Paroxysmal bosiu hemoglobinuria (PNH)

Paroxysmal nocturnal hemoglobinuria ke lefu le sa tloaelehang leo ho lona lisele tse khubelu tsa mali li senyehang pejana ho tloaelehileng.

Batho ba nang le lefu lena ba na le lisele tsa mali tse haelloang ke lefutso le bitsoang PIG-A. Jini ena e lumella ntho e bitsoang glycosyl-phosphatidylinositol (GPI) ho thusa liprotheine tse itseng ho khomarela lisele.

Ntle le PIG-A, liprotheine tsa bohlokoa li ke ke tsa hokahana le bokaholimo ba sele le ho sireletsa sele ho lintho tse maling tse bitsoang complement. Ka lebaka leo, lisele tse khubelu tsa mali li senyeha kapele haholo. Lisele tse khubelu li lutlisetsa hemoglobin maling, e ka fetelang le morong. Sena se ka etsahala neng kapa neng, empa se ka etsahala bosiu kapa hoseng haholo.

Lefu lena le ka ama batho ba lilemo tsohle. E kanna ea amahanngoa le aplastic anemia, myelodysplastic syndrome, kapa leukemia e matla ea myelogenous.

Lisosa tsa kotsi, ntle le phokolo ea mali e tsoang pele, ha li tsejoe.

Matšoao a ka kenyelletsa:

• Bohloko ba mpeng
• Bohloko ba morao
• Ho koala mali, ho ka hlaha ho batho ba bang
• Moroto o lefifi, oa tla ebe oa feta
• Ho loma habonolo kapa ho tsoa mali
• Ho opeloa ke hlooho
• Phefumoloho e khutšoanyane
• Bofokoli, mokhathala
• Pallor
• Bohloko ba sefuba
• Ho thata ho koenya

Lisele tse khubelu le tse tšoeu tsa lisele tsa mali le lipalo tsa platelet li ka ba tlase.

Moroto o mofubelu kapa o mosootho o bontša ho senyeha ha lisele tse khubelu tsa mali le hore hemoglobin e ntse e lokolloa hore e potolohe 'mele ebe qetellong e ea le morong.

Liteko tse ka etsoang ho fumana boemo bona li kenyelletsa:

• Palo e felletseng ea mali (CBC)
• Teko ea li-coombs
• Phallo ea cytometry ho lekanya liprotheine tse itseng
• Teko ea Ham (acid hemolysin)
• Serum hemoglobin le haptoglobin
• Teko ea Sucrose hemolysis
• Ho hlahloba urinal
• Moroto hemosiderin, urobilinogen, hemoglobin
• Teko ea LDH
• Palo ea Reticulocyte

Li-steroid kapa meriana e meng e hatellang sesole sa 'mele e ka thusa ho fokotsa ho senyeha ha lisele tse khubelu tsa mali. Ho ka 'na ha hlokahala hore motho a tšeloe mali. Ho fanoa ka tšepe ea tlatsetso le folic acid. Ho fokotsa mali ho ka boela ha hlokahala ho thibela maqeba ho theha.

Soliris (eculizumab) ke sethethefatsi se sebelisetsoang ho phekola PNH. E thibela ho senyeha ha lisele tse khubelu tsa mali.

Ho kenyelletsa 'mele oa masapo ho ka folisa lefu lena. E ka boela ea emisa kotsi ea ho ba le PNH ho batho ba nang le phokolo ea mali.

Batho bohle ba nang le PNH ba lokela ho fumana liente khahlanong le mefuta e itseng ea libaktheria ho thibela tšoaetso. Botsa mofani oa tlhokomelo ea bophelo hore na ke life tse u loketseng.

Phello e fapana. Batho ba bangata ba phela lilemo tse fetang 10 kamora ho fumanoa. Lefu le ka bakoa ke mathata a joalo ka sebopeho sa mali (thrombosis) kapa ho tsoa mali.

Maemong a sa tloaelehang, lisele tse sa tloaelehang li ka fokotseha ha nako e ntse e feta.

Mathata a ka kenyelletsa:

• Lefu la mali le matla haholo
• Phokolo ea mali ea aplastic
• Ho koala mali
• Lefu
• Phokolo ea mali ea hemolytic
• Khaello ea khaello ea tšepe
• Myelodysplasia

Bitsa mofani oa hau haeba u fumana mali ka morong oa hau, haeba matšoao a mpefala kapa a sa ntlafatse kalafo, kapa haeba matšoao a macha a hlaha.

Ha ho na mokhoa o tsebahalang oa ho thibela lefu lena.

PNH

• Lisele tsa mali

Brodsky RA. Paroxysmal bosiu hemoglobinuria. 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 31.

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.