Paroxysmal ozizira hemoglobinuria (PCH)

Paroxysmal ozizira hemoglobinuria (PCH) ndi matenda osowa magazi omwe chitetezo chamthupi chimatulutsa ma antibodies omwe amawononga maselo ofiira. Zimachitika munthuyu akakumana ndi kutentha kwazizira.

PCH imangochitika kuzizira, ndipo imakhudza makamaka manja ndi mapazi. Ma antibodies amamatira (kumanga) kumaselo ofiira ofiira. Izi zimalola mapuloteni ena m'magazi (otchedwa complement) kuti nawonso atseke. Ma antibodies amawononga maselo ofiira akamayenda mthupi. Maselowo akawonongedwa, hemoglobin, yomwe ndi mbali ya maselo ofiira omwe amatenga mpweya, imatulutsidwa m'magazi ndikupita mkodzo.

PCH yakhala ikugwirizanitsidwa ndi syphilis yachiwiri, syphilis yapamwamba, ndi matenda ena a ma virus kapena bakiteriya. Nthawi zina chifukwa chake sichidziwika.

Matendawa ndi osowa.

Zizindikiro zimaphatikizapo:

• Kuzizira
• Malungo
• Ululu wammbuyo
• Kupweteka kwa mwendo
• Kupweteka m'mimba
• Mutu
• Kusapeza bwino, kusakhazikika, kapena kudwala (malaise)
• Magazi mu mkodzo (mkodzo wofiira)

Mayeso a labotale atha kuthandiza kuzindikira izi.

• Magulu a Bilirubin amakhala ndi magazi ndi mkodzo wambiri.
• Kuwerengera magazi kwathunthu (CBC) kumawonetsa kuchepa kwa magazi.
• Mayeso a Coombs ndiosalimbikitsa.
• Mayeso a Donath-Landsteiner ndiabwino.
• Mulingo wa lactate dehydrogenase ndiwokwera.

Kuthana ndi vutoli kungathandize. Mwachitsanzo, ngati PCH imayambitsidwa ndi syphilis, zizindikilo zimatha kukhala bwino pamene chindoko chimathandizidwa.

Nthawi zina, mankhwala omwe amaletsa chitetezo cha mthupi amagwiritsidwa ntchito.

Anthu omwe ali ndi matendawa nthawi zambiri amachira msanga ndipo samakhala ndi zizindikilo pakati pazigawo. Nthawi zambiri, ziwopsezo zimatha posachedwa pomwe ma cell owonongeka amasiya kuyenda mthupi.

Zovuta zingaphatikizepo:

• Kupitiliza kuukiridwa
• Impso kulephera
• Kuchepa kwa magazi m'thupi

Itanani omwe akukuthandizani ngati muli ndi zodabwitsazi. Wothandizirayo atha kuyambitsa zina zomwe zimayambitsa zizindikirazo ndikusankha ngati mukufuna chithandizo.

Anthu omwe adapezeka kuti ali ndi matendawa amatha kupewa ziwopsezo zamtsogolo popewa kuzizira.

PCH

• Maselo amwazi

Michel M. Autoimmune ndi intravascular hemolytic anemias. Mu: Goldman L, Schafer AI, olemba. Mankhwala a Goldman-Cecil. 26 wa. Philadelphia, PA: Elsevier; 2020: chap 151.

Win N, Richards SJ. Anapeza haemolytic anaemias. Mu: Bain BJ, Bates I, Laffan MA, eds. Dacie ndi Lewis Othandiza Hematology. Wolemba 12. Philadelphia, PA: Elsevier; 2017: mutu 13.