The causes of more common, mild, inherited MCB remain unknown. Diagnostic testing is helpful in identifying deficiencies of platelet function or VWF in some, but not all, patients with MCB. Efforts to standardize available testing
will help us to optimally interpret these tests. New ways of evaluating patients with no diagnostic abnormalities in traditional testing are required, including Selleckchem AG 14699 assays that will measure aspects of the platelet–vessel wall interaction. “
“Summary. Platelet transfusions, main therapy of Glanzmann Thromboasthenia (GT), can induce an allo-immunization against human leucocyte antigen and integrin αIIbβ3. We have investigated in our GT patients the rate of allo-immunization and of refractoriness to platelet transfusions. From 1975 until December 2005, we have followed 17 GT patients: 14 type 1, 3 variant type; nine females, eight males; median age at diagnosis 9.8 years (range 1–44.5); median
age at the time of the study 35.5 years (range 23.6–68.5). In our patients, 121 bleeding episodes occurred (24 severe, 37 moderate, and 60 mild). Ten major and 22 minor surgical procedures have been performed. Two spontaneous deliveries and three caesarian sections with five live births were performed; moreover, MK-8669 datasheet one late foetal loss occurred, and one voluntary abortion was performed. Sixteen of 17 patients have been transfused at least once in life with platelets and/or red blood cells (RBC). All transfused patients have been investigated for the presence of anti-HLA and anti-integrin αIIbβ3 allo-antibodies. The positiveness selleck chemicals llc of allo-antibodies has been demonstrated in 4/16 transfused patients (25%): isolated for anti-HLA in two; isolated for anti-integrin αIIbβ3 in one; and combined in one. In spite of the presence of allo-antibodies, platelet transfusions have always been effective and the haemostasis was not compromised. “
“Summary. A descriptive survey was conducted in Region V-E of the United States to bridge the gap in available information
on pain issues in the bleeding disorders population. The aim of this study was to a) determine language used by patients to describe and differentiate acute and persistent pain, b) describe pharmacological and non-pharmacological strategies utilized to control pain, c) determine the providers of pain management to this population and d) evaluate quality of life incorporating the SF-36 QOL tool. A total of 202 surveys were returned. For the purposes of this paper, it was decided to analyse only haemophilia data (n = 114). Average persistent daily pain levels were 5/10 (P < 0.001). The three most common word descriptors for both acute and persistent pain were the same – achy, throbbing and tender; the most utilized pain medications were NSAIDs and acetaminophen.