I-Paroxysmal nocturnal hemoglobinuria: yintoni kunye nendlela isifo esenziwe ngayo

Umxholo

• Iimpawu eziphambili
• Uxilongo lwenziwa njani
• Unyango njani

I-Paroxysmal nocturnal hemoglobinuria, ekwabizwa ngokuba yi-PNH, sisifo esinqabileyo semvelaphi yemfuza, esibonakala ngokutshintsha kwilungu leeseli ezibomvu zegazi, esikhokelela ekutshatyalalisweni nasekuphelisweni kwamalungu eeseli ezibomvu zegazi kumchamo, oko kuthathwa njengehemolytic engapheliyo. ianemia.

Igama elithi nocturne libhekisa kwixesha lomhla apho inqanaba eliphezulu lokutshatyalaliswa kweeseli ezibomvu labonwa kubantu abanesifo, kodwa uphando lubonise ukuba ihemolysis, okt ukutshatyalaliswa kweeseli ezibomvu zegazi, kwenzeka nangaliphi na ixesha losuku abantu abanesi sifo.

I-PNH ayinalo unyango, nangona kunjalo unyango lunokwenziwa ngokufakelwa komongo wethambo kunye nokusetyenziswa kwe-Eculizumab, lelona nyango lonyango kunyango lwesi sifo. Funda ngakumbi nge-Eculizumab.

Iimpawu eziphambili

Iimpawu eziphambili zobusuku paroxysmal hemoglobinuria zezi:

• Umchamo wokuqala omnyama kakhulu, ngenxa yoxinzelelo oluphezulu lweeseli ezibomvu zegazi kumchamo;
• Ubuthathaka;
• Ubuthathaka;
• Iinwele neenzipho ezibuthathaka;
• Ukucotha;
• Ubuhlungu bemisipha;
• Rhoqo usulelo;
• Ziva gula;
• Intlungu zesisu;
• IJaundice;
• Ukungasebenzi kakuhle kwamadoda;
• Ukuncipha komsebenzi wezintso.

Abantu abane-paroxysmal nocturnal hemoglobinuria banethuba elonyukayo le-thrombosis ngenxa yotshintsho kwinkqubo yokunqanda igazi.

Uxilongo lwenziwa njani

Ukuxilongwa kwe-paroxysmal hemoglobinuria yasebusuku kwenziwa ngeendlela zovavanyo, ezinje:

• Ubalo lwegaziukuba kubantu abane-PNH, i-pancytopenia iboniswa, ehambelana nokuncipha kwawo onke amacandelo egazi-yazi ukutolika ubalo lwegazi;
• Ubungakanani be bilirubin yasimahla, eyandisiweyo;
• Ukuchongwa kunye nokufakwa kumthamo, ngokusebenzisa i-cytometry yokuhamba, ye I-CD55 kunye ne-CD59 antigens, Eziproteni ezikhoyo kwi-membrane yeeseli ezibomvu zegazi, kwaye kwimeko ye-hemoglobinuria, zincitshisiwe okanye azikho.

Ukongeza kwezi mvavanyo, i-hematologist inokucela iimvavanyo ezongezelelekileyo, ezinje ngovavanyo lwe-sucrose kunye novavanyo lwe-HAM, olunceda ekufumaneni isifo separxysmal hemoglobinuria yasebusuku. Ngokwesiqhelo ukuxilongwa kwenzeka phakathi kweminyaka engama-40 ukuya kwengama-50 kwaye ukusinda komntu kujikeleze iminyaka eli-10 ukuya kweli-15.

Unyango njani

Unyango lwe-paroxysmal hemoglobinuria yasebusuku lunokwenziwa ngokutshintshwa kweeseli ze-hematopoietic kunye neyeza le-Eculizumab (Soliris) 300mg rhoqo kwiintsuku ezili-15. La mayeza anokubonelelwa yi-SUS ngokuthatha amanyathelo asemthethweni.

Ukongezwa kwesinyithi nge-folic acid kuyacetyiswa, ukongeza kwisondlo esaneleyo kunye nokulandelwa kwehematological.