Paroxysmal kubanda hemoglobinuria (PCH)

I-Paroxysmal cold hemoglobinuria (PCH) sisifo esingaqhelekanga segazi apho amajoni omzimba avelisa izilwa-buhlungu ezitshabalalisa iiseli ezibomvu zegazi. Kwenzeka xa umntu eve kumaqondo obushushu abandayo.

I-PCH yenzeka kuphela kwingqele, kwaye ichaphazela ikakhulu izandla neenyawo. Iintsholongwane zincamathele (zibopha) kwiiseli ezibomvu zegazi. Oku kuvumela ezinye iiproteni egazini (ezibizwa ngokuba zincedisayo) ukuba ziphinde zibambe. Amachiza omzimba atshabalalisa iiseli ezibomvu zegazi njengoko zihamba emzimbeni. Njengokuba iiseli zitshatyalalisiwe, ihemoglobin, eyinxalenye yeeseli ezibomvu zegazi ethwala ioksijini, ikhutshelwa egazini ize idluliselwe kumchamo.

I-PCH inxulunyaniswa ne-syphilis yesibini, i-syphilis ephezulu, kunye nolunye usulelo lwentsholongwane okanye ibacteria. Ngamanye amaxesha unobangela awaziwa.

Isifo sinqabile.

Iimpawu zingabandakanya:

• Ukugodola
• Ifiva
• Umqolo obuhlungu
• Intlungu yomlenze
• Intlungu zesisu
• Intloko ebuhlungu
• Ukungakhululeki ngokubanzi, ukungonwabi, okanye ukuziva ungonwabanga (malaise)
• Igazi kumchamo (umchamo obomvu)

Iimvavanyo zaselebhu zinokunceda ukufumanisa imeko.

• Amanqanaba eBilirubin aphezulu kwigazi kunye nomchamo.
• Inani elipheleleyo legazi (CBC) libonisa i-anemia.
• Uvavanyo lweCombomb alunabungozi.
• Uvavanyo lweDonath-Landsteiner luqinisekile.
• Inqanaba le-Lactate dehydrogenase liphezulu.

Ukunyanga imeko engaphantsi kunokunceda. Umzekelo, ukuba i-PCH ibangelwa yi-syphilis, iimpawu zingangcono xa i-syphilis inyangwa.

Ngamanye amaxesha, amayeza athintela amajoni omzimba ayasetyenziswa.

Abantu abanesi sifo bahlala bebangcono ngokukhawuleza kwaye abanazimpawu phakathi kweziqendu. Kwiimeko ezininzi, uhlaselo luphela kwakamsinya nje ukuba iiseli ezonakeleyo ziyeke ukuhamba emzimbeni.

Iingxaki zinokubandakanya:

• Uhlaselo oluqhubekayo
• Ukungaphumeleli kwezintso
• Anemia kakhulu

Fowunela umboneleli wakho wezempilo ukuba uneempawu zesi sifo. Umboneleli unokulawula ezinye izizathu zeempawu kwaye athathe isigqibo sokuba ufuna unyango.

Abantu abaye bafumanisa ukuba banesi sifo banokuthintela ukuhlaselwa kwangaphambili ngokuhlala ngaphandle kwengqele.

PCH

• Iiseli zegazi

UMichel M. Ukuzenzekelayo kunye ne-intravascular hemolytic anemias. Ku: IGoldman L, iSchafer AI, ii-eds. Amayeza eGoldman-Cecil. Ngomhla wama-26. IPhiladelphia, PA: Elsevier; 2020: isahl. 151.

Ukuphumelela N, uRichards SJ. Ukufumana i-haemolytic anaemias. Ku: Bain BJ, iBates I, Laffan MA, ii-eds. UDacie kunye noLewis beHematology esebenzayo. Umhla we-12. IPhiladelphia, PA: Elsevier; Ngo-2017: isahluko 13.