Treatment of paroxysmal nocturnal hemoglobinuria in pregnancy with eculizumab: A case report

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare disease caused by mutations in hematopoietic stem cells leading to pancytopenia and a predisposition for thromboembolic events. In pregnancy, these manifestations can be amplified, leading to increased neonatal and maternal morbidity and mortality. Although data are limited, eculizumab has emerged as a potential treatment of PNH in pregnancy. This report describes a case of a woman with PNH successfully treated with eculizumab during two pregnancies. Although during both pregnancies she experienced breakthrough hemolysis requiring intermittent blood transfusions, she had no thromboembolic events and had term vaginal births. Granted more research is needed, eculizumab may be an acceptable therapy for PNH in pregnancy.

Diagnóstico de Hemoglobinúria Paroxística Noturna em gestante que já cursava com trombocitopenia há 04 anos: Relato de Caso

“…Esta doença aumenta as chances de complicações materno-fetais, originando uma maior mortalidade com taxa variando em torno de 10% (Guibert et al, 2009). As mais conhecidas associações são: fetos pequenos para a idade gestacional, prematuridade, morte fetal, pré-eclâmpsia e eclâmpsia, HELLP, hemólise, infecções, citopenias e trombose (Fassett, 2021;Kelly et al, 2014). Os eventos tromboembólicos assumem o papel de destaque como a principal complicação para esse contexto, sendo mais evidenciado no período pós-parto e em mulheres jovens.…”