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Paroxysmal nocturnal hemoglobinuria (PNH) is a debilitating and life-threatening disease in which lysis of PNH red blood cells frequently manifests with chronic hemolysis, anemia, and thrombosis. Renal damage in PNH is associated with chronic hemosiderosis and/or microvascular thrombosis. We determined the incidence of renal dysfunction or damage, defined(More)
Mapping the functional response of the somatosensory cortex is useful both for characterizing normal brain activity and for determining the functional integrity of damaged cortex compromised by stroke or other neurological insults. A variety of stimulators have been used to produce somatosensory cortex activation in functional brain imaging, including(More)
BACKGROUND We tested the safety and efficacy of eculizumab, a humanized monoclonal antibody against terminal complement protein C5 that inhibits terminal complement activation, in patients with paroxysmal nocturnal hemoglobinuria (PNH). METHODS We conducted a double-blind, randomized, placebo-controlled, multicenter, phase 3 trial. Patients received(More)
Paroxysmal nocturnal haemoglobinuria (PNH) is characterized by chronic, uncontrolled complement activation resulting in elevated intravascular haemolysis and morbidities, including fatigue, dyspnoea, abdominal pain, pulmonary hypertension, thrombotic events (TEs) and chronic kidney disease (CKD). The long-term safety and efficacy of eculizumab, a humanized(More)
The molecular pathobiology of membrane-associated iron is clearly illustrated by the sickle red blood cell. The cytosolic aspect of the membranes of these cells carries several discrete iron compartments, including denatured hemoglobin and free heme, as well as molecular iron associated with membrane aminophospholipid and denatured globin. Affinity of the(More)
Pyrrolo-1,5-benzoxazepine-15 (PBOX-15) is a novel microtubule depolymerization agent that induces cell cycle arrest and subsequent apoptosis in a number of cancer cell lines. Chronic lymphocytic leukemia (CLL) is characterized by clonal expansion of predominately nonproliferating mature B cells. Here, we present data suggesting PBOX-15 is a potential(More)
Several physiological and pathophysiological events involving vascular endothelium occur at the microvascular level. Studies on human microvasculature require homogenous primary cultures of microvascular endothelial cells. However, procedures available for isolating and culturing human dermal microvascular cells (HDMEC) result in significant contamination(More)
Wilms' tumor gene 1 (WT1) is overexpressed in the majority (70-90%) of acute leukemias and has been identified as an independent adverse prognostic factor, a convenient minimal residual disease (MRD) marker and potential therapeutic target in acute leukemia. We examined WT1 expression patterns in childhood acute lymphoblastic leukemia (ALL), where its(More)
Vasoocclusive episodes in sickle cell anemia may be initiated by adherence of erythrocytes to endothelium, one mechanism of which involves thrombospondin binding to CD36 on the red blood cell (RBC). We compared CD36 expression and its relationship to stress reticulocytosis in patients with sickle cell anemia and other chronic hemolytic disorders, including(More)
BACKGROUND The vascular wall participates in the pathogenesis of sickle cell disease. To determine whether the endothelium is activated in this disease, we studied the number, origin, and surface phenotype of circulating endothelial cells in patients with sickle cell anemia. METHODS We used immunohistochemical examination of buffy-coat smears to enumerate(More)