Navigating Bernard soulier syndrome in pregnancy: A multidisciplinary case report

 

Kuwait Board of Anesthesia, Kuwait Institute for Medical Specialization, Kuwait

²Kuwait Board of Obstetrics and Gynecology, Kuwait Institute for Medical Specialization, Kuwait

³Department of Anesthesia and ICU, Maternity Hospital, Kuwait

 

 

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INTRODUCTION

 

Bernard-Soulier Syndrome (BSS) is a rare inherited bleeding disorder caused by a defect or deficiency in the glycoprotein Ib-IX-V complex on platelets.

 

Increases the risk of excessive bleeding. Managing pregnancy in women with BSS requires a multidisciplinary approach to address bleeding risks, transfusion challenges, medication, and planned 

 

CASE REPORT

A 29-year-old woman at 36 weeks of pregnancy (G3, P0+2) with BSS and HLA antibodies presented in early labor.

• HLA antibodies, which impair platelet survival after transfusion.

• ICU transfer for labor augmentation.

 

Past Obstetric History

• Marked by PPH

 

Investigations at presentation:

• Hemoglobin: 9.2g/dL

• Platelets: 42 x 10⁹/L

 

Management

• Intravenous immunoglobulin

• Recombinant factor VIIa

• HLA-matched platelets

• Packed red blood cells

 

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DISCUSSION / CONCLUSION

The condition is highly variable and somewhat unpredictable.

Close monitoring of the mother’s hematocrit and platelet count is essential to promptly identify and address any bleeding during the peripartum period.

Managing pregnancy multidisciplinary approach and in such cases remains a subject of debate, necessitating a personalized medical care.

 

REFERENCES

 

1. Almomani MH, Mangla A. Bernard-Soulier Syndrome. 2024 Jan 11. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2024 Jan–. PMID: 32491603.

2. Pham A, Wang J. Bernard-Soulier syndrome: an inherited platelet disorder. Arch Pathol Lab Med. 2007 Dec;131(12):1834-6. doi: 10.5858/2007-131-1834- BSAIPD. PMID: 18081445.

3. Reisi N. Bernard-Soulier syndrome or idiopathic thrombocytopenic purpura: A case series. Caspian J Intern Med. 2020 Winter;11(1):105-109. doi: 10.22088/cjim.11.1.105. PMID: 32042394; PMCID: PMC6992729.

4. Demirci U, Erbilen EA, Ümit EG, İnan C, Sayın NC, Demir AM. Successful pregnancy and delivery management in a patient with Bernard Soulier Syndrome. Obstet Med. 2023 Sep;16(3):203-205. doi: 10.1177/1753495X211067119. Epub 2022 Jan 13. PMID: 37719997; PMCID: PMC10504883.