Hemorrhagiparous Thrombocytic Dystrophy (Bernard-Soulier Syndrome): A Case Report of a Rare Bleeding Syndrome
Author(s):
Yaser Khalid, Neethi Dasu*, Herman Suga, Debashis Reja, Kirti Dasu, Amy Polansky, Keith Brown and Richard Gordon
Bleeding syndromes, such as Bernard-Soulier Syndrome (BSS), are extremely rare with an incidence of 1 in 1,000,000 persons. Very few cases (approximately a hundred) have been reported in the literature. However, it is very important to quickly recognize this condition as soon as possible before life-threatening complications occur. Here, we present a case of a 25-yr-old with chronic thrombocytopenia who was found to have BSS on peripheral blood smear prior to a routine dental procedure. Rare disorders, such as BSS, need to be on the differential diagnosis for physicians because missing this condition could have led to catastrophic side effects, such as uncontrolled bleeding for our patient. She was transferred to a tertiary care center for her procedure to be managed for potential bleeding complications.