International Practices in Managing Preconception, Pregnancy and Childbirth in Women With Glanzmann Thrombasthenia: A Survey From the European Association of Haemophilia and Allied Disorders (EAHAD).

The authors have nothing to report.

The GTWG and the WGBD+WG of EAHAD worked together to design a survey on clinical practices in the management of the preconception phase, pregnancy and childbirth in women with GT. The survey was designed by two investigators (KG and KR). Relevant literature was reviewed to identify important items and knowledge gaps. The survey was peer reviewed in two rounds by the other authors to assess content, clarity and clinical relevance. This survey was carried out using Survey Monkey and was promoted during EAHAD congress 2025 by distributing flyers and via LinkedIn through the EAHAD platform. Additionally, an email was sent to 72 respondents of the previous GT survey conducted by the GTWG of EAHAD. Healthcare professionals involved in the care of women with GT were invited to respond to the survey. Respondents were asked to complete 30 questions, covering respondent demographics, clinical experience and proposed management in relation to any delay in conceiving, HMB while trying to conceive, screening for platelet alloimmunisation and optimal treatment to prevent PPH at delivery. The survey remained open from February first 2025 until 15 April 2025. The survey questionnaire is available in Supplement 1 . Minimally completed survey responses, defined as having fewer than five answers, were excluded from the analysis. Partially completed surveys, in which the majority of questions were answered, and fully completed surveys were included. The data was analysed using IBM SPSS Statistics version 29.0.1. Descriptive statistics are presented as frequencies and percentages.

After excluding three incomplete responses (≤ 5 answers), the final sample comprised 37 respondents from 18 countries, of which 32 respondents fully completed the survey. The geographic distribution of survey respondents is shown in Figure  1 . The majority of the respondents were haematologists treating adults (27/37, 73%). Nineteen percent (7/37) were haematologists treating children and 3% (1/37) treated both adults and children. One respondent was a physician specialised in internal medicine, and another was a nurse. Only 13 respondents (35%) managed a pregnancy in a woman with GT in the past 10 years, whereas 25 respondents (68%) took care of woman with an active wish to become pregnant. Of the respondents who previously managed a pregnancy in a woman with GT, the median number of pregnancies was 2 (range 1–10). Detailed respondent characteristics are shown in Supplement 2 . Geographical distribution of survey respondents within Europe. Ninety‐five percent (35/37) of the respondents would recommend preconception counselling for women with GT. A joint clinic—a clinic where the patient is seen by both an obstetrician and gynaecologist and a haematologist at the same time—is available in only 24% (9/37) of the centres represented by the respondents. Obstetrics and gynaecology service with an interest in bleeding disorders was available in 65% (24/37), of which 16% (6/37) was available at another centre. Eleven percent (4/37) of the respondents did not have any access to obstetrics and gynaecology service with an interest in bleeding disorders. All but one respondent (36/37, 97%) would perform laboratory testing during the preconception phase. The respondents would advise to check blood count, ferritin, anti‐HLA antibodies and anti‐αIIbβ3 antibodies in 91%, 91%, 70% and 58%, respectively. Genetic carrier screening of the partner was recommended by 18% (6/33) of respondents in all patients and only in case of consanguinity by 52% (16/33). Physicians were asked whether they would prefer rFVIIa instead of platelet transfusion to treat a bleed in women with a pregnancy wish to prevent alloimmunisation, which was the case in 94% (31/33). Physicians were asked to rate the top five reasons to advise against pregnancy (Figure  2 ). A history of a complicated pregnancy or childbirth was most frequently ranked among the top five factors, and 58% (19/33) of respondents identified severity of bleeding phenotype as the most important factor. Seventy‐nine percent (26/33) of respondents would consider advising a woman with GT against pregnancy, based on these factors. Healthcare professional responses on when they may advise against pregnancy. Iron deficiency requiring iron supplementation was seen by all physicians who previously managed women with GT trying to get pregnant. The majority (77%, 17/22) reported seeing this often (in ≥50% of women). The most common treatment goal for iron deficiency was achieving ferritin levels within the normal range (67%, 22/33). Other goals amongst respondents were achieving a normal haemoglobin level (27%, 9/33) and managing asymptomatic anaemia (6%, 2/33). Sixty‐two percent (16/26) of respondents who had previously managed women with GT who are trying to conceive reported them to have had a delay in becoming pregnant, which was defined as the inability to conceive naturally within 1 year after discontinuing contraceptives. Most physicians (76%, 25/33) would offer assisted reproductive treatment (ART) to women with GT with difficulty conceiving. ART is reimbursed by the state in most countries (79%, 22/28). Endometriosis and ovulation bleeds were reported by respectively 30% (8/26) and 53% (16/28) of physicians in women with GT. Almost all respondents would screen for anti‐HLA antibodies and anti‐αIIbβ3 antibodies, respectively, 94% (31/33) and 91% (30/33). The proportion of respondents recommending screening for anti‐HLA antibodies and anti‐αIIbβ3 antibodies during the different stages of pregnancy is presented in Figure  3 . Current practice regarding maternal anti‐HLA and anti‐αIIbβ3 antibody screening. (A) Timing of screening during different phases of pregnancy. (B) Frequency of screening during pregnancy. The combination of therapies used for the treatment of bleeding during pregnancy included TXA in 79% (26/33), rFVIIa in 67% (22/33), HLA‐matched platelet transfusion in 46% (15/33) and random platelet transfusion in 21% (7/33). Only 6% (2/33) would add desmopressin to the treatment. Thirty percent (10/33) of physicians would administer rFVIIa combined with TXA and another 30% (10/33) would combine this treatment with platelet transfusion. The majority of physicians (75%, 24/32) advised a mode of delivery as per obstetric indication. Sixteen percent (5/32) preferred caesarean section, and another 9% (3/32) preferred vaginal birth. Most physicians (69%, 22/32) would consider GT patients unsuitable for epidural or subdural anaesthesia, even under haemostatic cover. Among physicians without experience in managing pregnancies in GT, opinions were divided (55%, 11/20 would advise against it), whereas among physicians with such experience, the vast majority would advise against (85%, 11/13). The combination of treatments used to prevent PPH and rescue treatments is shown in Figure  4 . The most commonly recommended preventive treatments were TXA and rFVIIa (25%, 8/32), followed by TXA, rFVIIa and platelet transfusion (22%. 7/32). The most commonly recommended rescue treatments were TXA, rFVIIa and platelet transfusion (41%, 13/32). Among the respondents who recommended platelet transfusion, 71% and 76%, respectively, preferred the use of HLA‐matched platelets over random donor platelets as preventive therapy or rescue therapy. The application and duration of the various haemostatic agents are detailed in Table  2 . Preventive therapy and rescue therapy to prevent postpartum haemorrhage during delivery in women with GT. Treatment provided by survey respondents to prevent postpartum haemorrhage. Abbreviations: rFVIIa, recombinant activated factor VII; TXA, tranexamic acid. Exact duration was not specified in the survey. Of the respondents who would administer platelet transfusions prophylactically, 74% would administer at least two pools of platelets. Forty percent would administer subsequent platelet transfusion for 1–2 days post‐partum, 50% for 3–4 days and 10% for ≥ 5 days. Opinions were divided regarding the administration of pharmacological thromboprophylaxis around childbirth for women with GT. Fifty‐nine percent (19/32) would allow thromboprophylaxis if indicated following a risk assessment, while 41% (13/32) would not. The proportion of physicians who would advise against thromboprophylaxis was higher in the group with experience in managing pregnancies in GT (67%, 8/12) compared with physicians without such experience (45%, 11/20). Of the physicians who have previously managed a pregnancy in a woman with GT, 80% (16/20) had never observed FNAIT in the child. However, of the four physicians who have seen FNAIT, two physicians reported to have seen FNAIT in ≥50% of pregnancies. To prevent FNAIT, immunoglobulins could be administered during pregnancy. The majority of physicians (63%, 20/32) would recommend this treatment, specifically in patients with a history of FNAIT or intracranial haemorrhage (25%), in patients with αIIbβ3 antibodies or if titres rise (16%), or in both (22%).

Women with GT who wish to conceive are confronted with significant risks for both themselves and their children. This international survey highlights the heterogeneity in managing the preconception phase, pregnancy and childbirth. Multidisciplinary care is mandatory and there is a need for improved implementation of current guidelines.

This is the first international survey focusing on the management of women with GT in the preconception phase, pregnancy and childbirth. With responses from 37 physicians from 18 countries, it provides an overview of current clinical practices within Europe. There was an almost unanimous response that preconception counselling by a multidisciplinary team at a hospital with expertise in GT should be offered to all women with GT who are planning a pregnancy. Despite respondents from well‐resourced and large haemostasis centres, 11% of the responding centres lack access to obstetrics and gynaecology services focused on bleeding disorders. Prior to pregnancy, the majority of respondents indicated they would screen for iron deficiency anaemia and treat it if necessary. This is important because women are at risk of HMB and ovulation bleeds after discontinuation of hormonal contraceptives. Additionally, anaemia is a risk factor for PPH as well as detrimental for foetal development [ 8 , 9 ]. Preconception counselling can include genetic counselling if there is consanguinity between partners and most respondents screen for the presence of anti‐HLA and anti‐αIIbβ3 antibodies in the preconception phase. Delay of more than 12 months to conceive in women with GT was reported by 62% of respondents. In a review, the need for ART in women with GT was 50% in the pregnancies wherein the mode of conception was reported [ 10 ]. However, in our survey, no questions were asked regarding fertility assessment. One possible explanation for a relatively high need for ART is that these women are not referred to ART because of sub‐fertility but to expedite conception minimising time without effective hormonal therapies and consequently heavy bleeding. HMB, ovulation bleeding and retrograde menstruation may increase the risk of endometriosis and lead to subfertility in women with GT, but there is a lack of data to draw conclusions on this issue. During pregnancy, most respondents preferred treatment of bleeds with TXA and/or rFVIIa instead of platelet transfusion to decrease the risk of platelet alloimmunisation. If platelet transfusion is inevitable, HLA‐matched platelets could be administered to decrease the risk of anti‐HLA antibody formation [ 7 ]. However, the availability of HLA‐matched platelet transfusion may differ between centres and countries and is also influenced by the rarity of the patients HLA type. In current guidelines there are no recommendations provided regarding the screening for HLA antibodies during pregnancy. The UKHCDO guideline does recommend anti‐αIIbβ3 antibody screening at booking, and at 28 and 34 weeks of gestation [ 7 ]. Given that platelet transfusions, frequently administered during childbirth to prevent PPH, significantly increase the risk of anti‐αIIbβ3 antibody formation, there is a clear rationale for recommending postpartum anti‐αIIbβ3 antibody screening [ 11 ]. Nonetheless, this is not advised in the UKHCDO guideline [ 7 ], and only 24% respondents indicated they would recommend such screening. The UKHCDO guideline does include recommendations regarding the treatment with intravenous immunoglobulins in women with high or rising anti‐αIIbβ3 antibody levels, or a history of giving birth to a child with FNAIT. Management of pregnant women with GT and anti‐αIIbβ3 antibodies should involve a specialised multidisciplinary team, including a foetal medicine specialist and neonatal physician. At childbirth, women with GT are at an increased risk of PPH, and the approach to prevent PPH described in the literature is very heterogenous [ 4 ]. In line with the UKHCDO guideline, most respondents would recommend treatment with TXA in all patients [ 7 ]. Depending on factors such as platelet transfusion refractoriness, presence of platelet alloimmunisation, and the availability of HLA‐matched platelets, either rFVIIa or HLA‐matched platelets could be added to this management. The recommendations regarding the number of platelet units and the duration of the treatment vary. Besides primary PPH, women with GT are at risk for secondary PPH [ 10 ]. In order to prevent secondary PPH, most respondents would continue TXA as long as excessive lochia exist. Regarding the mode of delivery, most physicians advised it as per obstetric indication. While vaginal delivery is not contraindicated in women with GT, it may not be the preferred mode of delivery in cases with a significant risk of foetal bleeding, for example due to genetic inheritance of GT in case of consanguinity or the potential presence of FNAIT in the case of detectable αIIbβ3 antibodies. In some cases, for example if HLA‐matched platelets are deemed necessary, a planned delivery could be considered for logistic reasons. Refraining from neuraxial blocks in women with GT was recommended by half of the survey respondents. Opinions regarding pharmacological thrombosis prophylaxis were divided. These issues need further exploration before consensus could be reached. In current guidelines and expert‐based opinion papers, no recommendations regarding thromboprophylaxis are mentioned, which might explain the heterogeneity in respondents's answers. However, current guidelines do recommend to prevent neuraxial anaesthesia [ 7 , 12 ]. In this case, the heterogeneity appears to be between physicians with and without experience in managing pregnancies, with the group possessing such experience largely adhering to the guideline recommendation to prevent neuraxial anaesthesia. The relatively limited number of respondents and the low number of respondents who actually managed pregnancies in women with GT is inherent to the rarity of this condition. Another limitation of our survey is the loss of detail resulting from the use of multiple‐choice questions. Several other limitations should be noted. Despite the fact that all survey questions were mandatory, not all respondents completed the entire survey, which resulted from respondents being able to exit the survey halfway. Due to the method of survey distribution, it is difficult to define the sampling frame and response rate, and it is therefore unclear whether this may have introduced bias. Furthermore, clustering may have led to bias by overrepresenting the views of centres with multiple respondents. In the Netherlands, one centre had three respondents and another had wo. Whereas all other centres had only one respondent. However, the survey explicitly specified that responses should reflect personal experience rather than centre‐level experience. Although a guideline is currently available from UKHCDO, there remains significant heterogeneity in the recommended treatment approaches among caregivers. There is a need for improved implementation of current guidelines, for example by educational workshops or webinars including case‐based discussions.

Glanzmann thrombasthenia (GT) is an inherited platelet disorder resulting from a quantitative and/or qualitative defect in the platelet membrane glycoprotein αIIbβ3. In patients with GT, platelet aggregation is reduced or absent, leading to impaired haemostasis and an increased risk of bleeding [ 1 ]. These bleeds comprise most often mucocutaneous bleeds and almost all women suffer from heavy menstrual bleeding (HMB) [ 2 , 3 ]. The postpartum haemorrhage (PPH) rate in women with GT is 34% [ 4 ] compared to 6.4% in the general population [ 5 ]. Among the treatments used for the prevention and/or management of PPH are tranexamic acid (TXA), recombinant activated factor VII (rFVIIa) and platelet transfusion. However, the dosage, duration of treatment and specific combination of therapies vary [ 4 ]. Additionally, pregnancy increases the risk of both anti‐human leukocyte antigen (HLA) and anti‐αIIbβ3 antibody formation. Besides platelet refractoriness, the latter may cause foetal/neonatal alloimmune thrombocytopenia (FNAIT), which increases the risk of severe foetal and neonatal bleeding such as intracranial haemorrhage and possible foetal death [ 6 ]. Therefore, current guidelines recommend screening for αIIbβ3 antibodies during pregnancy and treatment with intravenous immunoglobulins in women with high or rising αIIbβ3 antibodies or a history of giving birth to a child with FNAIT [ 7 ]. Both pregnancy and childbirth present a significant challenge for both mother and child, which requires a multidisciplinary approach for management. The main risks are summarised in Table  1 . Risks for mother and child before, during and after pregnancy in Glanzmann thrombasthenia. Severe HMB and iron deficiency anaemia following discontinuation of contraceptives Severe bleeding following invasive fertility procedures Primary and/or secondary PPH following childbirth Severe bleeding following miscarriage or termination of pregnancy Platelet alloimmunisation Early and late pregnancy loss FNAIT Fetal ICH or other bleeding complications Inheritance Abbreviations: FNAIT, foetal neonatal alloimmune thrombocytopaenia; HMB, heavy menstrual bleeding; ICH, intracranial haemorrhage; PPH, postpartum haemorrhage. As GT is a rare disease, current management strategies are based on small cohort studies and case reports. These previous studies have shown that the management of pregnant women with GT is very heterogeneous [ 4 ]. To gather insights and expert opinions on management strategies, the Glanzmann Thrombasthenia Working Group (GTWG) and the Women and Girls with Bleeding Disorders + Working Group (WGBD+WG) of the European Association of Hemophilia and Allied Disorders (EAHAD) have conducted a survey among healthcare professionals. The objective is to present the current clinical practices in pregnancy and childbirth in GT.

Roger Schutgens received speaker's fees and/or research grants from Bayer, CSL Behring, Hemab, Novo Nordisk, Octapharma, Sanofi, Sobi and Takeda. Roseline d'Oiron reports honoraria for lectures or advisory boards from Takeda, BioMarin, CSL Behring, LFB, NovoNordisk, Octapharma, Roche/Chugai and Sobi/Sanofi. Mathieu Fiore received fees from NovoNordisk. Mary Mathias has been a paid speaker by Octapharma and Sobi, has been funded to attend meetings by Octapharma and Sobi, has been an advisory board member for Sobi, and is principal‐investigator for interventional and non‐interventional trials for Roche, Sobi, Sanofi and Octapharma. Michelle Lavin has served as a consultant for Sobi, Star Therapeutics and Band Therapeutics, received speaker fees from Sobi and Takeda and research funding from Takeda. Interpretation of treatment effects of tranexamic acid and recombinant activated factor VII was independent of funders/manufacturers.

Supporting File 1 : hae70186‐sup‐0001‐SuppMat.docx Supporting File 2 : hae70186‐sup‐0002‐SuppMat.docx