Paroxysmal batang hemoglobinuria (PCH)

Paroxysmal cold hemoglobinuria (PCH) ke lefu le sa tloaelehang la mali leo masole a 'mele a hlahisang masole a' mele a senyang lisele tse khubelu tsa mali. E etsahala ha motho a pepesetsoa ke mocheso o batang.

PCH e etsahala feela serameng, mme e ama haholo-holo matsoho le maoto. Masole a mmele a hokella (tlama) disele tse kgubedu tsa madi. Sena se lumella liprotheine tse ling maling (tse bitsoang complement) hore le tsona li qete. Masole a mmele a senya disele tse kgubedu tsa madi ha a ntse a tsamaya mmeleng. Ha lisele li senngoa, hemoglobin, e leng karolo ea lisele tse khubelu tsa mali tse tsamaisang oksijene, e ntša mali ebe e fetela ka morong.

PCH e hokahantsoe le syphilis ea bobeli, syphilis e phahameng, le mafu a mang a vaerase kapa a baktheria. Ka linako tse ling sesosa ha se tsejoe.

Boloetse bona ha bo fumanehe hangata.

Matšoao a ka kenyelletsa:

• Ho bata
• Feberu
• Bohloko ba morao
• Bohloko ba maoto
• Bohloko ba mpeng
• Ho opeloa ke hlooho
• Ho se utloise bohloko ka kakaretso, ho se ts'oenyehe kapa ho kula (malaise)
• Mali ka morong (moroto o mofubelu)

Liteko tsa laboratori li ka thusa ho fumana boemo bona.

• Litekanyo tsa Bilirubin li na le mali a mangata le moroto.
• Palo e felletseng ea mali (CBC) e bontša phokolo ea mali.
• Teko ea li-coombs e fosahetse.
• Teko ea Donath-Landsteiner e ntle.
• Lactate dehydrogenase level e phahame.

Ho sebetsana le bothata bo ka thusa. Mohlala, haeba PCH e bakoa ke syphilis, matšoao a ka ntlafala ha syphilis e phekoloa.

Maemong a mang, ho sebelisoa meriana e hatellang sesole sa 'mele.

Batho ba nang le lefu lena hangata ba fola kapele mme ha ba na matšoao lipakeng tsa linako. Maemong a mangata, litlhaselo li fela hang ha lisele tse senyehileng li emisa ho tsamaea 'meleng.

Mathata a ka kenyelletsa:

• Tsoela pele litlhaselo
• Ho hloleha ha liphio
• Phokolo ea mali e matla

Bitsa mofani oa tlhokomelo ea bophelo haeba u na le matšoao a lefu lena. Mofani oa thepa a ka hlakola lisosa tse ling tsa matšoao mme a nka qeto ea hore na o hloka kalafo.

Batho ba fumanoeng ba na le lefu lena ba ka thibela litlhaselo tse tlang ka ho lula ka ntle ho serame.

PCH

• Lisele tsa mali

Michel M. Autoimmune le li-intravascular hemolytic anemias. Ka: Goldman L, Schafer AI, li-eds. Phekolo ea Goldman-Cecil. La 26th. Philadelphia, PA: Elsevier; 2020: khaolo ea 151.

Hlōla N, Richards SJ. E fumanoeng ke haemolytic anaemias. Ka: Bain BJ, Bates I, Laffan MA, eds. Dacie le Lewis e sebetsang Hematology. 12th ed. Philadelphia, PA: Elsevier; 2017: khaolo ea 13.