Background: Hemophagocytic lymphohistiocytosis (HLH) can be familial or secondary, which is often triggered by infection or malignancy. HLH therapy includes dexamethasone and etoposide. However, therapy is associated with significant morbidity and mortality. Anakinra, a recombinant interleukin-1 receptor antagonist, has been reported to treat macrophage activation syndrome (MAS), rheumatic sHLH. We report our experience with anakinra to treat patients with nonrheumatic secondary HLH (sHLH). Procedure: Six children were diagnosed with HLH from December 2014 to August 2016 and were treated with subcutaneous anakinra (6-10 mg/kg/day divided over four doses) with or without dexamethasone (10 mg/m 2 /day). Therapy was either escalated or weaned based on clinical and laboratory response. Results: Five of six patients were treated with anakinra and dexamethasone, and one with anakinra alone due to active cytomegalovirus (CMV) pneumonitis. The median age of diagnosis was 1.8 years (range 0.8-14.9 years). No pathogenic mutations associated with HLH were identified, but three of six possessed genetic variants of unknown significance. Infectious triggers were identified for four patients and two patients had malignancies. The average treatment duration was 8 weeks with 3.5-5.5 years of follow up. No patient needed escalation of therapy to include etoposide. All patients achieved remission. Anakinra was well tolerated without significant adverse effects. Conclusion: Initial treatment with anakinra (with or without dexamethasone) is a feasible treatment alternative for patients with secondary HLH and may allow for avoidance of etoposide. We recommend early initiation of anakinra when HLH is suspected. A broader investigation of the use of anakinra as a first-line agent for HLH is ongoing.

Agenesis of the inferior vena cava (IVC) is a rare anomaly which can be identified as incidental finding or can be associated with iliofemoral vein thrombosis. IVC agenesis has a known association with renal anomalies which are mainly confined to the right kidney. We describe a case of a 14-year-old male who presented with left leg swelling and pain. Ultrasonography confirmed the presence of left leg deep vein thrombosis (DVT). No underlying hematologic risk factors were identified. A CT scan was obtained which demonstrated absent infrarenal IVC and extensive thrombosis in the left deep venous system and development of collateral venous flow into the azygous/hemiazygous system, with extension of thrombus into paraspinal collaterals. An additional finding in the patient was an atrophic left kidney and stenosis of an accessory left renal artery. Agenesis of the IVC should be considered in a young patient presenting with lower extremity DVT, especially in patients with no risk factors for thrombosis. As agenesis of the IVC cannot be corrected, one should be aware that there is a lifelong risk of lower extremity DVT.

No abstract

Novel human astroviruses (HAstVs) have recently been implicated as rare causes of fatal encephalitis in immunocompromised patients, for which there is no proven treatment. We report 2 cases from our institution in which HAstV-VA1 was detected in the cerebrospinal fluid by metagenomic next-generation sequencing after the initial evaluation revealed no etiology.

Introduction: Premedication with acetaminophen and/or diphenhydramine to prevent febrile nonhemolytic transfusion reactions and minor allergic transfusion reactions is a common practice based on historical recommendations. However, recent small randomized-controlled trials showed no benefit of premedication. This inconsistency leads to practice variability, which results in the inefficiency of our institution’s blood product ordering process. This project aimed to improve the number of transfusion encounters with premedication plan documentation from a baseline of 19% to 80% in 12 months. Methods: A multidisciplinary quality improvement (QI) team used QI tools to design interventions to improve the efficiency of the ordering process for blood products. Measures were tracked monthly and analyzed using statistical process control. Results: From September 2018 to January 2021, 5,351 blood product transfusion visits were scheduled. At baseline, 34% of patients received premedication, and 19% had premedication plans documented. Interventions included a passive computerized provider order entry alert, clinical care pathway development, and clinician education. Postimplementation, the average number of encounters with a premedication plan increased from 19% to 87%, whereas encounters receiving premedication decreased from 34% to 25%. There was no change in the average number of transfusion reactions (1.8 per 100 transfusions). Conclusions: Using QI methods, our team successfully standardized the blood product premedication plan documentation despite unclear best practices regarding blood product transfusion premedication. The team added premedication plan documentation training to new employee orientation for sustainability.

No abstract