Innovative therapeutic approach: Sequential treatment with plasma exchange and eculizumab in a pregnant woman affected by atypical hemolytic-uremic syndrome

Mussoni, Maria Pia; Veneziano, Francesco Antonio; Boetti, L.; Tassi, Cristina; Calisesi, C.; Nucci, S; Rigotti, Angelo; Panzini, Ilaria; Ardissino, Gianluigi

doi:10.1016/j.transci.2014.08.027

Cited by 20 publications

(14 citation statements)

References 8 publications

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“…Exposure to eculizumab during pregnancy was associated with a comparable rate of live births and a low rate of maternal complications in contrast to reports of elevated maternal mortality rates observed among patients with PNH not receiving eculizumab (Kelly et al , ). In aHUS, use of eculizumab in pregnancy and post‐partum is associated with good maternal and fetal outcomes, including normalization of haematological outcomes and renal function (Ardissino et al , ; Delmas et al , ; Zschiedrich et al , ; Canigral et al , ; Mussoni et al , ; De Sousa Amorim et al , ; Demir et al , ; Saad et al , ; Servais et al , ; Tsai & Kuo, ; Andries et al , ; Bruel et al , ; Chua et al , ; Gately et al , ). The rate of congenital malformation reported in neonates born to mothers treated with eculizumab lies within the range of that reported in the general population (UK Teratology Information Service, ).…”

Section: Discussionmentioning

confidence: 99%

Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10‐year pharmacovigilance analysis

et al. 2019

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Summary Eculizumab is the first and only medication approved for paroxysmal nocturnal haemoglobinuria (PNH) and atypical haemolytic uraemic syndrome (aHUS) treatment. However, eculizumab safety based on long‐term pharmacovigilance is unknown. This analysis summarises safety data collected from spontaneous and solicited sources from 16 March 2007 through 1 October 2016. Cumulative exposure to eculizumab was 28 518 patient‐years (PY) (PNH, 21 016 PY; aHUS, 7502 PY). Seventy‐six cases of meningococcal infection were reported (0·25/100 PY), including eight fatal PNH cases (0·03/100 PY). Susceptibility to meningococcal infections remained the key risk in patients receiving eculizumab. The meningococcal infection rate decreased over time; related mortality remained steady. The most commonly reported serious nonmeningococcal infections were pneumonia (11·8%); bacteraemia, sepsis and septic shock (11·1%); urinary tract infection (4·1%); staphylococcal infection (2·6%); and viral infection (2·5%). There were 434 reported cases of eculizumab exposure in pregnant women; of 260 cases with known outcomes, 70% resulted in live births. Reporting rates for solid tumours (≈0·6/100 PY) and haematological malignancies (≈0·74/100 PY) remained stable over time. No new safety signals affecting the eculizumab benefit‐risk profile were identified. Continued awareness and implementation of risk mitigation protocols are essential to minimise risk of meningococcal and other Neisseria infections in patients receiving eculizumab.

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Section: Discussionmentioning

confidence: 99%

Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10‐year pharmacovigilance analysis

et al. 2019

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“…In a study of 75 pregnancies in 61 women with paroxysmal nocturnal hemoglobinuria (PNH) treated with eculizumab during pregnancy and postpartum, fetal mortality rates were not increased [38]. In these patients, eculizumab was present at low levels in 35 % of cord blood samples, but not in breast milk [42].…”

Section: Discussionmentioning

confidence: 99%

Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab

2016

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Atypical hemolytic uremic syndrome (aHUS) is a rare, genetic, progressive, life-threatening form of thrombotic microangiopathy (TMA) predominantly caused by dysregulation of the alternative pathway of the complement system. Complement-amplifying conditions (CACs), including pregnancy complications [preeclampsia, HELLP (hemolysis, elevated liver enzymes, low platelet count) syndrome], malignant hypertension, autoimmune diseases, transplantation, and others, are associated with the onset of TMA in up to 69 % of cases of aHUS. CACs activate the alternative pathway of complement and may be comorbid with aHUS or may unmask a previously undiagnosed case. In this review, three case reports are presented illustrating the onset and diagnosis of aHUS in the setting of different CACs (pregnancy complications, malignant hypertension, renal transplantation). The report also reviews the evidence for a variety of CACs, including those mentioned above as well as infections and drug-induced TMA, and the overlap with aHUS. Finally, we introduce an algorithm for diagnosis and treatment of aHUS in the setting of CACs. If TMA persists despite initial management for the specific CAC, aHUS should be considered. The terminal complement inhibitor eculizumab should be initiated for all patients with confirmed diagnosis of aHUS, with or without a comorbid CAC.

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“…48 Eculizumab has been used safely during pregnancy in women with paroxysmal nocturnal hemoglobinemia 49 and in a more limited number of women with aHUS. 50 Fetal levels of this IgFcg2/4 hybrid antibody are low and no fetal harm has been reported, nor has the drug been detected in breast milk. 49 Pregnancy and apheresis increase drug requirements.…”

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confidence: 99%

Thrombocytopenia in pregnancy

Cines¹,

Levine

2017

Blood

127

137

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Thrombocytopenia develops in 5% to 10% of women during pregnancy or in the immediate postpartum period. A low platelet count is often an incidental feature, but it might also provide a biomarker of a coexisting systemic or gestational disorder and a potential reason for a maternal intervention or treatment that might pose harm to the fetus. This chapter reflects our approach to these issues with an emphasis on advances made over the past 5 to 10 years in understanding and managing the more common causes of thrombocytopenia in pregnancy. Recent trends in the management of immune thrombocytopenia translate into more women contemplating pregnancy while on treatment with thrombopoietin receptor agonists, rituximab, or mycophenylate, which pose known or unknown risks to the fetus. New criteria to diagnose preeclampsia, judicious reliance on measurement of ADAMTS13 to make management decisions in suspected thrombotic thrombocytopenic purpura, new evidence supporting the efficacy and safety of anticomplement therapy for atypical hemolytic uremic syndrome during pregnancy, and implications of thrombotic microangiopathies for subsequent pregnancies are evolving rapidly. The goals of the chapter are to help the hematology consultant work through the differential diagnosis of thrombocytopenia in pregnancy based on trimester of presentation, severity of thrombocytopenia, and coincident clinical and laboratory manifestations, and to provide guidance for dealing with some of the more common and difficult diagnostic and management decisions.

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Innovative therapeutic approach: Sequential treatment with plasma exchange and eculizumab in a pregnant woman affected by atypical hemolytic-uremic syndrome

Cited by 20 publications

References 8 publications

Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10‐year pharmacovigilance analysis

Eculizumab in paroxysmal nocturnal haemoglobinuria and atypical haemolytic uraemic syndrome: 10‐year pharmacovigilance analysis

Atypical hemolytic uremic syndrome in the setting of complement-amplifying conditions: case reports and a review of the evidence for treatment with eculizumab

Thrombocytopenia in pregnancy

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