2010
DOI: 10.1590/s0104-42302010000200022
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Hemoglobinúria paroxística noturna: da fisiopatologia ao tratamento

Abstract: ResumoHemoglobinúria paroxística noturna (HPN) é uma anemia hemolítica crônica adquirida rara, de curso clínico extremamente variável. Apresenta-se frequentemente com infecções recorrentes, neutropenia e trombocitopenia, e surge em associação com outras doenças hematológicas, especialmente com síndromes de falência medular, como anemia aplásica e síndrome mielodisplásica. É considerada ainda um tipo de trombofilia adquirida, apresentando-se com tromboses venosas variadas, com especial predileção por trombose d… Show more

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Cited by 17 publications
(42 citation statements)
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“…The involved different mutations result in the deficient synthesis of glycosylphosphatidylinositol (GPI)-anchor molecule and the resulting partial or absolute deficiency, in all blood cell lineages, of the expression of GPI-anchored membrane proteins, like CD55, CD59, CD14, CD16, CD24, CD48, among others (1,7,15,18) .…”
mentioning
confidence: 99%
“…The involved different mutations result in the deficient synthesis of glycosylphosphatidylinositol (GPI)-anchor molecule and the resulting partial or absolute deficiency, in all blood cell lineages, of the expression of GPI-anchored membrane proteins, like CD55, CD59, CD14, CD16, CD24, CD48, among others (1,7,15,18) .…”
mentioning
confidence: 99%
“…After careful review of the case, it was observed that the patient had positive markers of hemolysis (very high LDH, indirect hyperbilirubinemia, reticulocytosis) and nonreactive direct Coombs. [3,4,5] Due to the presence of hemolytic anemia of unknown cause, there was the decision to investigate the possibility of paroxysmal nocturnal hemoglobinuria. The diagnostic investigation proceeded with the request of a Flow Cytometry (FCM) gold standard test for diagnosis of the PNH.…”
Section: Discussionmentioning
confidence: 99%
“…El loxocelismo produce nefrotoxicidad por hemoglobinuria en casos de afectación sistémica 9 , pero se puede descartar porque no existe el hallazgo de las lesiones cutáneas características ni el antecedente de la mordedura de araña. La hemoglobinuria paroxística nocturna puede desencadenar crisis hemolíticas intravasculares masivas (como en este caso), pero se asocia a ausencia de marcación para CD55 Y CD59 10 , descartada en la citometría de flujo de sangre periférica.…”
Section: Discussionunclassified