Paroxysmal nocturnal hemoglobinuria: menene menene kuma yadda ake gane asali

Wadatacce

• Babban bayyanar cututtuka
• Yadda ake ganewar asali
• Yadda za a bi da

Paroxysmal hemoglobinuria, wanda aka fi sani da PNH, cuta ce mai saurin gaske ta asalin halitta, wanda ke tattare da canje-canje a cikin membrane na jinin ja, wanda ke haifar da lalacewa da kuma kawar da abubuwan da ke cikin jajayen ƙwayoyin jini a cikin fitsari, don haka ana ɗaukarsa mai cutar hemolytic karancin jini

Kalmar nocturne tana nufin lokacin yini lokacin da aka lura da mafi girman ƙwayar jinin jini a cikin mutanen da ke fama da cutar, amma bincike ya nuna cewa hemolysis, watau lalata jajayen ƙwayoyin jini, yana faruwa a kowane lokaci na yini a mutanen da suke da cutar. hemoglobinuria.

PNH ba shi da magani, duk da haka za a iya yin maganin ta hanyar dusar ƙashi da amfani da Eculizumab, wanda shine takamaiman magani don maganin wannan cuta. Learnara koyo game da Eculizumab.

Babban bayyanar cututtuka

Babban alamun cututtukan haemoglobinuria na paroxysmal:

• Farkon fitsari mai tsananin duhu, saboda yawan ƙwayoyin jan jini a cikin fitsarin;
• Rashin rauni;
• Rashin hankali;
• Raunin gashi da kusoshi;
• Sannu a hankali;
• Ciwon tsoka;
• Yawaitar cututtuka;
• Jin rashin lafiya;
• Ciwon ciki;
• Jaundice;
• Mutuwar namiji;
• Rage aikin koda.

Mutanen da ke tare da haemoglobinuria na paroxysmal suna da damar thrombosis saboda canje-canje a tsarin daskarewar jini.

Yadda ake ganewar asali

Ganewar asali na haemoglobinuria na paroxysmal nocturnal ana yin shi ta hanyar gwaje-gwaje da yawa, kamar:

• Yawan jini, cewa a cikin mutanen da ke da PNH, ana nuna pancytopenia, wanda ya yi daidai da ragin dukkan abubuwan da ke cikin jini - san yadda ake fassara ƙidayar jini;
• Sashi na bilirubin kyauta, wanda ya karu;
• Tabbatarwa da yin allurai, ta hanyar tsarin kimiyyar motsa jiki, na CD55 da CD59 antigens, waxanda suke da sunadaran da suke cikin membrane na jajayen jini kuma, a game da haemoglobinuria, sun ragu ko ba su nan.

Baya ga waɗannan gwaje-gwajen, likitan jini na iya buƙatar ƙarin gwaje-gwaje, kamar su sucrose test da gwajin HAM, wanda ke taimaka wa wajen gano cutar hemoglobinuria ta paroxysmal. Yawancin lokaci akan gano cutar ne tsakanin shekaru 40 zuwa 50 kuma rayuwar mutum tana kusan shekaru 10 zuwa 15.

Yadda za a bi da

Za'a iya yin maganin hemoglobinuria na paroxysmal na dare tare da dasawa na kwayoyin halittar hematopoietic da kuma maganin Eculizumab (Soliris) 300mg kowane kwana 15. Wannan SUS na iya bayarwa ta SUS ta hanyar ɗaukar doka.

Hakanan ana ba da shawarar ƙarin ƙarfe tare da folic acid, ban da isasshen abinci mai gina jiki da kuma bin jini.