I-anemia ye-spherocytic enofuzo

I-anherered spherocytic anemia sisifo esingaqhelekanga somaleko womphezulu (inwebu) yeeseli ezibomvu zegazi. Ikhokelela kwiiseli ezibomvu zegazi ezimile okwe-spheres, kunye nokonakala kwangoko kweeseli ezibomvu zegazi (i-hemolytic anemia).

Esi sifo sibangelwa yimfuza engalunganga. Isiphene sibangela inwebu ebomvu yegazi engaqhelekanga. Iiseli ezichaphazelekayo zinendawo engaphezulu encinci yevolumu yazo kuneeseli ezibomvu eziqhelekileyo, kwaye zinokuvula ngokulula.

I-anemia iyahluka ukusuka kubumnene ukuya kubunzima. Kwiimeko ezinzima esi sifo sinokufunyanwa kwasebuntwaneni. Kwiimeko ezinobuzaza inokungaqwalaselwa de ube mdala.

Esi sifo sixhaphake kakhulu kubantu abavela kumantla eYurophu, kodwa siye safunyanwa kuzo zonke iintlanga.

Iintsana zinokuba tyheli kolusu kunye namehlo (i-jaundice) kunye nombala omhlophe (pallor).

Ezinye iimpawu zingabandakanya:

• Ukudinwa
• Ukucaphuka
• Ukuqhawukelwa ngumphefumlo
• Ubuthathaka

Kwiimeko ezininzi, udakada uyandiswa.

Iimvavanyo zaselebhu zinokunceda ukufumanisa imeko. Uvavanyo lunokubandakanya:

• Igazi smear ukubonisa iiseli ezimile ngokungaqhelekanga
• Inqanaba leBilirubin
• Gcwalisa ubalo lwegazi ukujonga i-anemia
• Uvavanyo lweCombombs
• Inqanaba le-LDH
• Ubuqhetseba be-Osmotic okanye uvavanyo olukhethekileyo lokuvavanya isiphako seeseli ezibomvu zegazi
• Ukubala kweReticulocyte

Utyando lokususa udakada (splenectomy) lunyanga i-anemia kodwa alulungisi imilo yeseli engaqhelekanga.

Iintsapho ezinembali ye-spherocytosis kufuneka ukuba abantwana bayo bahlolwe esi sifo.

Abantwana kufuneka balinde de kube yiminyaka emi-5 ukuze babe ne-splenectomy ngenxa yomngcipheko wosulelo. Kwiimeko ezinobulali ezifunyenwe kubantu abadala, kusenokungabikho mfuneko yokususa udakada.

Abantwana kunye nabantu abadala kufuneka banikwe isitofu sokugonya se-pneumococcal ngaphambi kotyando lokususa udakada. Kananjalo kufuneka bafumane izongezo ze-folic acid. Amayeza okugonya ongezelelweyo anokufuneka ngokusekwe kwimbali yomntu.

Ezi zixhobo zilandelayo zinokubonelela ngolwazi ngakumbi malunga ne-anherred spherocytic anemia:

• IZiko loLwazi lweZifo zeMvelo kunye noRare - rarediseases.info.nih.gov/diseases/6639/redited-spherocytosis
• Umbutho weSizwe woNxunguphalo oluNqabileyo- rarediseases.org/rare-diseases/anemia-redited-spherocytic-hemolytic

Isiphumo sihlala silungile kunyango. Emva kokuba udakada ususwe, ubude bexesha lobomi beseli ebomvu lubuyela esiqhelweni.

Iingxaki zinokubandakanya:

• Amatye egall
• Ukuveliswa kweeseli ezibomvu ezisezantsi kakhulu (ingxaki ye-aplastic) ebangelwa yintsholongwane, enokwenza ukuba i-anemia ibe mandundu

Fowunela umboneleli wakho wezempilo ukuba:

• Iimpawu zakho ziba zibi.
• Iimpawu zakho aziphucuki ngonyango olutsha.
• Uba neempawu ezintsha.

Esi sisifo esizuzwe njengelifa kwaye akunakuthintelwa. Ukwazi umngcipheko wakho, njengembali yosapho yokuphazamiseka, kunokukunceda ufumane isifo kwaye unyangwe kwangoko.

I-spherocytic anemia ye-hemolytic engokwemvelo; I-Spherocytosis; I-anemia yeHemolytic - i-spherocytic

• Iiseli ezibomvu zegazi-ziqhelekileyo
• Iiseli ezibomvu zegazi - i-spherocytosis
• Iiseli zegazi

IGallagher PG. Ukuphazamiseka kwembala yeseli ebomvu. 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 45.

I-Merguerian MD, iGallagher PG. I-spherocytosis yelifa. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, ii-eds. Incwadi kaNelson yeNcwadi yePediatrics. Umhla wama-21. IPhiladelphia, PA: Elsevier; 2020: isahluko 485.