Lefu la spherocytic khaello ea mali

Hereditary spherocytic anemia ke lefu le sa tloaelehang la bokaholimo ba lera la lisele tse khubelu tsa mali. E lebisa ho lisele tse khubelu tsa mali tse bopehileng joaloka likarolo, le ho senyeha pele ho nako ha lisele tse khubelu tsa mali (hemolytic anemia).

Boloetse bona bo bakoa ke lefutso le sa sebetseng. Bothata bo baka lera le sa tloaelehang la lisele tse khubelu tsa mali. Lisele tse amehileng li na le karolo e nyane ea bokaalo ba tsona ho feta lisele tse khubelu tsa mali tse tloaelehileng, 'me li ka buleha habonolo.

Phokolo ea mali e ka fapana ho tloha bonyenyaneng ho ea ho matla. Maemong a boima bothata bo ka fumanoa bongoaneng. Maemong a bobebe e kanna ea se hlokomelehe ho fihlela motho e moholo.

Boloetse bona bo atile haholo bathong ba litloholo tse ka leboea ho Europe, empa bo fumanoe merabeng eohle.

Masea a ka ba le bosehla ba letlalo le mahlo (jaundice) le 'mala o sootho (pallor).

Matšoao a mang a kenyeletsa:

• Mokhathala
• Ho teneha
• Phefumoloho e khutšoanyane
• Bofokoli

Maemong a mangata, spleen e holisoa.

Liteko tsa laboratori li ka thusa ho fumana boemo bona. Liteko li ka kenyelletsa:

• Mali smear ho bonts'a lisele tse bopehileng ka mokhoa o sa tloaelehang
• Boemo ba Bilirubin
• Palo e felletseng ea mali ho lekola phokolo ea mali
• Teko ea li-coombs
• Boemo ba LDH
• Bofokoli ba Osmotic kapa liteko tse ikhethang ho lekola sekoli se khubelu sa lisele tsa mali
• Palo ea Reticulocyte

Ho buoa ho tlosa spleen (splenectomy) ho phekola phokolo ea mali empa ha ho lokise sebopeho se sa tloaelehang sa sele.

Malapa a nang le nalane ea spherocytosis a lokela ho etsa hore bana ba bona ba hlahlojoe lefu lena.

Bana ba lokela ho ema ho fihlela lilemo tse 5 ho ba le splenectomy ka lebaka la kotsi ea tšoaetso. Maemong a bonolo a fumanoeng ho batho ba baholo, ho kanna ha se hlokahale ho tlosa spleen.

Bana le batho ba baholo ba lokela ho fuoa ente ea pneumococcal pele ba buuoa ka spleen. Hape ba lokela ho fumana litlatsetso tsa folic acid. Meento e meng e ka hlokahala ho latela nalane ea motho.

Lisebelisoa tse latelang li ka fana ka tlhaiso-leseling e batsi ka lefu la phokolo ea mali ea lefutso:

• Setsi sa Boitsebiso ba Mafu le a Rare - rarediseases.info.nih.gov/diseases/6639/hereditary-spherocytosis
• Mokhatlo oa Naha oa Mathata a Rare - rarediseases.org/rare-diseases/anemia-hereditary-spherocytic-hemolytic

Phello hangata e ntle ka kalafo. Kamora hore spleen e tlosoe, bolelele ba bophelo ba sele e khubelu ea mali bo khutlela setloaeling.

Mathata a ka kenyelletsa:

• Majoe a majoe
• Tlhahiso e fokolang haholo ea lisele tse khubelu tsa mali (aplastic crisis) e bakoang ke tšoaetso ea vaerase, e ka mpefatsang phokolo ea mali

Bitsa mofani oa tlhokomelo ea bophelo haeba:

• Matšoao a hau a mpefala.
• Matšoao a hau ha a ntlafatse kalafo e ncha.
• U hlahisa matšoao a macha.

Ena ke boloetse bo futsitsoeng mme e kanna ea se ke ea qojoa. Ho tseba kotsi ea hau, joalo ka nalane ea lelapa ea lefu lena, ho ka u thusa ho fumanoa le ho phekoloa kapele.

Congenital spherocytic hemolytic anemia; Spherocytosis; Hemolytic khaello ea mali - spherocytic

• Lisele tse khubelu tsa mali - li tloaelehile
• Lisele tse khubelu tsa mali - spherocytosis
• Lisele tsa mali

Gallagher PG. Mathata a lisele tse khubelu tsa lisele tsa mali. Ka: Hoffman R, Benz EJ, Silberstein LE, et al, eds. Hematology: Melao-motheo le Tloaelo ea Motheo. La 7th. Philadelphia, PA: Elsevier; 2018: khaolo ea 45.

MD ea Merguerian, Gallagher PG. Lefa spherocytosis. Ka: Kliegman RM, St. Geme JW, Blum NJ, Shah SS, Tasker RC, Wilson KM, bahlophisi. Buka ea Nelson ea Pediatrics. La 21 Ed. Philadelphia, PA: Elsevier; 2020: khaolo ea 485.