I-anemia ye-spherocytic eyifa

I-anered spherocytic anemia yisifo esingajwayelekile sendawo engaphezulu (ulwelwesi) lwamaseli abomvu egazi. Kuholela kumaseli abomvu egazi abunjwe njengezigaba, nokuwohloka ngaphambi kwesikhathi kwamaseli abomvu egazi (i-hemolytic anemia).

Lesi sifo sidalwa yisakhi sofuzo esingalungile. Ukukhubazeka kubangela ulwelwesi lwegazi olubomvu ngokungajwayelekile. Amaseli athintekile anendawo encane yomthamo wawo kunamaseli abomvu ejwayelekile, futhi angavuleka kalula.

I-anemia ingahluka kusuka kumnene kuya kokubi. Ezimweni ezinzima lesi sifo singatholakala ebuntwaneni. Ezimweni ezimnene ingahle ingabonwa ize ibe mdala.

Lesi sifo sivame kakhulu kubantu bohlanga olusenyakatho neYurophu, kodwa sitholakale kuzo zonke izinhlanga.

Izinsana zingase zibe nokuphuzi kwesikhumba namehlo (i-jaundice) nombala ophaphathekile (i-pallor).

Ezinye izimpawu zingafaka:

• Ukukhathala
• Ukuthukuthela
• Ukuphelelwa umoya
• Ubuthakathaka

Ezimweni eziningi, ubende buyakhuliswa.

Ukuhlolwa kwelabhorathri kungasiza ukuxilonga lesi simo. Ukuhlolwa kungafaka:

• I-blood smear ukukhombisa amaseli abunjwe ngokungajwayelekile
• Izinga leBilirubin
• Qedela ukubalwa kwegazi ukuhlola i-anemia
• Ukuhlolwa kwama-Coombs
• Izinga le-LDH
• Ubuhle be-Osmotic noma ukuhlolwa okukhethekile ukuhlola ukukhubazeka kweseli elibomvu
• Ukubalwa kwe-Reticulocyte

Ukuhlinzwa ukukhipha ubende (splenectomy) kwelapha i-anemia kepha akulungisi ukwakheka kweseli okungajwayelekile.

Imindeni enomlando we-spherocytosis kufanele ihlolwe izingane zayo ngalesi sifo.

Izingane kufanele zilinde kuze kube yiminyaka engu-5 ukuze zibe ne-splenectomy ngenxa yengozi yokutheleleka. Ezimweni ezimnene ezitholwe kubantu abadala, kungahle kungadingeki ukususa ubende.

Izingane kanye nabantu abadala kufanele banikezwe umuthi wokugomela i-pneumococcal ngaphambi kokuhlinzwa kokukhishwa kobende. Futhi kufanele bathole izengezo ze-folic acid. Imithi yokugoma eyengeziwe ingadingeka ngokuya ngomlando womuntu.

Izinsizakusebenza ezilandelayo zinganikeza imininingwane eminingi nge-anared spherocytic anemia:

• Isikhungo Sokwaziswa Sezifo Zofuzo kanye Nezingajwayelekile - rarediseases.info.nih.gov/diseases/6639/redited-spherocytosis
• Inhlangano Kazwelonke Yezinkinga Ezingajwayelekile - rarediseases.org/rare-diseases/anemia-hereditary-spherocytic-hemolytic

Umphumela uvame ukuba muhle ngokwelashwa. Ngemuva kokuthi uswazi selususiwe, ubude bempilo beseli elibomvu buyela kokujwayelekile.

Izinkinga zingafaka:

• Amatshe amatshe amade
• Ukukhiqizwa kwamangqamuzana egazi abomvu amancane kakhulu (inkinga ye-aplastic) ebangelwa ukutheleleka ngegciwane, okungenza i-anemia ibe yimbi kakhulu

Shayela umhlinzeki wakho wezempilo uma:

• Izimpawu zakho ziba zimbi kakhulu.
• Izimpawu zakho azithuthuki ngokwelashwa okusha.
• Uba nezimpawu ezintsha.

Lesi yisifo esizuzwe njengefa futhi kungenzeka singavikeleka. Ukwazi ubungozi bakho, njengomlando womndeni walesi sifo, kungakusiza ukuthi utholakale futhi welashwe kusenesikhathi.

I-anemia ye-hemolytic ye-spherocytic yokuzalwa; I-Spherocytosis; I-hemolytic anemia - i-spherocytic

• Amaseli abomvu egazi - ejwayelekile
• Amaseli abomvu egazi - i-spherocytosis
• Amaseli egazi

IGallagher PG. Ukuphazamiseka kwamangqamuzana egazi abomvu. Ku: Hoffman R, Benz EJ, Silberstein LE, et al, ama-eds. I-Hematology: Izimiso Eziyisisekelo Nokuzijwayeza. Umhla wesi-7. IPhiladelphia, PA: Elsevier; 2018: isahluko 45.

IMerguerian MD, iGallagher PG. I-spherocytosis eyifa. Ku: Kliegman RM, St Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, eds. Incwadi kaNelson Yezingane. Umhlaka 21. IPhiladelphia, PA: Elsevier; 2020: isahluko 485.