Paroxysmal mara lafiyar haemoglobinuria (PNH)

Paroxysmal nomocikin haemoglobinuria wata cuta ce mai saurin gaske inda jajayen jini ke farfasawa da wuri kamar yadda aka saba.

Mutanen da ke da wannan cutar suna da ƙwayoyin jini waɗanda suka ɓace wata kwayar halitta da ake kira PIG-A. Wannan kwayar halitta tana bada damar wani sinadari da ake kira glycosyl-phosphatidylinositol (GPI) don taimakawa wasu sunadarai da ke manne da kwayoyin halitta.

Ba tare da PIG-A ba, mahimman sunadarai ba za su iya haɗuwa da saman tantanin halitta ba kuma su kare kwayar daga abubuwa a cikin jini da ake kira mentara. A sakamakon haka, jajayen ƙwayoyin jini suna narkewa da wuri. Jajayen kwayoyin halitta suna zuba haemoglobin cikin jini, wanda zai iya wucewa zuwa fitsarin. Wannan na iya faruwa a kowane lokaci, amma zai iya faruwa a cikin dare ko sanyin safiya.

Cutar na iya shafar mutane na kowane zamani. Zai iya kasancewa yana haɗuwa da anemia na ruɓaɓɓen jini, cututtukan myelodysplastic, ko kuma cutar sankarar myelogenous.

Ba a san abubuwan haɗari ba, ban da cutar karancin jini da ke faruwa a baya.

Kwayar cutar na iya haɗawa da:

• Ciwon ciki
• Ciwon baya
• Jigilar jini, na iya haifar da wasu mutane
• Fitsari mai duhu, yakan zo ya tafi
• Easyarami mai sauƙi ko zub da jini
• Ciwon kai
• Rashin numfashi
• Rauni, gajiya
• Maɗaukaki
• Ciwon kirji
• Matsalar haɗiyewa

Redididdigar ƙwayoyin jini na jini ja da fari suna iya zama ƙasa.

Fitsari mai launin ja ko launin ruwan kasa yana ba da alamar lalacewar ƙwayoyin jinin jini da cewa haemoglobin ana sake shi cikin zagawar jiki kuma daga ƙarshe zuwa cikin fitsarin.

Gwaje-gwajen da za'a iya yi don tantance wannan yanayin sun haɗa da:

• Kammala ƙididdigar jini (CBC)
• Gwajin kabo
• Gudura cytometry don auna wasu sunadarai
• Ham (acid hemolysin) gwaji
• Maganin haemoglobin da haptoglobin
• Sucrose hemolysis gwajin
• Fitsari
• Fitsarin hemosiderin, urobilinogen, haemoglobin
• LDH gwaji
• Icididdigar Reticulocyte

Steroids ko wasu magungunan da ke dankwafar da garkuwar jiki na iya taimakawa rage ragowar jajayen ƙwayoyin jini. Ana iya buƙatar ƙarin jini. Ana ba da ƙarin ƙarfe da folic acid. Hakanan za'a iya buƙatar sikanin jini don hana daskarewa daga yin ta.

Soliris (eculizumab) magani ne da ake amfani dashi don magance PNH. Yana toshe raunin jinin ja.

Yin dashen ƙashi na kashin baya na iya warkar da wannan cuta. Hakanan yana iya dakatar da haɗarin ɓarkewar PNH a cikin mutanen da ke fama da cutar rashin jini.

Duk mutanen da ke da PNH ya kamata su karɓi rigakafin wasu nau'in ƙwayoyin cuta don hana kamuwa da cuta. Tambayi mai ba ku kiwon lafiya wanne ne daidai a gare ku.

Sakamakon ya bambanta. Yawancin mutane suna rayuwa fiye da shekaru 10 bayan ganowar su. Mutuwa na iya haifar da rikice-rikice irin su samuwar jini (thrombosis) ko zub da jini.

A cikin wasu lokuta, ƙananan ƙwayoyin cuta na iya raguwa a kan lokaci.

Matsaloli na iya haɗawa da:

• Mutuwar cutar sankarar bargo
• Ruwan jini
• Jinin jini
• Mutuwa
• Anaemia mai raunin jini
• Karancin karancin baƙin ƙarfe
• Myelodysplasia

Kira mai ba ku sabis idan kun sami jini a cikin fitsarinku, idan alamun bayyanar sun taɓarɓare ko kuma ba su inganta da magani, ko kuma idan sababbin alamu sun bayyana.

Babu wata hanyar da aka sani don hana wannan cuta.

PNH

• Kwayoyin jini

Brodsky RA. Paroxysmal mara lafiyar haemoglobinuria. A cikin: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Ka'idoji da Aiki. 7th ed. Philadelphia, PA: Elsevier; 2018: babi na 31.

Michel M. Autoimmune da cutar hemolytic anemias. A cikin: Goldman L, Schafer AI, eds. Magungunan Goldman-Cecil. 26th ed. Philadelphia, PA: Elsevier; 2020: babi na 151.