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How we treat the platelet glycoprotein defects; Glanzmann thrombasthenia and Bernard Soulier syndrome in children and adults
Author(s) -
Grainger John D.,
Thachil Jecko,
Will Andrew M.
Publication year - 2018
Publication title -
british journal of haematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.907
H-Index - 186
eISSN - 1365-2141
pISSN - 0007-1048
DOI - 10.1111/bjh.15409
Subject(s) - thrombasthenia , medicine , bernard–soulier syndrome , glanzmann's thrombasthenia , platelet disorder , platelet , pediatrics , blood platelet disorders , pregnancy , surgery , platelet aggregation , biology , genetics
Summary The inherited platelet glycoprotein deficiencies, Glanzmann thrombasthenia ( GT ) and Bernard Soulier syndrome ( BSS ) are rare but important long‐term bleeding disorders. Once diagnosed, affected patients should be referred to a specialist centre for bleeding disorders for general advice and ongoing management. Patients do not require prophylactic treatment and so the management of GT and BSS focuses around prophylactic treatment prior to high risk procedures and treatment in response to non‐surgical bleeding events and, in women, the management of menorrhagia and pregnancy. There is no consistent approach to the treatment or prevention of bleeding complications. Management must be tailored for each individual and the approach may not be the same for different events, even for the same patient, depending on the type of accident or invasive procedure, the extent of bleeding and the presence or not of platelet refractoriness.