Platelets play a critical role in hemostasis and are involved in wound repair and inflammatory processes. Contact to the subendothelial matrix induces platelet activation that is followed by release of cytokines and growth factors and formation of a vascular plug. Several inherited defects of platelet function resulting in bleeding diathesis are known. However, only in the minority of these dis...

A patient (OG) with Glanzmann thrombasthenia became refractory to platelet transfusion after the production of an immunoglobulin G (IgG) isoantibody (Ab1) specific for the integrin subunit beta 3. To determine the frequency at which the OG idiotype is found in the general population and in immune-mediated disease states, we developed a rabbit polyclonal antibody (Ab2) specific for affinity-puri...

Crohn's disease (CD), a polygenic disorder, is the major subtype of inflammatory bowel disease. There heterogeneity and many patients present extraintestinal manifestations. However, association crohn's with another genetic has not been previously described. We report exceptional CD Glanzmann’s thrombasthenia which rare inherited bleeding disorder. Indeed, diagnosis in patient kidney amylosis, ...

Patients with Glanzmann thrombasthenia (GT) may form isoantibodies which induce refractoriness or inhibition of function of transfused platelets. We monitored the survival and function of transfused platelets by flow cytometry and thrombelastography in a patient with GT. Gating on CD42a+ allowed identification of even a few transfused platelets. Only by gating on these CD41+ CD42a+ cells were w...