Alloimmunization in sickle cell disease: changing antibody specificities and association with chronic pain and decreased survival.

Background: Alloimmunization remains a significant complication of transfusion and has been associated with multiple factors, including inflammation, an important pathophysiologic mechanism in sickle cell disease (SCD). We explored whether alloimmunization is associated with disease severity in SCD.

Study Design And Methods: Adult SCD patients were enrolled in a study of outcome-modifying genes in SCD.

Management of sickle cell disease: summary of the 2014 evidence-based report by expert panel members.

Importance: Sickle cell disease (SCD) is a life-threatening genetic disorder affecting nearly 100,000 individuals in the United States and is associated with many acute and chronic complications requiring immediate medical attention. Two disease-modifying therapies, hydroxyurea and long-term blood transfusions, are available but underused.

Objective: To support and expand the number of health professionals able and willing to provide care for persons with SCD.

Seasonal association of thrombotic thrombocytopenic purpura.

Background: Thrombotic thrombocytopenic purpura (TTP), a thrombotic microangiopathy, is a clinical diagnosis, characterized by microangiopathic hemolytic anemia and thrombocytopenia without another likely explanation. Some initiators of the disease are well represented in the literature, such as certain drugs, malignancies, and viral illness; however, there are less objective factors still being investigated, with references to hormonal, stress, and seasonal variations considered anecdotally. A better insight of these factors would aid in understanding the pathophysiology of the disease.

Lack of Duffy antigen expression is associated with organ damage in patients with sickle cell disease.

Background: The Duffy glycoprotein (Fy) on red blood cells (RBCs) has been hypothesized to promote clearance of inflammatory cytokines, which may play a role in the pathogenesis of vasoocclusion in sickle cell disease (SCD). Persons with the African-type Fy(a-b-) phenotype--whose RBCs lack expression of Duffy--may less efficiently clear inflammatory cytokines. Therefore, the Duffy-negative genotype may be associated with more severe disease among patients with SCD.

Blood bank management of sickle cell patients at comprehensive sickle cell centers.

Background: Transfusion therapy has been integral to decreasing morbidity and mortality in sickle cell disease (SCD). Several studies however, have demonstrated variation in the blood bank (BB) practices for these patients. The purpose of this study was to examine BB practices for SCD patients at NIH Comprehensive Sickle Cell Centers (CSCCs) and to determine whether consensus in BB management exists.

Update on multi-center clinical trials in the United States.

This article reviews numerous multi-center clinical trials, either ongoing or in planning stages, which involve diverse clinical applications and emerging technologies in apheresis and transfusion medicine. The investigations summarized herein involve the following specific areas: platelet dosing strategy, thrombotic thrombocytopenia purpura, inflammatory bowel disease, seven-day platelet storage, dendritic cell vaccines, and age-related macular degeneration.

Opportunities to improve outcomes in sickle cell disease.

Sickle cell disease represents a spectrum of inherited hemoglobin disorders. The pathophysiology involves abnormalities not just in red blood cells but also vascular endothelium, white blood cell function, coagulation, and inflammatory response. Known sequelae of sickle cell disease include invasive infections, painful episodes, acute chest syndrome, strokes, and chronic pulmonary hypertension.