Distinguishing ASH clinical practice guidelines from other forms of ASH clinical advice.

The American Society of Hematology (ASH) develops a variety of resources that provide guidance to clinicians on the diagnosis and management of blood diseases. These resources include clinical practice guidelines (CPGs) and other forms of clinical advice. Although both ASH CPGs and other forms of clinical advice provide recommendations, they differ with respect to the methods underpinning their development, the principal type of recommendations they offer, their transparency and concordance with published evidence, and the time and resources required for their development.

A multilevel mHealth intervention boosts adherence to hydroxyurea in individuals with sickle cell disease.

Hydroxyurea reduces sickle cell disease (SCD) complications, but medication adherence is low. We tested 2 mobile health (mHealth) interventions targeting determinants of low adherence among patients (InCharge Health) and low prescribing among providers (HU Toolbox) in a multi-center, non-randomized trial of individuals with SCD ages 15-45. We compared the percentage of days covered (PDC), labs, healthcare utilization, and self-reported pain over 24 weeks of intervention and 12 weeks post-study with a 24-week preintervention interval.

What does it mean to be affiliated with care?: Delphi consensus on the definition of "unaffiliation" and "specialist" in sickle cell disease.

Accruing evidence reveals best practices for how to help individuals living with Sickle Cell Disease (SCD); yet, the implementation of these evidence-based practices in healthcare settings is lacking. The Sickle Cell Disease Implementation Consortium (SCDIC) is a national consortium that uses implementation science to identify and address barriers to care in SCD. The SCDIC seeks to understand how and why patients become unaffiliated from care and determine strategies to identify and connect patients to care.

A randomised controlled provider-blinded trial of community health workers in sickle cell anaemia: effects on haematologic variables and hydroxyurea adherence.

Hydroxyurea (hydroxycarbamide) (HU) for sickle cell anaemia (SCA) is underutilised. Case management is an evidence-based health management strategy and in this regard patient navigators (PNs) may provide case management for SCA. We hypothesised that HU-eligible patients exposed to PNs would have improved indicators of starting HU and HU adherence.

Outcomes of an Emergency Department Observation Unit-Based Pathway for the Treatment of Uncomplicated Vaso-occlusive Events in Sickle Cell Disease.

Study Objective: This was a prospective, pre-post, 13-year observational study documenting the multiyear implementation of an observation unit sickle cell pathway for patients with uncomplicated vaso-occlusive events.

Methods: The sickle cell pathway begins with rapid triage to identify patients with uncomplicated vaso-occlusive events for immediate transfer to the observation unit and initiation of patient-controlled analgesia followed by repeated evaluations of pain and identification of other complications. Data were abstracted from the electronic medical record or observation unit database.

Integration of Mobile Health Into Sickle Cell Disease Care to Increase Hydroxyurea Utilization: Protocol for an Efficacy and Implementation Study.

Background: Hydroxyurea prevents disease complications among patients with sickle cell disease (SCD). Although its efficacy has been endorsed by the National Health Lung and Blood Institute evidence-based guidelines, its adoption is low, both by patients with SCD and providers. Mobile health (mHealth) apps provide benefits in improving medication adherence and self-efficacy among patients with chronic diseases and have facilitated prescription among medical providers.

A user guide to the American Society of Hematology clinical practice guidelines.

Since November 2018, Blood Advances has published American Society of Hematology (ASH) clinical practice guidelines on venous thromboembolism, immune thrombocytopenia, and sickle cell disease. More ASH guidelines on these and other topics are forthcoming. These guidelines have been developed using consistent processes, methods, terminology, and presentation formats.

Inferior vena cava filters in trauma patients: a national practice patterns survey of U.S. Trauma centers.

Inferior vena cava filters (IVCFs) for thromboprophylaxis in trauma patients are being increasingly used despite a lack of strong clinical data in support of their efficacy and conflicting clinical practice guidelines. This national survey elucidates practice patterns of IVCF use across U.S.

Approach to the diagnosis and treatment of acute subarachnoid hemorrhage in a patient with sickle cell disease.

We report a case of subarachnoid hemorrhage in a 38-year-old patient with sickle cell anemia. This case highlights the limitations of non contrast computed tomography in the diagnosis of subarachnoid hemorrhage in patients with sickle cell disease as well as the special needs of this patient population in addressing the treatment of stroke.

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.

Drug-loaded sickle cells programmed ex vivo for delayed hemolysis target hypoxic tumor microvessels and augment tumor drug delivery.

Selective drug delivery to hypoxic tumor niches remains a significant therapeutic challenge that calls for new conceptual approaches. Sickle red blood cells (SSRBCs) have shown an ability to target such hypoxic niches and induce tumoricidal effects when used together with exogenous pro-oxidants. Here we determine whether the delivery of a model therapeutic encapsulated in murine SSRBCs can be enhanced by ex vivo photosensitization under conditions that delay autohemolysis to a time that coincides with maximal localization of SSRBCs in a hypoxic tumor.

Transfusion practices in the management of sickle cell disease: a survey of Florida hematologists/oncologists.

The purpose of this study was to characterize transfusion practices in the management of sickle cell disease and to identify factors attributing to differences in prescribing practices among Florida hematologists/oncologists. A cross-sectional study was performed in 2005-2006 utilizing a mail survey. The survey instrument addressed practice characteristics, sickle cell patient populations, transfusion settings, indications and techniques, red blood cell phenotype specifications/modifications, use of practice guidelines, and educational resource utilization.

A pilot study on the randomization of inferior vena cava filter placement for venous thromboembolism prophylaxis in high-risk trauma patients.

Background: Placement of prophylactic inferior vena cava filters (pIVCFs) for the prevention of pulmonary embolism (PE) in high-risk trauma patients (HRTPs) are widely practiced despite the lack of Level I data supporting this use. We report the 2-year interim analysis of the Filters in Trauma pilot study.

Methods: This is a single institution, prospective randomized controlled pilot feasibility study in a Level I trauma center.

Survival of trauma patients after massive red blood cell transfusion using a high or low red blood cell to plasma transfusion ratio.

Objective: Early and aggressive treatment of trauma-associated coagulopathy through transfusion of high plasma to packed red blood cell ratios is gaining favor. Whether this strategy is associated with improved survival is unclear. We performed a systematic review to determine whether higher plasma to packed red blood cell ratios compared with lower plasma to packed red blood cell ratios were associated with a survival advantage.

Pulmonary embolism prophylaxis with inferior vena cava filters in trauma patients: a systematic review using the meta-analysis of observational studies in epidemiology (MOOSE) guidelines.

Prophylactic inferior vena cava filters (pIVCFs) for the prevention of pulmonary embolism (PE) are controversial. Current practice guidelines (EAST and ACCP) are based on the critical appraisal of observational studies. As a result, their recommendations are conflicting and may account for practice pattern variation.

Bacterial adaptation and infection.

There is evidence that many pathogens co-evolve with their hosts. This is often reflected in species specific virulence factors that can selectively interfere with host defense mechanisms, innate and acquired as well as a range of interactions with host homeostatic pathways that contribute to the course and severity of an infection. In this review, we highlight a number of select examples of these interactions and suggest that understanding of molecular pathogenesis requires a broad systems approach that can evaluate the multiple and dynamics interactions that are occurring during infection.

Thrombotic storm in Kimura disease.

We report herein a patient with Kimura disease who experienced life threatening visceral venous thromboses and a clinical course fitting the description of "thrombotic storm". Kimura disease is an indolent chronic inflammatory disease common in Asians and characterized by angiolymphoid proliferation with ample eosinophil infiltration, peripheral blood eosinophilia and elevated serum immunoglobulin E levels. The clinical course of the disease is thought to be benign.