Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Dilemma in Treatment and Thromboprophylaxis

Abstract

Paroxysmal nocturnal hemoglobinuria (PNH) is a hematologic disorder characterized by an acquired somatic mutation in the phosphatidylinositol glycan class A gene which leads to a higher risk for increased venous and arterial thrombosis. Current treatment for PNH includes eculizumab. Pregnant patients who have PNH have higher risk for thrombosis and hemorrhage with both pregnancy and their underlying PNH. Treatment frequently poses conundrum. The safety and efficacy of eculizumab during pregnancy and breast feeding have not been extensively studied and contraception has been recommended due to potential for teratogenicity. We present a case of a patient who was safely on both eculizumab and modest prophylactic anticoagulation for 6 weeks post-partum.

Pregnancy with paroxysmal nocturnal hemoglobinuria: A case series with review of the literature

“…The efficacy of anticoagulants in pregnancy with PNH have been supported strongly by Morita et al [ 33 ] and Patel et al [ 27 ] whereas de Guibert et al . [ 19 ] reported some cases with thrombosis even after the use of anticoagulants.…”

Pregnancy in paroxysmal nocturnal hemoglobinuria: from survival to life

“…В случае сочетания прогрессирующей тромбоцитопении с нарастанием печеночных трансаминаз встает вопрос о развитии тяжелого акушерского осложнения -HELLP-синдрома. Длительное время считалось, что гемолиз при ПНГ имеет четкие отличия от HELLP [5,13], однако по мере увеличения количества наблюдений за ходом беременности у пациенток с ПНГ мы сталкиваемся со сложностью дифференциальной диагностики [14]. ПЭ характеризуется локальной дисрегуляцией системы комплемента в плаценте [9,12].…”

Hemoglobinuria paroxística nocturna durante el embarazo: reporte de un caso y revisión de la literatura

“…El embarazo supone per se un estado procoagulante, por lo que asociado a la HPN aumenta significativamente el riesgo de trombosis y coagulación intravascular diseminada. Un dilema terapéutico es el papel de la anticoagulación durante el embarazo para disminuir el riesgo de trombosis (3) . A pesar del alto riesgo de trombosis y muerte, no existen protocolos específicos de anticoagulación durante el embarazo.…”

“…Se ha estimado que la mortalidad materna durante el embarazo y el periodo inmediato posparto es 12 a 21%. Asimismo, existe riesgo de aborto o prematuridad (3) .…”