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Screening in Pregnancy for Fetal Or Neonatal Alloimmune Thrombocytopenia: Systematic Review

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Screening in Pregnancy for Fetal Or Neonatal Alloimmune Thrombocytopenia: Systematic Review DOI: 10.1111/j.1471-0528.2010.02657.x Systematic review www.bjog.org Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia: systematic review MM Kamphuis,a N Paridaans,a L Porcelijn,b M De Haas,c CE van der Schoot,c A Brand,d GJ Bonsel,e D Oepkesa a Department of Obstetrics, Leiden University Medical Centre, Leiden b Sanquin Diagnostic Services, Amsterdam c Department of Experimental Immunohematology, Sanquin Research and Landsteiner Laboratory, Academic Medical Centre, University of Amsterdam, Amsterdam d Department of Immunohematology, Leiden University Medical Centre, Leiden e Department of Obstetrics and Prenatal Care, Erasmus Medical Centre, Rotterdam University, the Netherlands Correspondence: D Oepkes, Leiden University Medical Centre, Department of Obstetrics, K6-32, Box 9600, 2300 RC Leiden, the Netherlands. Email [email protected] Accepted 7 June 2010. Published Online 8 July 2010. Background Fetal and neonatal alloimmune thrombocytopenia data were prevalence of HPA-1a negativity, HPA-1a (FNAIT) is a potentially devastating disease, which may lead immunisation, platelet count at birth and perinatal ICH. We to intracranial haemorrhage (ICH), with neurological damage as aimed to compare outcome with and without intervention. a consequence. In the absence of screening, FNAIT is only Main results HPA-1a alloimmunisation occurred in 294/3028 diagnosed after bleeding symptoms, with preventive options (9.7%) pregnancies at risk. Severe FNAIT occurred in 71/227 limited to a next pregnancy. (31%) immunised pregnancies, with perinatal ICH in 7/71 (10%). Objectives To estimate the population incidence of FNAIT and True natural history data were not found because interventions its consequences to prepare for study design of a screening were performed in most screen-positive women. programme. Authors’ conclusions Screening for HPA-1a alloimmunisation Search strategy An electronic literature search using MEDLINE, detects about two cases in 1000 pregnancies. The calculated risk EMBASE and Cochrane database, and references of retrieved for perinatal ICH of 10% in pregnancies with severe FNAIT is articles. No language restrictions were applied. an underestimation because studies without interventions were lacking. Screening of all pregnancies together with effective Selection criteria Prospective studies on screening for human antenatal treatment such as intravenous immunoglobulin may platelet antigen 1a (HPA-1a) alloimmunisation in low-risk reduce the mortality and morbidity associated with FNAIT. pregnant women. Keywords Fetal and neonatal alloimmune thrombocytopenia, Data collection and analysis Two reviewers independently fetus, intracranial haemorrhage, pregnancy, screening. assessed studies for inclusion and extracted data. Main outcome Please cite this paper as: Kamphuis M, Paridaans N, Porcelijn L, De Haas M, van der Schoot C, Brand A, Bonsel G, Oepkes D. Screening in pregnancy for fetal or neonatal alloimmune thrombocytopenia: systematic review. BJOG 2010;117:1335–1343. Introduction 85% of immunisations are caused by alloantibodies against HPA-1a. Older literature suggests that most immunisations Fetal and neonatal alloimmune thrombocytopenia (FNAIT) occur during pregnancy.2–4 Recently, a large prospective is a potentially devastating condition, which may lead to study showed that most immunisations probably occurred intracranial haemorrhage in the fetus or neonate, often during or shortly after birth.5 The immunoglobulin G with death or major neurological damage as a consequence. (IgG) antibodies against fetal HPA-1a can cross the In Caucasians, between 1.6 and 4.6% are negative for placenta and cause destruction of fetal platelets. The chance human platelet antigens (HPA) 1bb or 1a.1 In this group, of immunisation is correlated with maternal expression ª 2010 The Authors Journal compilation ª RCOG 2010 BJOG An International Journal of Obstetrics and Gynaecology 1335 Kamphuis et al. of human leucocyte antigen (HLA) DRB3*0101 type. Typ- form HPA alloantibodies during pregnancy, what propor- ing for this antigen may contribute to identifying the preg- tion of their offspring is affected by FNAIT, and how nancies at risk for FNAIT.6 severely are they affected? Second, what are the effects of Several studies aimed to identify a threshold of HPA- interventions in screen-positive pregnant women? antibody level in the maternal serum below which severe FNAIT would not occur but with conflicting results.7–9 Definitions The reported incidence of FNAIT ranges from 1:35010 to HPA-1a status is defined straightforwardly, but the defini- 1:100011 in the largest studies. The most severe complica- tion of FNAIT manifestations varies in the literature. tion is intracranial haemorrhage (ICH), leading to perinatal Thrombocytopenia, in adults and children as well as in mortality in 1–7% and to surviving children with often fetuses, is defined as a platelet count <150 · 109/l. Severe severe neurological sequelae including mental retardation, thrombocytopenia, associated with an increased risk of cerebral palsy, cortical blindness and seizures in 14–26% of bleeding is commonly defined as a platelet count affected pregnancies.4,12–14 Reviewing all published cases of <50 · 109/l, and therefore has been used in many studies ICH due to FNAIT, Spencer and Burrows found that on FNAIT as a single endpoint. However, only a minority 80.5% originated before birth.4 of fetuses or neonates with such a low platelet count actu- The recurrence rate of FNAIT in a subsequent pregnancy ally suffer from bleeding complications. The key complica- is estimated at 90%.14 Radder et al. reviewed the scarce tion of the disease to be prevented is ICH. However, even literature on ICH risk in subsequent, untreated pregnancies. the use of ICH as an endpoint has become controversial In women with a previous child with ICH, the estimated through the application of ever-improving imaging modali- recurrence risk was between 61 and 97%.15 Furthermore, ties, which now may detect minute areas of haemorrhage similar to red cell alloimmunisation, the severity of FNAIT is that may be clinically irrelevant. The truly meaningful end- assumed to increase with each pregnancy.14,16,17 point would be neurodevelopment, to be assessed later in In the absence of screening, the disease is only diagnosed life, e.g. Bayley score at 2 years of age or later. For our lit- after the birth of symptomatic neonates, i.e. fetal or neonatal erature review, we aimed to identify studies that at least bleeding, or occasionally by chance in the case of neonatal provided information of the incidence of HPA-1a negativ- blood tests for other reasons. As a consequence, ante- ity, HPA alloantibodies, neonatal platelet counts and signs natal treatment is currently provided only for women with a of bleeding in the newborns. As a definition of severe previously affected child. In such a pregnancy, preventive FNAIT we used a platelet count <50 · 109/l. measures, such as fetal blood sampling with platelet transfu- sion or weekly intravenous infusions with immunoglobulins Search and study selection (IVIG), are taken. Until recently, insufficient specificity of Relevant literature was identified using the electronic bib- the diagnostic tests and the risks of treatment precluded the liographic databases PubMed, Embase, and Cochrane. We introduction of population-based screening programmes. used the following keywords: ‘mass screening’ [MESH] OR However, laboratory methods have improved, and IVIG screen* AND ‘Thrombocytopenia, neonatal alloim- treatment without any invasive testing was shown, at least in mune’[MESH] OR NAITP OR Alloimmune thrombocyto- women with a previous affected child, to be both safe and penia. We accepted original articles, short communications effective.18 and letters to the editor. In addition a search was per- As a preparation to introduce a provisional national formed from the reference list of all identified articles. screening programme for FNAIT in a general population of When needed, we contacted authors for additional, unpub- pregnant women, we performed a systematic review of the lished information. There were no language restrictions. literature. We subsequently excluded all non-prospective studies. Studies were excluded when screening was not performed Methods by HPA-typing, or when screening was applied in a specific or high-risk population. Research questions Two of the authors (M.K. and N.P.) initially screened all We aimed to review all prospective screening studies in the titles and abstracts of papers, identified by the review cohorts of low-risk pregnant women, where HPA-typing search strategy, for relevance. Only studies that were obvi- was performed to identify pregnancies at risk for FNAIT. ously irrelevant were excluded at this stage. All other stud- The time range of publication was January 1980 to October ies were assessed on the basis of their full text for inclusion 2008. Study data were pooled to the extent that study pop- versus exclusion by two reviewers independently (M.K. and ulations seemed comparable. The specific research ques- N.P.) using the criteria indicated above. Discrepancies were tions were first: what is the incidence of HPA-1a negativity to be resolved by discussion with a third reviewer but this among pregnant women, what proportion of these women proved to be unnecessary. 1336 ª 2010 The Authors Journal compilation ª RCOG 2010 BJOG An International Journal of Obstetrics and Gynaecology Screening for alloimmune thrombocytopenia Methodological quality
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