Like Father, Like Daughter: A Family With a Constitutional Thrombocytopenia Variant Due to a Novel Heterozygous Missense Mutation in GP1BA

Constitutional platelet disorders have become better understood since Bernard and Soulier first described a case in 1948. Their diagnosis can also be challenging due to overlap in clinical presentation and lab findings with platelet type von Willebrand. Bernard-Soulier syndrome is a disorder caused by GPIb receptor mutations that decrease its affinity for von Willebrand factor resulting in reduced platelet function and macrothrombocytopenia. It is associated with multiple genetic mutations in either GPIBA, GPIBB, GP9, or GP5. We present a case report of a family with a Bernard Soullier-like thrombocytopenia variant due to a heterozygous missense mutation in GPIBA.

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Publication Details

Journal
Journal of Pediatric Hematology/Oncology
Published
2024-12-31
DOI
https://doi.org/10.1097/mph.0000000000002980
Primary Topic
Platelet Disorders and Treatments
Type
article
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article

Like Father, Like Daughter: A Family With a Constitutional Thrombocytopenia Variant Due to a Novel Heterozygous Missense Mutation in GP1BA

Jennifer Light, Gerardo Quezada
Journal of Pediatric Hematology/Oncology
Platelet Disorders and Treatments
article

Like Father, Like Daughter: A Family With a Constitutional Thrombocytopenia Variant Due to a Novel Heterozygous Missense Mutation in GP1BA

Jennifer Light, Gerardo Quezada
article en

Abstract

Constitutional platelet disorders have become better understood since Bernard and Soulier first described a case in 1948. Their diagnosis can also be challenging due to overlap in clinical presentation and lab findings with platelet type von Willebrand. Bernard-Soulier syndrome is a disorder caused by GPIb receptor mutations that decrease its affinity for von Willebrand factor resulting in reduced platelet function and macrothrombocytopenia. It is associated with multiple genetic mutations in either GPIBA, GPIBB, GP9, or GP5. We present a case report of a family with a Bernard Soullier-like thrombocytopenia variant due to a heterozygous missense mutation in GPIBA.

Journal of Pediatric Hematology/OncologyVol. 47(1)
University of North Carolina at Chapel Hill (US), Illinois College (US)
Peace, Justice and strong institutions
Openalex Percentile: Top 55%
Platelet Disorders and Treatments
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