Urgent use of voxelotor in sickle cell disease when immediate transfusion is not safe.

The use of blood transfusions to improve anemia resulting from sickle cell disease (SCD) is often limited by alloimmunization, which occurs due to exposure to incompatible antigen present on donor red blood cells (RBCs). This complication occasionally manifests as delayed hemolytic transfusion reactions (DHTRs) that cause hemolysis of the recipient's own RBCs and can lead to fatal anemia. In this case study, we report a patient with SCD who experienced a DHTR following chronic transfusion and was successfully treated with voxelotor, an orally administered sickle hemoglobin (HbS) polymerization inhibitor for the treatment of SCD.

Changes in Mean Corpuscular Volume and RBC Distribution Width Predict Erythrocyte Engraftment Following ABO-Incompatible Hematopoietic Stem Cell Transplantation.

Objectives: The purpose of this study was to identify laboratory parameters representing erythrocyte engraftment to be used as an indicator to change the recipient to donor ABO group and Rh type following an ABO-incompatible hematopoietic stem cell transplant (HSCT). Studies have shown that ABO incompatibility does not have an effect on outcome of HSCT; however, the serologic consequences of these ABO-incompatible transplants can make it difficult to decide when to begin support with donor ABO/Rh-type blood products.

Methods: This study explored the use of RBC distribution width (RDW), mean corpuscular volume, and hemoglobin as regularly tested laboratory parameters that could be used as surrogate markers for RBC engraftment in 65 patients who received ABO/Rh-incompatible HSCT.

Comorbidity, Pain, Utilization, and Psychosocial Outcomes in Older versus Younger Sickle Cell Adults: The PiSCES Project.

. Patients with SCD now usually live well into adulthood. Whereas transitions into adulthood are now often studied, little is published about aging beyond the transition period.

Daily home opioid use in adults with sickle cell disease: The PiSCES project.

Background: Although opioid prescribing in sickle cell disease (SCD) can be controversial, little is published about patterns of opioid use.

Objective: To report on home opioid use among adults with SCD.

Design: Cohort study.

Policies and procedures related to testing for weak D phenotypes and administration of Rh immune globulin: results and recommendations related to supplemental questions in the Comprehensive Transfusion Medicine survey of the College of American Pathologists.

Context: Advances in RHD genotyping offer an opportunity to update policies and practices for testing weak D phenotypes and administration of Rh immune globulin to postpartum women.

Objectives: To repeat questions from a 1999 College of American Pathologists proficiency test survey, to evaluate current practices for testing for weak D and administration of Rh immune globulin, and to determine whether there is an opportunity to begin integrating RHD genotyping in laboratory practice.

Design: The College of American Pathologists Transfusion Medicine Resource Committee sent questions from the 1999 survey to laboratories that participated in the 2012 proficiency test survey.

Harvesting autologous stem cells from a patient with red blood cell abnormalities of β-thalassemia intermedia.

Background: Autologous stem cell transplants in patients with hemoglobinopathies are limited. Previous reports used granulocyte-colony-stimulating factor (G-CSF) for mobilization of stem cells; there are no reported cases undergoing plerixafor mobilization. We report such a patient, providing guidance for peripheral blood stem cells collection when aberrant red blood cells (RBCs) disrupt normal separation.

Perfluorocarbon emulsion improves oxygen transport of normal and sickle cell human blood in vitro.

Perfluorocarbons (PFC) are compounds with high gas solubility that could help deliver O2 to tissues and have been suggested as adjunct therapy to ischemia. Using a newly designed in vitro system, we tested the hypothesis that a third generation PFC emulsion (Oxycyte) increased O2 transport of blood by measuring changes in O2 extraction ratio. The system included a computer-controlled pump and blood-gas exchange chambers to oxygenate and deoxygenate the blood from nine sickle cell disease (SCD) patients and five healthy donors.

Red blood cell alloimmunization in sickle cell disease: prevalence in 2010.

Background: Transfusion of red blood cells (RBCs) is frequently required for care of individuals with sickle cell disease (SCD). Alloimmunization rates are high and may be reduced by matching for RBC antigens that can cause alloimmunization.

Study Design And Methods: During the PROACTIVE Feasibility Study, patients with SCD age 2 years or older admitted for pain without acute chest syndrome were enrolled for possible randomization to preventive blood transfusion or standard care.

Refining the value of secretory phospholipase A2 as a predictor of acute chest syndrome in sickle cell disease: results of a feasibility study (PROACTIVE).

Acute chest syndrome (ACS) is defined as fever, respiratory symptoms and a new pulmonary infiltrate in an individual with sickle cell disease (SCD). Nearly half of ACS episodes occur in SCD patients already hospitalized, potentially permitting pre-emptive therapy in high-risk patients. Simple transfusion of red blood cells may abort ACS if given to patients hospitalized for pain who develop fever and elevated levels of secretory phospholipase A2 (sPLA2).

Investigation of heparin-related hypotensive adverse events during photopheresis: utility of a patient care database.

Background: Extracorporeal photopheresis (ECP) is a procedure in which leukocytes are harvested from a patient's whole blood, treated with a DNA binding dye and ultraviolet light to inactivate lymphocytes, and then returned into the patient's circulation. In January 2008, we observed moderately severe anaphylactoid reactions in eight of 16 patients undergoing ECP.

Case Study: Each affected individual exhibited hypotension of sudden onset, usually with tachycardia, during the return of heparin-anticoagulated blood at the end of the first cycle of collection of leukocytes.

Blood component preferences of transfusion services supporting infant transfusions: a University HealthSystem Consortium benchmarking study.

Background: The extent of acceptability of red blood cells (RBCs) containing additive solutions (ASs) for low-volume neonatal transfusions among hospitals is unknown. Also unknown is whether hospitals have policies that address the risk of hyperkalemia associated with prolonged storage either with or without irradiation for neonatal transfusions.

Study Design And Methods: A benchmarking survey of University HealthSystem Consortium members included questions regarding the acceptability of RBC units containing ASs for low-volume neonatal transfusions, policies addressing the length of RBC storage in AS, and policies regarding storage periods after irradiation.

Pain site frequency and location in sickle cell disease: the PiSCES project.

Treatment options for sickle cell disease (SCD) pain could be tailored to pain locations. But few epidemiologic descriptions of SCD pain location exist; these are based on few subjects over short time periods. We examined whether SCD pain locations vary by disease genotype, gender, age, frequency of pain, depression, pain crisis or healthcare utilization.

Comparisons of high versus low emergency department utilizers in sickle cell disease.

Study Objective: Patients with sickle cell disease often receive a substantial amount of their health care in the emergency department (ED) and some come to the ED frequently, seeking treatment for pain. As a result, patients with sickle cell disease are often stigmatized as opioid-seeking ED overutilizers. We describe the proportion of sickle cell disease patients who are high utilizers of the ED and compare them with other sickle cell disease patients on demographics, pain characteristics, health data, psychosocial characteristics, and quality of life.

Daily assessment of pain in adults with sickle cell disease.

Background: Researchers of sickle cell disease have traditionally used health care utilization as a proxy for pain and underlying vaso-occlusion. However, utilization may not completely reflect the amount of self-reported pain or acute, painful episodes (crises).

Objective: To examine the prevalence of self-reported pain and the relationship among pain, crises, and utilization in adults with sickle cell disease.

Depression and anxiety in adults with sickle cell disease: the PiSCES project.

Objective: Depression and anxiety are common in sickle cell disease (SCD) but relatively little is known about their impact on SCD adults. This study measured prevalence of depression and anxiety in SCD adults, and their effects on crisis and noncrisis pain, quality-of-life, opioid usage, and healthcare utilization.

Methods: The Pain in Sickle Cell Epidemiology Study is a prospective cohort study in 308 SCD adults.

Alcohol abuse in sickle cell disease: the Pisces Project.

Alcohol abuse is common in patients with chronic painful medical disorders, but it has not been studied in sickle cell disease (SCD). In a prospective cohort study of SCD adults, 31.4% were identified as abusing alcohol.

Patient satisfaction in specialized versus nonspecialized adult sickle cell care centers: the PiSCES study.

Background: Sickle cell disease (SCD) patients can receive their ambulatory care from either SCD specialists (caregivers with sickle cell-only clinics) or nonspecialized care centers. Patient satisfaction, an important factor that may influence compliance and outcome, can differ between the two groups because of the perceived quality of care, outcomes or practice style.

Methods: We administered a patient satisfaction survey to 308 participants in an SCD prospective cohort study.

The role of catastrophizing in sickle cell disease--the PiSCES project.

In several types of chronic pain, catastrophizing has been related to higher pain intensity, and health care utilization but it has not been explored extensively in sickle cell disease (SCD). The objective of the study was to identify the role of catastrophizing in SCD, specifically in relation to painful crises, non-crisis pain, and responses to pain. Two hundred and twenty SCD adults were enrolled in a prospective cohort study of pain and completed between 30 and 188 daily diaries in 6 months.