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Mielodisplastični sindrom s pridobljeno korpuskularno hemolitično anemijo in kasnejšim prehodom v AML - prikaz primera = Myelodysplastic syndrome with acquired corpuscular haemolytic anaemia and later transition in AML - case report

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  • "Myelodysplastic syndrome with acquired corpuscular haemolytic anaemia and later transition in AML - case report"@sl

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  • "Izhodišča. Pridobljena korpuskularna hemolitična anemija je pri mielodisplastičnem sindromu neobičajen pojav. Vzrok za hemolizo je lahko pridobljeno pomanjkanje encima piruvat kinaze ali pridobljena bolezen hemoglobina H. Poročila, ki opisujejo korpuskularno hemolitično anemijo kot predhodnico akutne mieloblastne levkemije, so zelo redka. Prikaz bolnika. Pri 59-letnem bolniku so odkrili anemijo. Pred tem je bil bolnik ves čas zdrav. S kasnejšimi preiskavami smo ugotovili, da gre sočasno za pridobljeno korpuskularno hemolitično anemijo in mielodisplastični sindrom. Pri iskanju vzroka za hemolizo smo izključili večino možnih vzrokov, razen pomanjkanja piruvat kinaze. Deset mesecev kasneje se je v krvi pojavilo 1-2% blastov. Njihovo število je postopoma naraščalo. S preiskavo kostnega mozga smo ugotovili akutno mieloblastno levkemijo vrste AML-M2 po FAB razvstitvi. Kljub intenzivnemu zdravljenju s citostatiki remisije nismo dosegli in bolnik je 6 mesecev po začetku zdravljenja umrl. Zaključki. Avtor razpravlja o možnih vzrokih za hemolitično anemijo pri mielodisplastičnem sindromu. Pri pregledu literature je našel le nekaj poročil s podobno hematološko sliko. hemolitična anemija je v poteku mielodisplastičnega sindroma zelo redek zaplet."@sl
  • "Background. Acquredcorpuscular haemolytic anaemia may appear as an unusual manifestation of myelodysplastic syndrome. The underlying cause for red cell destruction are acquired severe pyruvate kinase deficiency or acquired haemoglobin H disease. Only very few papers describe acquired corpuscular haemolytic anaemia as an early symptom of acute leucoblastic leukaemia. Methods, results. A 59-year-old previously healthy man was found to have haemolytic anaemia. Further studies revealed a myelodysplastic syndrome and acquired corpuscular haemolytic anaemia. The underlying cause for the red celldestruction was not found, but pyruvate kinase was not evaluated. Ten months later 1-2% of blast cells in the peripheral blood were noted which increased progressively, Subsequently a diagnosis of acute myeloblastic leukaemia M2 was made according to the FAB co-operative group criteria. Although intensive chemotherapy was performed, the patient did not achieve remission and died 6 months later. Conclusions. The author discusses possible causes for underlying red cell defect. A review of literature reveals very fewprevious reports with this haematological picture. Hoemolytic anaemia is anunusual manifestation of myelodysplastic syndrome."@sl

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  • "Mielodisplastični sindrom s pridobljeno korpuskularno hemolitično anemijo in kasnejšim prehodom v AML - prikaz primera = Myelodysplastic syndrome with acquired corpuscular haemolytic anaemia and later transition in AML - case report"@sl