2017
DOI: 10.29046/tmf.018.1.008
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Case Report: Paroxysmal Nocturnal Hemoglobinuria

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Cited by 2 publications
(3 citation statements)
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“…PNH is a rare clonal haematologic disorder caused by a somatic mutation in the PIGA gene located on the Xp22.2 chromosome 17 , leading to a deficiency of GPI anchor proteins in the blood cells rendering the cells susceptible to complement-mediated lysis 4 . The clinical manifestations of PNH are characterised by a triad of features: haemolytic anaemia, thrombosis, and impaired bone marrow function 18 . Our patient had initially presented with symptoms such as haematuria, dark-coloured urine and abdominal pain, which raised suspicion of PNH 19 .…”
Section: Discussionmentioning
confidence: 99%
“…PNH is a rare clonal haematologic disorder caused by a somatic mutation in the PIGA gene located on the Xp22.2 chromosome 17 , leading to a deficiency of GPI anchor proteins in the blood cells rendering the cells susceptible to complement-mediated lysis 4 . The clinical manifestations of PNH are characterised by a triad of features: haemolytic anaemia, thrombosis, and impaired bone marrow function 18 . Our patient had initially presented with symptoms such as haematuria, dark-coloured urine and abdominal pain, which raised suspicion of PNH 19 .…”
Section: Discussionmentioning
confidence: 99%
“…The classical triad of PNH is hemolytic anemia, recurrent thrombosis, and impaired bone marrow functions [2]. These three impairments can be seen in different ratios and severities.…”
Section: Discussionmentioning
confidence: 99%
“…PIGA gene is localized on the X chromosome. In defective synthesis, the levels of CD55 [(decay accelerating factor) accelerates the decay of preformed C3 convertase] and CD59 [(membrane inhibitor of reactive lysis) protects the body from the effects of membrane-attack complexes by downregulating them] are reduced and erythrocytes become susceptible to hemolysis [2,3].…”
Section: Discussionmentioning
confidence: 99%