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BMI is an important determinant of VWF and FVIII levels and bleeding phenotype in patients with von Willebrand disease

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observed during eltrombopag treatment or at the follow-up visits routinely performed 30 days after surgery. As eltrombopag has been associated with occurrence of cataracts in patients with immune thrombocytopenia, and… Click to show full abstract

observed during eltrombopag treatment or at the follow-up visits routinely performed 30 days after surgery. As eltrombopag has been associated with occurrence of cataracts in patients with immune thrombocytopenia, and MYH9-RD is a syndromic disorder predisposing to cataracts, ophthalmological evaluation was carried out at every follow-up visit. None of our patients showed occurrence or worsening of cataracts, not even patients 1 to 3 who already presented cataracts at baseline and received repeated eltrombopag courses. In summary, eltrombopag successfully replaced platelet transfusions in preparation for surgery in 10 of 11 cases. Importantly, this drug provided a simple and safe option for increasing platelet count in case of refractoriness to platelet transfusions. Another advantage of eltrombopag compared to prophylactic platelet transfusions is that the drug induced a stable increase of platelet count throughout the perioperative period, thus also allowing the safe administration of a standard antithrombotic prophylaxis, and, in some cases, avoiding hospitalization. In our real-life setting, short-term eltrombopag was safe with no significant adverse events reported. However, since thrombopoietinmimetics have been associated with potentially severe adverse events in acquired thrombocytopenias, including thrombosis, the risk-benefit ratio of eltrombopag administration should be always carefully assessed for each MYH9-RD patient. In conclusion, our data suggest that shortterm eltrombopag should be considered as the first-line treatment to prepare MYH9-RD patients with severe thrombocytopenia for elective surgery.

Keywords: important determinant; determinant vwf; eltrombopag; bmi important; platelet transfusions; platelet

Journal Title: American Journal of Hematology
Year Published: 2019

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