndi chithandizo chamankhwala, kapena ngati zizindikiro zatsopano zikuyamba.

Palibe njira yodziwika yothetsera vutoli.

PNH

• Maselo amwazi

Brodsky RA. Paroxysmal usiku hemoglobinuria. Mu: Hoffman R, Benz EJ, Silberstein LE, et al, olemba. Hematology: Mfundo Zoyambira ndi Zochita. Wachisanu ndi chiwiri. Philadelphia, PA: Elsevier; 2018: mutu 31.

Michel M. Autoimmune ndi intravascular hemolytic anemias. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 151.