I-paroxysmal nocturnal hemoglobinuria: ukuthi iyini nokuthi ukuxilongwa kwenziwa kanjani

-Delile

• Izimpawu eziyinhloko
• Ukuxilongwa kwenziwa kanjani
• Indlela yokwelapha

I-paroxysmal nocturnal hemoglobinuria, eyaziwa nangokuthi i-PNH, isifo esingajwayelekile semvelaphi yezakhi zofuzo, esibonakala ngokuguqulwa kolwelwesi lwamaseli abomvu egazi, okuholela ekubhujisweni nasekuqedweni kwezingxenye zamaseli abomvu egazini emchameni, ngaleyo ndlela kuthathwe njenge-hemolytic engapheli i-anemia.

Igama elithi nocturne lisho isikhathi sosuku lapho izinga eliphezulu kakhulu lokubhujiswa kwamangqamuzana egazi abomvu kwabonwa kubantu abanalesi sifo, kepha uphenyo lukhombisile ukuthi i-hemolysis, okusho ukubhujiswa kwamangqamuzana egazi abomvu, yenzeka nganoma yisiphi isikhathi sosuku abantu abanalesi sifo hemoglobinuria.

I-PNH ayinawo umuthi, noma kunjalo ukwelashwa kungenziwa ngokufakwa komnkantsha wethambo nangokusetshenziswa kwe-Eculizumab, okuwumuthi othize wokwelashwa kwalesi sifo. Funda kabanzi nge-Eculizumab.

Izimpawu eziyinhloko

Izimpawu eziyinhloko ze-paroxysmal hemoglobinuria yasebusuku yilezi:

• Umchamo wokuqala omnyama kakhulu, ngenxa yokuxinana okuphezulu kwamaseli abomvu emchameni;
• Ubuthakathaka;
• Ubuthakathaka;
• Izinwele nezinzipho ezibuthakathaka;
• Ukuhamba kancane;
• Ubuhlungu bemisipha;
• Ukutheleleka njalo;
• Ukuzizwa ngiyagula;
• Ubuhlungu besisu;
• I-jaundice;
• Ukungasebenzi kahle kowesilisa;
• Ukwehla kokusebenza kwezinso.

Abantu abane-paroxysmal nocturnal hemoglobinuria banethuba elandayo le-thrombosis ngenxa yezinguquko enqubeni yokuvala igazi.

Ukuxilongwa kwenziwa kanjani

Ukuxilongwa kwe-paroxysmal hemoglobinuria yasebusuku kwenziwa ngokusebenzisa izivivinyo eziningana, njenge:

• Ukubalwa kwegazi, ukuthi kubantu abane-PNH, i-pancytopenia ikhonjisiwe, ehambelana nokwehla kwazo zonke izingxenye zegazi - yazi ukutolika inani legazi;
• Isilinganiso se- i-bilirubin yamahhala, okwandisiwe;
• Ukuhlonza nokufaka imithamo, ngokusebenzisa i-flow cytometry, ye- Ama-antigen e-CD55 ne-CD59, okungamaprotheni akhona kulwelwesi lwamaseli abomvu futhi, esimweni se-hemoglobinuria, ancishisiwe noma awekho.

Ngaphezu kwalezi zivivinyo, i-hematologist ingacela ukuhlolwa okuhambisanayo, njengokuhlolwa kwe-sucrose kanye nokuhlolwa kwe-HAM, okusiza ekutholeni i-paroxysmal hemoglobinuria yasebusuku. Imvamisa ukuxilongwa kwenzeka phakathi kweminyaka engama-40 nengama-50 kanti ukusinda komuntu kuba cishe eminyakeni eyi-10 kuye kwengu-15.

Indlela yokwelapha

Ukwelashwa kwe-paroxysmal hemoglobinuria yasebusuku kungenziwa ngokufakelwa kabusha kwama-allogeneic hematopoietic stem cells kanye nomuthi i-Eculizumab (Soliris) 300mg njalo ezinsukwini eziyi-15. Lo muthi unganikezwa yi-SUS ngezenzo zomthetho.

I-iron supplementation ene-folic acid nayo iyanconywa, ngaphezu kokulandelwa okwanele kokudla okunomsoco nokulandelwa kwe-hematological.