Bernard-Soulier syndrome is an uncommon bleeding disorder caused by a quantitative or qualitative defect in the platelet glycoprotein (GP)Ib/IX complex. The complex is composed of four subunits, GPIbalpha, GPIbbeta, GPIX, and GPV. Here we describe the molecular basis of a novel Bernard-Soulier syndrome variant in a patient in whom GPIbalpha and GPIX were undetectable on the platelet surface. DN...

The platelets of three patients with the hereditary giant platelet syndrome of Bernard and Soulier failed to aggregate in response to either ristocetin or bovine fibrinogen. The results of aggregation experiments using mixtures of platelets and plasma suggest that a reaction between a plasma factor deficient in von Willebrand's disease and a platelet component lacking in our patients, and leadi...

REFERENCES 1. Tsukahara T, Takasawa A, Murata M, et al. NK/T-cell lymphoma of bilateral adrenal glands in a patient with pyothorax. Diagn Pathol 2012;7:114. 2. Rashidi A, Fisher SI. Primary adrenal lymphoma: a systematic review. Ann Hematol 2013;92:1583-93. 3. Kasaliwal R, Goroshi M, Khadilkar K, et al. Primary adrenal lymphoma: a single-center experience. Endocr Pract 2015;21:71924. 4. Santhos...