ORIGNAL ARTICLES: Case reportSuccessful thyroidectomy in a patient with Hermansky–Pudlak syndrome treated with recombinant activated factor VII and platelet concentratesdel Pozo Pozo, A. I.; Jiménez-Yuste, V.; Villar, A.; Quintana, M.; Hernández-Navarro, F.Author Information The authors are with the Servicio de Hematología, Hospital Universitario La Paz, Madrid, Spain. (Received 9 August 2001; revised 15 April 2002; accepted 22 April 2002) Address correspondence to Ana Isabel del Pozo Pozo, Plaza de la Beata María Ana de Jesús, n° 3, 3° A, Madrid 28045, Spain. Tel: (+34) 91 409 47 99; fax: (+34) 91 727 71 16; e-mail: email@example.com Blood Coagulation & Fibrinolysis: September 2002 - Volume 13 - Issue 6 - p 551-553 Buy Abstract Hermansky–Pudlak syndrome is a rare autosomal recessive disorder characterized by the absence of platelet dense bodies in association with albinism. We present the use of recombinant activated factor VII (rFVIIa) in a patient with Hermansky–Pudlak syndrome who underwent total thyroidectomy because of a large richly vascularized nodule (10 cm) compressing the trachea. The patient had a prolonged bleeding time (> 20 min) that remained unchanged after platelet transfusions. However, after infusion of platelets plus rFVIIa, it diminished to 5 min. The platelet aggregation response to adenosine diphosphate and collagen was diminished. Since an early age, the patient had repeated nose bleeding and an episode of melena requiring several tampons, cauterization and transfusions of packed red cells. In this case, we used rFVIIa in bolus for 1 day (four doses of 120 μg/kg every 2 h and six doses of 100 μg/kg every 3 h) and transfusion of platelet concentrates beginning just prior to surgery. No evidence of local bleeding complication could be detected during the entire post-operative period. The hemoglobin level remained normal and no transfusions of packed red cells were necessary. No adverse events occurred. © 2002 Lippincott Williams & Wilkins, Inc.