I-Paroxysmal nocturnal hemoglobinuria (PNH)

I-Paroxysmal nocturnal hemoglobinuria sisifo esinqabileyo apho iiseli ezibomvu zegazi zehla ngaphambi kwexesha eliqhelekileyo.

Abantu abanesi sifo baneeseli zegazi ezingekhoyo kwimfuza ebizwa ngokuba yi-PIG-A. Olu hlobo luvumela into ebizwa ngokuba yi-glycosyl-phosphatidylinositol (GPI) ukunceda iiproteni ezithile zinamathele kwiiseli.

Ngaphandle kwe-PIG-A, iiproteni ezibalulekileyo azinakho ukuqhagamshela kumphezulu weseli kwaye zikhusele iseli kwizinto ezisegazini ekuthiwa sisincedisi. Ngenxa yoko, iiseli ezibomvu zegazi ziqhekeka kwangoko. Iiseli ezibomvu zivuza ihemoglobin egazini, enokudlula ichame. Oku kunokwenzeka nanini na, kodwa kunokwenzeka kakhulu ukuba kwenzeke ebusuku okanye kusasa.

Esi sifo sinokuchaphazela abantu nabuphi na ubudala. Inokuthi idibaniswe ne-aplastic anemia, i-myelodysplastic syndrome, okanye i-leukemia enomdla.

Imiba yomngcipheko, ngaphandle kwe-anemia yangaphambili, ayaziwa.

Iimpawu zingabandakanya:

• Intlungu zesisu
• Umqolo obuhlungu
• Amahlwili egazi, anokuvela kwabanye abantu
• Umchamo omnyama, uyeza uhambe
• Ukutyumza ngokulula okanye ukopha
• Intloko ebuhlungu
• Ukuqhawukelwa ngumphefumlo
• Ubuthathaka, ukudinwa
• Umtsalane
• Iintlungu zesifuba
• Kunzima ukugwinya

Ubalo lweeseli ezibomvu nezimhlophe zegazi kunye nokubalwa kweplatelet kunokuba sezantsi.

Umchamo obomvu okanye omdaka ubonakalisa ukonakala kweeseli ezibomvu zegazi kunye nokuba ihemoglobin iyakhutshwa ijikeleze emzimbeni kwaye ekugqibeleni iye emchameni.

Uvavanyo olunokwenziwa ukufumanisa le meko lubandakanya:

• Gcwalisa ubalo lwegazi (CBC)
• Uvavanyo lweCombombs
• Ukuhamba kwecytometry ukulinganisa iiproteni ezithile
• Uvavanyo lweHam (i-acid hemolysin)
• I-serum hemoglobin kunye ne-haptoglobin
• Uvavanyo lweSucrose hemolysis
• Uhlalutyo lomchamo
• Umchamo hemosiderin, urobilinogen, hemoglobin
• Uvavanyo lwe-LDH
• Ukubala kweReticulocyte

I-Steroids okanye amanye amayeza acinezela amajoni omzimba anokunceda ekunciphiseni ukonakala kweeseli ezibomvu zegazi. Ukutofelwa igazi kusenokufuneka. Isinyithi esongezelelweyo kunye nefolic acid zibonelelwa. Ukucoca igazi kunokufuneka kwakhona ukuthintela amahlwili ekubunzeni.

I-Soliris (eculizumab) sisiyobisi esisetyenziselwa ukunyanga i-PNH. Ibhloka ukonakala kweeseli ezibomvu zegazi.

Ukufakelwa komongo wethambo kungasinyanga esi sifo. Inokumisa umngcipheko wokuphuhlisa i-PNH kubantu abane-aplastic anemia.

Bonke abantu abane-PNH kufuneka bafumane ugonyo ngokuchasene neentlobo ezithile zebacteria ukuthintela usulelo. Buza umboneleli wakho wezempilo ukuba zeziphi ezifanelekileyo.

Iziphumo ziyahluka. Uninzi lwabantu lusinda ngaphezulu kweminyaka eli-10 emva kokuchongwa kwesifo. Ukufa kunokubangelwa ziingxaki ezinjengokwakheka kwegazi (thrombosis) okanye ukopha.

Kwiimeko ezinqabileyo, iiseli ezingaqhelekanga zinokuhla ngokuhamba kwexesha.

Iingxaki zinokubandakanya:

• Igazi leukemia myelogenous
• I-aplastic anemia
• Amahlwili egazi
• Ukufa
• I-anemia yeHemolytic
• Ukunqongophala kwe-anemia
• UMyelodysplasia

Fowunela umboneleli wakho ukuba ufumana igazi kumchamo wakho, ukuba iimpawu ziya zisiba mbi okanye aziphuculi ngonyango, okanye ukuba iimpawu ezintsha ziyavela.

Akukho ndlela yaziwayo yokuthintela esi sifo.

PNH

• Iiseli zegazi

UBrodsky RA. Paroxysmal ebusuku hemoglobinuria. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ii-eds. I-Hematology: Imigaqo esisiseko kunye nokuziqhelanisa. Umhla wesi-7. IPhiladelphia, PA: Elsevier; I-2018: isahluko 31.

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.