Clinical Management of Paroxysmal Nocturnal Hemoglobinuria in Pregnancy: A Case Report and Updated Review

Abstract

After completion of this article, the reader should be able to recall that paroxysmal nocturnal hemoglobinuria (PNH) during pregnancy increases adverse events for both the mother and the fetus, state that maternal and fetal mortality are both high, and explain that the major complications occur in the postpartum period.

Paroxysmal nocturnal hemoglobinuria and pregnancy before the eculizumab era: the French experience

“…Another possible explanation for the high rate of caesarian deliveries is the PNH-related smooth muscle dystonia that may compromise the progress of labor in PNH patients. Fetal mortality was 4%, which is lower than the 7.2% reported by Fieni et al 4 In our study, newborn birth weight was generally low, though overall outcome was favorable for these children.…”

“…Thromboses occurred in patients who received anticoagulation as well as in patients who did not, confirming that prophylactic antithrombotic therapy is not always sufficient to prevent thromboembolism in PNH patients. 24 In comparison with previous reports, 4,28 we recorded a high incidence of premature delivery and caesarian section. A planned and therapeutically induced delivery was preferred in the vast majority of cases to improve management of the patient during this particularly risky period.…”

“…[25][26] Among 43 pregnancies reported in the medical literature between 1965 and 2006, 8 were associated with thrombosis. Two incidents of thrombosis occurred during pregnancy 5,19 and the remaining 6 occurred during the postpartum period, 4,7,19,[21][22]27 leading to maternal death in 3 cases. 19,21,27 In our series, we recorded no thrombotic events during pregnancy, possibly because most (64%) of our patients received antithrombotic prophylaxis.…”

“…To date, fewer than 30 reports [4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21][22] have been published, comprising case reports or small series of no more than 7 patients. Fieni et al 4 reviewed obstetric literature about pregnancy and PNH published between 1965 and 2005. They assessed 43 cumulative cases which revealed maternal and fetal mortality rates of 11.6% and 7.2%, respectively.…”

“…Although there is little information about PNH and pregnancy in the literature, a recent review identified 23 clinical reports published between 1965 and 2005 describing pregnancy outcomes in 43 women with PNH. 4 Maternal and fetal mortality rates were 11.6% and 7.2%, respectively. Venous thrombotic events (VTEs) were the major cause of maternal death.…”