Postpartum thrombotic complication in a patient with paroxysmal nocturnal hemoglobinuria

Gessoni, Gianluca; Canistro, Rosa; Bergamini, Luca; Valverde, Sara; Gessoni, Francesca; Nani, Giovanna; Beggio, Samanta; Spillare, Pietro; Tregnaghi, Alberto

doi:10.1097/mbc.0000000000000250

Cited by 6 publications

(3 citation statements)

References 22 publications

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“…[ 22 ] The high possibility of thrombosis is likely related to pregnancy physiological changes such as increase in complement activity and hypercoagulability state and also with the pathophysiology of PNH that might augment the risk of emergency delivery. [ 18 22 29 34 ] Thrombosis is associated with serious complications for the mother and the fetus, and thus obstetrician experts are involved in care of pregnant patients with PNH. [ 4 17 ]…”

Section: Discussionmentioning

confidence: 99%

Pregnancy with Paroxysmal Nocturnal Hemoglobinuria

Al-Dosari¹,

Al-Zahrani

Almohareb

et al. 2021

Saudi Journal of Medicine &Amp; Medical Sciences

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Paroxysmal nocturnal hemoglobinuria (PNH) is a rare, acquired hematopoietic stem cell disorder, and eculizumab and ravulizumab are its two approved therapies. Only few case series/reports have reported the outcomes of pregnancies in patients with PNH despite the increased risk of thrombosis. Similarly, there is limited knowledge regarding the effect of the approved treatments on conception and pregnancy outcomes. Here, we report the first series of pregnancies in PNH patients from the Middle Eastern region from our tertiary care hospital. Ten pregnancies in four females after diagnosis with PNH were identified. In terms of PNH management, only eculizumab was used, as the safety of ravulizumab use in pregnancies has not yet been established. In the antepartum period, the patients had variable symptoms that ranged from mild symptoms including epistaxis, tea-colored urine and vaginal bleeding to life-threatening vessel thrombosis. Further, red blood cell and platelet transfusions were required because of bleeding and hemolysis in four pregnancies. The pregnancy outcomes varied, but based on these, the safety of eculizumab use during pregnancy remained inconclusive. The postpartum period was complicated in one case by portal vein thrombosis and was managed accordingly. In conclusion, pregnant females with PNH are at an increased risk for complications due to PNH, and thus experienced hematologists and obstetricians should be involved jointly in their care.

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Section: Discussionmentioning

confidence: 99%

Pregnancy with Paroxysmal Nocturnal Hemoglobinuria

Al-Dosari¹,

Al-Zahrani

Almohareb

et al. 2021

Saudi Journal of Medicine &Amp; Medical Sciences

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“…The literature review showed only 15 studies [8,[17][18][19][20][21][22][23][24][25][26][27][28][29], including 24 pregnancies. Pooled data showed a high rate of caesarean delivery (57.1%), admission to neonatal intensive care unit (NICU) (8.7%), and preterm birth (83%).…”

Section: Main Findingsmentioning

confidence: 99%

Pregnancy outcome of women with Paroxysmal Nocturnal Hemoglobinuria

Zullo¹,

Gragnano²,

Saccone³

et al. 2023

It Journ Gyn Obs

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Objective. To evaluate maternal and perinatal outcomes in pregnant women affected by Paroxysmal Nocturnal Hemoglobinuria (PNH) treated with eculizumab with a case series and literature review. Materials and Methods. This was a case series study with literature review. Clinical records of all consecutive pregnant women with PNH were included in the study. The systematic review was conducted using electronic databases from inception of each database through May 2021. No restrictions for language or geographic location were applied. All reports of women with PNH in pregnancy, treated with eculizumab, were included in the review. Reports of women not treated or treated with other drugs rather than eculizumab (e.g., low molecular weight heparin alone) were excluded from the study. Maternal and perinatal outcomes were evaluated. Results. Fifteen studies, including 24 pregnancies with PNH, were included in the review. All included women received eculizumab during the pregnancy, of them 10 received the treatment for the entire length of their gestation. Gestational age at delivery was reported in 18 cases, with preterm birth at less than 37 weeks occurring in fifteen women (83%). 57.1% of the women delivered by caesarean delivery. The cases series added three new cases in the literature. Two cases were already on eculizumab before pregnancy, while in one case eculizumab therapy was initiated in the second trimester of pregnancy. In all the three cases, there were no thrombotic complications, maternal or neonatal deaths, or foetal structural abnormalities. Conclusions. PNH in pregnant women may be associated with an increased risk of obstetric complications, such us caesarean delivery or preterm birth. Eculizumab appears to be safe and effective for managing PNH during pregnancy.

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“…The published series differ regarding the timing of the introduction of eculizumab, administered doses, infusion frequencies, and the type of anticoagulation used. [11][12][13][14][15][16][17][18]…”

Section: Introductionmentioning

confidence: 99%

Eculizumab treatment in pregnant women with paroxysmal nocturnal hemoglobinuria: A Polish experience

Czyż¹,

Szukalski²,

Szukalska³

et al. 2022

Adv Clin Exp Med

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Background.Eculizumab is an antibody targeting the C5 complement protein. Clinical trials suggest that eculizumab significantly reduces transfusion requirements and prevents disease complications in patients with paroxysmal nocturnal hemoglobinuria (PNH). Objectives.To analyze the outcome of pregnancies among Polish women with PNH treated with eculizumab as a part of the Polish National Health Fund program. Materials and methods.We report the outcomes of 3 pregnancies among women treated with eculizumab between 2017 and 2020. For 1 of these woman, it was the 1 st pregnancy, while the remaining 2 patients had previously had 1 previous successful pregnancy each.Results. All 3 mothers survived pregnancy, and all children were born alive. One of the patients had a vaginal delivery. Another required cesarean delivery at the 34 th week due to a decreasing platelet count. In 1 case, premature rupture of the fetal membranes occurred at week 36, followed by artificial labor induction. All children were born without any inborn defects. The 2 prematurely born babies required a prolonged hospital stay.Conclusions. Treatment with eculizumab seems to reduce the risk to a mother and a child associated with PNH. However, more data are necessary to confirm this notion.

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Postpartum thrombotic complication in a patient with paroxysmal nocturnal hemoglobinuria

Cited by 6 publications

References 22 publications

Pregnancy with Paroxysmal Nocturnal Hemoglobinuria

Pregnancy with Paroxysmal Nocturnal Hemoglobinuria

Pregnancy outcome of women with Paroxysmal Nocturnal Hemoglobinuria

Eculizumab treatment in pregnant women with paroxysmal nocturnal hemoglobinuria: A Polish experience

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