I-hemoglobinuria ebandayo ye-Paroxysmal (PCH)

I-Paroxysmal cold hemoglobinuria (PCH) yisifo segazi esingajwayelekile lapho amasosha omzimba akhiqiza amasosha omzimba abulala amangqamuzana abomvu egazi. Kwenzeka lapho umuntu echayeka emazingeni okushisa abandayo.

I-PCH yenzeka kuphela emakhazeni, futhi ithinta ikakhulu izandla nezinyawo. Ama-antibody anamathisela (hlanganisa) kumaseli abomvu egazi. Lokhu kuvumela amanye amaprotheni asegazini (abizwa ngokuthi umphelelisi) ukuthi abambelele futhi. Amasosha omzimba abhubhisa amangqamuzana abomvu egazi njengoba ehamba emzimbeni. Njengoba amaseli ebhujiswa, i-hemoglobin, ingxenye yamaseli abomvu egazi ethwala umoya-mpilo, iyadedelwa iye egazini bese idluliselwa emchameni.

I-PCH ixhunyaniswe ne-syphilis yesibili, i-syphilis ephakeme, nezinye izifo ezibangelwa amagciwane noma amagciwane. Kwesinye isikhathi imbangela ayaziwa.

Lesi sifo asivamile.

Izimpawu zingafaka:

• Amakhaza
• Imfiva
• Ukuhlungu obusemhlane
• Ubuhlungu bomlenze
• Ubuhlungu besisu
• Ubuhlungu bekhanda
• Ukungakhululeki okuvamile, ukungakhululeki, noma ukugula (malaise)
• Igazi emchameni (umchamo obomvu)

Ukuhlolwa kwelabhorathri kungasiza ukuxilonga lesi simo.

• Amazinga eBilirubin aphezulu egazini nasemchameni.
• Ukubalwa kwegazi okuphelele (CBC) kukhombisa i-anemia.
• Ukuhlolwa kwamaCombomb akulungile.
• Ukuhlolwa kukaDonath-Landsteiner kulungile.
• Izinga le-Lactate dehydrogenase liphezulu.

Ukwelapha isimo esingaphansi kungasiza. Isibonelo, uma i-PCH ibangelwa ugcunsula, izimpawu zingaba ngcono lapho i-syphilis ilashwa.

Kwezinye izimo, kusetshenziswa imithi ecindezela amasosha omzimba.

Abantu abanalesi sifo bavame ukuba ngcono ngokushesha futhi abanazo izimpawu phakathi kweziqephu. Ezimweni eziningi, ukuhlaselwa kuphela lapho amangqamuzana alimele eyeka ukuhamba emzimbeni.

Izinkinga zingafaka:

• Ukuhlaselwa okuqhubekayo
• Ukuhluleka kwezinso
• I-anemia enamandla

Shayela umhlinzeki wakho wezokunakekelwa kwezempilo uma unezimpawu zalesi sifo. Umhlinzeki angazikhipha ezinye izimbangela zezimpawu bese anqume ukuthi ngabe uyakudinga yini ukwelashwa.

Abantu abatholwe benalesi sifo bangavimbela ukuhlaselwa esikhathini esizayo ngokuhlala ngaphandle kwamakhaza.

PCH

• Amaseli egazi

UMichel M. Autoimmune kanye ne-intravascular hemolytic anemias. Ku: IGoldman L, iSchafer AI, ama-eds. Imithi yeGoldman-Cecil. Umhla ka-26. IPhiladelphia, PA: Elsevier; 2020: isahluko 151.

UWin N, uRichards SJ. Ama-anaemias we-haemolytic atholakele. Ku: Bain BJ, Bates I, Laffan MA, eds. UDacie noLewis I-Hematology Esebenzayo. Umhla ka-12. IPhiladelphia, PA: Elsevier; I-2017: isahluko 13.