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Muscle pain in the legs and lack of efficacy: case report A 76-year-old woman exhibited a lack of efficacy during treatment with factor-VIII/von-Willebrand-factor for melaena secondary to inherited type 3 von Willebrand disease (VWD). Later, she developed muscle pain in the legs during treatment with atorvastatin for angiodysplasia in inherited type 3 VWD. The woman, who had been previously evaluated on multiple occasions in Egypt and European centres specialised in bleeding disorders, presented to a centre in the Italy. During the previous 10 years, she had suffered from multiple episodes of melaena, which recurred with high frequency and were poorly handled with red cell transfusion and cryoprecipitate. She had undergone several attempts for endoscopic control of bleeding that were unable to prevent recurrences due to presence of multiple angiodysplasia lesions in the upper gastrointestinal (GI) tract. Also, she was born to consanguineous parents, and she had been diagnosed with inherited type 3 VWD. She started receiving a regular prophylactic regimen of factor-VIII/von-Willebrand-factor [pasteurised plasma-derived VWF/FVIII concentrate] 50 U/kg, which was infused every other day; however, throughout the observation period of 3 months, she continued to develop episodes of melaena, while von Willebrand factor:ristocetin cofactor (VWF:RCo) plasma levels maintained at trough levels not lower than 40–50 U/dL. Also, the factor VIII coagulant activity (FVIII:C) plasma level had reached a very high value (values >200 U/dL at times). She exhibited a lack of efficacy to factor-VIII/vonWillebrand-factor treatment. Therefore, the woman started receiving atorvastatin at a high dosage of 80mg daily to stop the formation of new GI tract lesions. She tolerated atorvastatin therapy for 3–4 months; however, she developed a side effect of moderate muscle pain in the legs secondary to atorvastatin [time to reaction onset not stated]. She continued to develop recurrent episodes of GI bleeding, and endoscopy showed an evidence of new angiodysplasia lesions. Atorvastatin was therefore discontinued, and then, she moved back to Egypt. Prophylactic treatment with cryoprecipitate and factor-VIII/von-Willebrand-factor concentrate was recommended to her at home (if available). Later, she died suddenly due to a myocardial infarction, which had developed after an episode of GI bleeding that had led to severe anaemia [outcome of ADR not stated]. Mannucci PM. New therapies for von Willebrand disease. Hematology: the Education Program of the American Society of Hematology 2019: 590-595, No. 1, 6 Dec 2019. 803503137 Available from: URL: http://doi.org/10.1182/hematology.2019000368

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