American journal of hematology
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Practice Guideline Guideline
Factors affecting choice of hemostatic agent for the hemophilia patient with an inhibitor antibody.
Achieving adequate hemostasis in patients with hemophilia and high-titer inhibitor antibodies remains a challenge despite the development of plasma-derived and recombinant therapies to bypass the inhibitory effect. A group of hemophilia treaters met to examine the factors that influence physician choices among the very costly and cumbersome available therapies. Specific knowledge deficits in need of prospective research were identified with respect to the rational treatment of inhibitor patients.
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The aim of our study was to assess the cytokine profile of sickle cell disease (SCD) patients in steady state and in vaso-occlusive crisis (VOC). VOC has a complex nature, involving interactions between sickle red blood cells (RBC), the endothelium, and leucocytes. Endothelial damage due to recurrent adhesion of sickle RBCs may disrupt endothelial function, leading to altered cytokine release. ⋯ It was also observed that SCD patients in steady state showed a significant elevation in IL-1beta (P < 0.04), IL-6 (P < 0.0001), and IFN-gamma (P < 0.02) as compared to normal subjects. It is thus evident that both type I and type II cytokines are significantly altered in SCD patients. In steady state, type II proinflammatory cytokines are elevated, whereas in crisis, an additional augmentation of type I cytokines occurs, with persistent elevation of type II cytokines, emphasizing the role of perturbed endothelium and activated monocytes in the pathophysiology of vaso-occlusion in sickle cell crisis.