Sickle Cell

A higher TRV was significantly associated with cerebrovascular disease and persistent albuminuria in children with SCD across two large cohorts.

A recent multi-center study identified risk factors linked to premature mortality in sickle cell disease, including male gender and hematologic and biochemical parameters.

In a nationwide study of an adult SCD population, the risk of mortality increased with the number of HVOCs in the year before death.

Our March 2024 month-in-review in hematology features updates to the anemia pipeline, the latest research in sickle cell disease, and the role of SGLT2 inhibition on hematologic outcomes.

A systematic review highlights the available evidence on the use of intravenous fluid in sickle cell vaso-occlusive crises and reported adverse outcomes.

A new analysis summarized evidence on the effects of allogeneic HSCT on SCD-related organ dysfunction in pediatric and adult patients with SCD.

Chronic kidney disease was linked to increased mortality, higher costs, and the need for mechanical ventilation among hospitalized patients with sickle cell disease.

Results from the PHEDRE study show nitrous oxide in a gas mixture for pain was not problematic for most patients with sickle cell disease in France.

In a registry-based analysis, more hospitalized children with SCD and COVID-19 had severe infection requiring supplemental oxygen, compared with the general population.

The 2016 CDC guidelines led to significant decreases in opioid prescriptions and an increase in pain-related healthcare utilization for people with sickle cell disease.

An ancillary study of the phase 2 HOPE-KIDS 1 trial revealed voxelotor is associated with reduced sickling and hemolysis in children with SCD.

A meta-analysis revealed a higher prevalence of abnormal ECG findings in individuals with SCD compared to those without the disease.

Voxelotor decreases oxygen extraction fraction and cerebral blood flow towards levels observed in healthy children.

No concerning patterns of long-term or increasing opioid use were observed within 3 years after first opioid prescription in opioid-naive children with SCD.

Hearing loss was more prevalent among children and adults with sickle cell disease and its traits compared to matched controls with normal hemoglobin.

Over the study period, pain screening procedures were integrated within routine clinical visits and providers’ workflow in sickle cell disease care.

A systematic review combined with a pair of case studies suggest men who discontinue the front-line treatment for conditions like polycythaemia vera may improve their fertility.

Presented at ASH 2023, findings of a new study showed patients with sickle cell disease and venous thromboembolism have longitudinal worsening of left ventricular diastolic and right-sided cardiac function.

Presented at ASH 2023, opioid use disorder in sickle cell disease patients with vaso-occlusive is associated with a longer hospital stay and greater cost.

A higher prevalence of STI diagnosis is observed in adolescents and young adults with sickle cell disease compared with controls.

Extension analysis from a phase 1 trial show the investigative oral mitapivat was not associated with any discontinuations nor deaths over 2 years.

New case-control data from ASH 2023 show a particularly increased link between sickle cell trait and monocytic and myeloid leukemia progression.

The findings show liver complications and cirrhosis as major causes of morbidity in patients with SCD, with implications for treatment and care.

Experts emphasize the fundamental principles of integrative care, the success seen in treating rare diseases, and how it can be applied to all chronic diseases.

According to investigators, this is among the largest studies describing the risk of bacteremia in young patients with sickle cell disease.

Among other associations, those in educational attainment, employment status, mental health, and pain episode frequency and severity highlight the need for comprehensive care that considers the psychological well-being of individuals with SCD.

Qualitative findings show children with sickle cell disease have the desire to feel normal and utilize personal strengths to manage their condition.

Results show a high level of adherence to hydroxyurea medication is essential for lowering the incidence of negative clinical outcomes like VOCs and acute chest syndrome in children with sickle cell anemia.

Individuals with sickle cell disease report a higher rate of infertility than the general population, but seeking fertility care treatment is lower.

Investigators address 2 questions: How can we better understand the biology of these rare disorders? How can we stimulate research and optimize the regulatory process to improve diagnosis and targeted treatment?