ABSTRACT
Bernard soulier syndrome [BSS] is a rare congenital bleeding disorder characterized by thrombocytopenia. BSS causes bruising, epistaxis, gingival bleeding, menorrhagia, post partum bleeding, gastrointestinal bleeding and post traumatic hemorrhage, but there is no report of hemoptysis in BSS. A 14 year-old girl was referred to our center due to massive hemoptysis. Her chest x-ray revealed complete collapse of the right lung. Rigid bronchoscopy was preformed and the intrabronchial clots were removed. Smear and culture of direct sputum was positive for Mycobacterium tuberculosis. She received anti-tuberculosis treatment. During treatment, she developed a massive vaginal bleeding, because of drug interaction between rifampin and low dose contraceptive [LD], which she had been taking for control of massive menstrual bleeding. Her vaginal bleeding was controlled by platelet infusion and recombinant factor 7 infusion. After 2 months of anti-TB treatment, sputum smear and culture for BK converted to negative. One year after treatment, the pulmonary tuberculosis was completely cured and no hemoptysis occurred. When hemoptysis occurs in patients with Bernard soulier syndrome, we should consider other differential diagnoses, and further diagnostic evaluation is recommended