Antenatal management in fetal and neonatal alloimmune thrombocytopenia: a systematic review

Winkelhorst, Dian; Murphy, Michael; Greinacher, Andreas; Shehata, Nadine; Bakchoul, T; Massey, Edwin; Baker, Jennifer M.; Lieberman, Lani; Tanael, Susano; Hume, Heather; Arnold, Donald M.; Baidya, Shoma; Bertrand, Gérald; Bussel, James B.; Kjær, Mette; Kaplan, C.; Kjeldsen‐Kragh, Jens; Oepkes, Dick; Ryan, Greg

doi:10.1182/blood-2016-10-739656

Cited by 102 publications

(112 citation statements)

References 47 publications

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“…IVIg should be offered to women whose pregnancies are at risk of FNAIT. There are no randomised controlled trials (RCTs) comparing IVIg to placebo or invasive management with platelet intrauterine transfusion (IUT): all evidence is based on case series . Following one pregnancy affected by FNAIT, maternal IVIg would normally be first‐line therapy in the next pregnancy at 1 g/kg/week from 18 weeks of gestation .…”

Section: Gestation At Which To Start Ivigmentioning

confidence: 99%

Prenatal Management of Pregnancies at Risk of Fetal Neonatal Alloimmune Thrombocytopenia (FNAIT)

Regan¹,

Lees²,

Jones³

et al. 2019

BJOG

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Plain language summary What is it? Fetal neonatal alloimmune thrombocytopenia (FNAIT), also known as neonatal alloimmune thrombocytopenia (NAIT) or fetomaternal alloimmune thrombocytopenia (FMAIT), is a rare condition which affects a baby's platelets. This can put them at risk of problems with bleeding, particularly into the brain. One baby per week in the UK may be seriously affected and milder forms can affect one in every 1000 births. How is it caused? Platelets are blood cells that are very important in helping blood to clot. All platelets have natural proteins on their surface called human platelet antigens (HPAs). In babies, half of these antigens are inherited from the mother and half from the father. During pregnancy, some of the baby's platelets can cross into the mother's bloodstream. In most cases, this does not cause a problem. But in cases of FNAIT, the mother's immune system does not recognise the baby's HPAs that were inherited from the father and develops antibodies, which can cross the placenta and attack the baby's platelets. These antibodies are called anti‐HPAs, and the commonest antibody implicated is anti‐HPA‐1a, but there are other rarer antibody types. If this happens, the baby's platelets may be destroyed causing their platelet count to fall dangerously low. If the platelet count is very low there is a risk to the baby of bleeding into their brain before they are born. This is very rare but if it happens it can have serious effects on the baby's health. How is it inherited? A baby inherits half of their HPAs from its mother and half from its father. Consequently, a baby may have different HPAs from its mother. As the condition is very rare, and even if the baby is at risk of the condition we have no way of knowing how severely they will be affected, routine screening is not currently recommended. What can be done? FNAIT is usually diagnosed if a previous baby has had a low platelet count. The parents are offered blood tests and the condition can be confirmed or ruled out. There are many other causes of low platelets in babies, which may also need to be tested for. As the condition is so rare, expertise is limited to specialist centres and normally a haematologist and fetal medicine doctor will perform and interpret the tests together. Fortunately, there is an effective treatment for the vast majority of cases called immunoglobulin, or IVIg. This ‘blood product’ is given intravenously through a drip every week to women at risk of the condition. It may be started from as early as 16 weeks in the next pregnancy, until birth, which would be offered at around 36–37 weeks. Less common treatments that may be considered depending on individual circumstances include steroid tablets or injections, or giving platelet transfusions to the baby. What does this paper tell you? This paper considers the latest evidence in relation to treatment options in the management of pregnancies at risk of FNAIT. Specifically, we discuss the role of screening, when IVIg should be started, what dose should be used, an...

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Section: Gestation At Which To Start Ivigmentioning

confidence: 99%

Prenatal Management of Pregnancies at Risk of Fetal Neonatal Alloimmune Thrombocytopenia (FNAIT)

Regan¹,

Lees²,

Jones³

et al. 2019

BJOG

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“…The diagnosis and management of neonatal/fetal thrombocytopenia are considered in detail elsewhere. 23 …”

Section: Neonatal Outcomementioning

confidence: 99%

Thrombocytopenia in pregnancy

Cines¹,

Levine

2017

Blood

134

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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“…In addition, ICHs caused by FNAIT have a high recurrence rate, in up to 79% of subsequent pregnancies [8]. Therefore, in the absence of population-based screening for FNAIT, current management is focused on preventing the occurrence of bleeding complications and ICHs in subsequent pregnancies through antenatal treatment with intravenous immunoglobulin (IVIG) [9]. ICH is estimated to occur in 1 out of 25,000 pregnancies and in 1 out of 10 cases of severe FNAIT [10].…”

Section: Introductionmentioning

confidence: 99%

Perinatal Outcome and Long-Term Neurodevelopment after Intracranial Haemorrhage due to Fetal and Neonatal Alloimmune Thrombocytopenia

et al. 2018

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Objectives: To evaluate the perinatal and long-term neurodevelopmental outcome in a cohort of children with intracranial haemorrhage (ICH) due to fetal and neonatal alloimmune thrombocytopenia (FNAIT) and to clearly outline the burden of this disease. Subjects and Methods: We performed an observational cohort study and included all consecutive cases of ICH caused by FNAIT from 1993 to 2015 at Leiden University Medical Centre. Neurological, motor, and cognitive development were assessed at a minimum age of 1 year. The primary outcome was adverse outcome, defined as perinatal death or severe neurodevelopmental impairment (NDI). Severe NDI was defined as any of the following: cerebral palsy (Gross Motor Function Classification System [GMFCS] level ≥II), bilateral deafness, blindness, or severe motor and/or cognitive developmental delay (<–2 SD). Results: In total, 21 cases of ICH due to FNAIT were included in the study. The perinatal mortality rate was 10/21 (48%). Long-term outcome was assessed in 10 children (n = 1 lost to follow-up). Severe and moderate NDI were diagnosed in 6/10 (60%) and 1/10 (10%) of the surviving children. The overall adverse outcome, including perinatal mortality or severe NDI, was 16/20 (80%). Conclusions: The risk of perinatal death or severe NDI in children with ICH due to FNAIT is high. Only screening and effective preventive treatment can avoid this burden.

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Antenatal management in fetal and neonatal alloimmune thrombocytopenia: a systematic review

Cited by 102 publications

References 47 publications

Prenatal Management of Pregnancies at Risk of Fetal Neonatal Alloimmune Thrombocytopenia (FNAIT)

Prenatal Management of Pregnancies at Risk of Fetal Neonatal Alloimmune Thrombocytopenia (FNAIT)

Thrombocytopenia in pregnancy

Perinatal Outcome and Long-Term Neurodevelopment after Intracranial Haemorrhage due to Fetal and Neonatal Alloimmune Thrombocytopenia

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