Rajiv K Pruthi

..Serious adverse events were related to continued or increased bleeding. In conclusion, haemostatic efficacy and safety of BI and CI of rFVIIa are comparable for the surgical management of haemophilia subjects with inhibitors...

..Future laboratory and clinical studies are necessary for advancement of pathophysiologic knowledge and therapeutic options for patients with this uncommon but clinically important disorder...

..This article provides a practical approach to utilization of genetic testing in vWD...

..This article provides a practical approach to using genetic testing for hemophilia A and B...

..This mutation in the heterozygous form was also present in a mildly affected, unrelated patient. We also report on the natural history of an FVII inhibitor in the patient with severe FVII deficiency...

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..The purpose of this study was to describe health-related quality of life and satisfaction after global endometrial ablation in women with bleeding disorders and a systematic review of the literature...

..Therapy-related APL is sensitive to standard therapy with no cases of resistance or relapse seen. The inferior OS of the t-APL was due to induction mortality, possibly reflecting prior therapy...

..Recognition of cancer-associated microangiopathic hemolytic anemia with thrombocytopenia (CA-MHA) is paramount to avoid inappropriate PE therapy and delays in cancer-specific chemotherapy...

..AVWS may be more prevalent than has been appreciated; we estimate up to 0.04%. Awareness of the existence of AVWS is essential for diagnosis and appropriate management. Therapy of associated diseases may improve the bleeding disorder...

..Endocarditis and septic arthritis in patients with hemophilia are rare infectious complications but should be considered in patients with persistent bacteremia...

..Although cardiac surgery is successful, exposure to factor concentrates in severe hemophilia early in life might predispose a patient to inhibitor development...

..Our study also suggests that the natural history of a JAK2V617F-positive "occult" MPN might be different from that of a typical MPN...

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..He was successfully treated with Bead Block and coil embolization followed by surgical extirpation of the lesion 24 hr later...

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..Intravenous immunoglobulins have also shown some efficacy in the management of AvWD, especially cases associated with monoclonal gammopathies. Awareness of AvWD is essential for diagnosis and appropriate management...

..Importantly, only CD38+ was significantly associated with high-risk FISH categories (+12, 11q- and 17p-) after adjustment for the effects of other variables...

..The low rate of clinically apparent thrombosis reflects our practice of not screening for thrombosis. The low infection rate reflects our practice of using and reinforcing the aseptic technique...

..His family history was negative for haemophilia. Review of our institutional experience as well as the literature indicates that intracranial bleeding as the initial presentation of haemophilia is rare...

..Some of our patients had significant bleeding despite adequate factor replacement before and after the procedure. Parents and patients must be aware that bleeding risk is a possibility despite adequate factor replacement for hemostasis...

..However, current manual or automated VWF:RCo assay methods have relatively poor operating characteristics. Our goal was to develop and validate a simple, accurate, specific and sensitive platelet-based VWF:RCo assay...

..Oral vitamin K supplementation resulted in partial resolution of proteins and complete resolution of bleeding...

..Prospective studies are urgently needed to define the true efficacy and long-term safety of rituximab...

..Following PE and subsequent CPB, neither patient had clinical or laboratory evidence of HIT. The literature regarding the use of PE for the treatment of complications of HIT and as prophylaxis before CPB is reviewed...

..Evidence-based guidelines for the management of acute coronary syndrome (ACS) in patients with congenital bleeding disorders are lacking and largely confined to case reports...

..Collaborative efforts between primary care providers, cardiologists and hemophilia center specialists remain essential in managing these complex patients...

..Imaging studies showed that by day 10 a popliteal cyst had ruptured, with diffusion of blood into the calf muscles. This case illustrates another possible bleeding complication in patients with a bleeding disorder and a popliteal cyst...

..To evaluate the efficacy of global endometrial ablation in women with bleeding disorders who presented with menorrhagia...

..021). These observations merit further study to gain insight into disease and/or therapy-specific pathogenesis of TE in this population and call for the prospective evaluation of appropriate prophylactic interventions...

..CONCLUSION: This analysis suggests that neutropenic colitis is a frequent and serious complication of idarubicin and cytosine arabinoside treatment...

..In view of previous studies linking the presence of the PlA2 allele of GPIIIa to a higher risk for coronary artery thrombosis, our data have physiologic relevance. However, they need to be confirmed in a larger study...