The evolution of hemophilia treatment and care is a fascinating one but has been fraught with many challenges at every turn. Over the last 50 years or so patients with hemophilia and providers have witnessed great advances in the treatment of this disease. With these advances, there has been a dramatic decrease in the mortality and morbidity associated with hemophilia. Even with the remarkable advancements in treatment, however, new and old challenges continue to plague the hemophilia community. The cost of factor replacement and the frequency of infusions, especially in patients with severe hemophilia on prophylaxis, remains a significant challenge for this population. Other challenges include obtaining reliable venous access, especially in younger patients, and the development of neutralizing alloantibodies (inhibitors). The development of extended half-life products, a bispecific antibody which mimics the coagulation function of factor VIII (FVIII) and inhibition of anticoagulation proteins such as antithrombin with antibodies, aptamers or RNA interference technology have offered novel therapeutic approaches to overcome some of these existing challenges. Additionally, ongoing gene therapy research offers a way to possibly cure hemophilia. These novel treatment tools in conjunction with the establishment of an increasing number of comprehensive hemophilia centers and worldwide advocacy efforts have continued to push the progress of hemophilia care to new frontiers. This review highlights and summarizes these novel therapeutic approaches and the current clinical progress of hemophilia A.

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This article highlights the range of osseous findings that can be encountered as well as the imaging features of extramedullary haematopoiesis. As iron overload remains a major cause of morbidity and mortality in these disorders, we also discuss the MRI evaluation of hepatic and cardiac hemosiderosis, to aid in the optimization of iron chelation therapy. Future imaging use will be dictated by evolving clinical needs, such as in screening for emerging morbidities, including hepatic fibrosis and hepatocellular carcinoma.

ObjectivesUnderstand the advantages of one type of decision support tool (DST) over another for advance care planning (ACP). Compare these two types of DST's for ACP and match a tool type to their own and their patient's needs.Original Research Background. Multiple paperbased and video-based decision support tools (DST) for advance care planning (ACP) exist and the advantages of one type or the other are unclear.Research Objectives. To explore the comparative effectiveness of video and paper DST for ACP.Methods. Researchers conducted a patient-centered multiple criteria decision analysis to compare video ACP DST against paper ACP DST across four broadly conceptualized outcome categories: (i) informed treatment choice; (ii) ACP status; (iii) opinions regarding ACP instrument; and (iv) patient-centered outcomes. Researchers identified studies that evaluated video or paper ACP DST, assigned each study a quality rating, and abstracted and categorized study outcome data. Using study quality ratings and the categorized study outcome data, researchers calculated an outcome category score and a standard error for the video DST and the paper DST. Differences between the video and paper outcome category scores were taken to indicate the DST format that favored a particular outcome category.Results. Three outcome categoriesdinformed treatment choice, opinion of ACP instrument, and patient-centered outcomesdeach favored the video DST format (p<0.001). The outcome category for ACP status favored the paper DST format (p<0.001).Conclusions. While video ACP DST formats were favored over paper DST formats for three out of the four outcome categories, the analysis does not account for patient preferences among the four outcome categories. The choice to use a video or paper ACP DST could vary depending on how patients prioritize the four different outcomes. Future research needs to identify patient preferences for these outcome categories to meaningfully assess which format best meets patient needs.Implications for Research, Policy, or Practice. The optimal type of decision support toold video as compared to paperdwill vary with the goals of the provider and the patient. ObjectivesGive the prevalence of opioid induced pruritus in a pediatric population. List at least 3 main risk factors for the development of opioid induced pruritus (OIP).Original Research Background. Opioids are frequently utilized in the pediatric hematology and oncology population for pain control. Pruritus from opioids can be as distressing and debilitating as pain. Little is known about opioid-induced pruritus (OIP) in the pediatric population, and even less in the pediatric hematology and oncology population.Research Objectives. This study sought to assess the prevalence and characteristics of OIP in the pediatric hematology and oncology population admitted to Virginia Commonwealth University Health System between June 2008 and December 2012. Methods. A retrospective chart review was per-formed. Descriptive statistics were calculated for pruritus, predictor v...

Since the advent of COVID-19 in 2019, the virus has affected all age groups and has a very wide clinical spectrum, ranging from asymptomatic infection to serious life-threatening complications including multi-organ dysfunction syndrome in children. The virus tends to affect all organ systems including the hematological system. There are many contradictory views on the effect of the COVID-19 pandemic on the incidence of hematological malignancies. Some studies have shown an increased incidence of acute lymphoblastic leukemia (ALL) after COVID-19 infection supporting the Greaves two-hit hypothesis of leukemogenesis, while others have shown a decline in the incidence of ALL postulated to be due to widespread lockdown and decreased exposure to environmental pathogens. We report the cases of three children who were diagnosed with acute lymphoblastic leukemia shortly after the initial diagnosis of multisystem inflammatory syndrome in children (MIS-C) or COVID related transient erythroblastopenia of childhood.