Perioperative Eltrombopag in Patients With Inherited Thrombocytopenia

Inclusion Criteria: * Symptomatic patients with bleeding history and chronic thrombocytopenia with strong presumption of constitutional origin on the basis of * the identified mutation and/or * a combination of the following criteria: familial antecedent with Mendelian transmission, duration of thrombocytopenia, suggestive syndromic presentation, and evidence against primary or secondary immune thrombocytopenia, especially absence of immunologic markers and failure of previous conventional or immunosuppressive therapies. * Averaged platelet counts during the last five years below the safety level required for the procedure. * Scheduled (\>4 weeks) surgery or invasive procedure with anticipated risk of bleeding: e.g. needle biopsy of solid organ (liver, kidney….etc.), interventional endoscopy, major surgeries, or surgery without possibility of mechanical control of haemostasis (e.g. tonsillectomy). Written informed consent of the patient or his (her) parents or tutors (patients \< 18 yrs). Patients included in the French national registry of rare platelet disorders * Patient with social insurance coverage Exclusion Criteria: * questionable constitutional origin; * definite platelet dysfunction associated to thrombocytopenia (eg: gray platelet syndrome, NBEAL2 and related gene mutations, homozygous Bernard-Soulier Syndrome); * thrombocytopenia with predisposition to hematologic malignancies (e.g; RUNX1, ETV6 or ANKRD26 gene mutations). * amegakaryocytic thrombocytopen…