Abstract:
:We present here a 63-year old woman with a long history of immune thrombocytopenia. She was hospitalized for a traumatic intracranial hemorrhage with thrombocytopenia. Following inefficient treatment of four platelet transfusions, immunoglobulins, and corticosteroids, we initiated treatment with a thrombopoietin (TPO) receptor agonist (eltrombopag 25 mg/d) with a good efficacy. Her mother and sister also had chronic thrombocytopenia. Clinical history, hemostasis results, and gene analysis revealed von Willebrand disease (VWD) type 2B with the mutation (c.3946G>A; p.V1316M), which combines a von Willebrand factor defect with severe thrombocytopenia, as well as a thrombocytopathy. The efficacy of TPO receptor agonists appears to counterbalance, at least to some extent, the thrombocytopathy associated with this mutation. As such, the use of TPO receptor agonists could represent an alternative therapeutic approach in cases of VWD type 2B with severe thrombocytopenia.
journal_name
Plateletsjournal_title
Plateletsauthors
Espitia O,Ternisien C,Agard C,Boisseau P,Denis CV,Fouassier Mdoi
10.1080/09537104.2016.1246717subject
Has Abstractpub_date
2017-07-01 00:00:00pages
518-520issue
5eissn
0953-7104issn
1369-1635journal_volume
28pub_type
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