The use of anakinra in the treatment of secondary hemophagocytic lymphohistiocytosis.

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/m2 /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.

Comparison of EMLA Cream versus Lidocaine Injection for Lumbar Puncture Pain Control in Pediatric Oncology Patients.

Background Subcutaneous lidocaine injection and topical EMLA cream are both used to control lumbar puncture (LP) pain; however, local analgesia usage is not standardized. Methods We conducted a prospective, single-blinded, randomized-controlled crossover trial comparing the two modalities in reducing LP pain. Pediatric patients requiring serial LPs were randomly assigned to receive EMLA cream or lidocaine injection prior to LP. On the subsequent LP, analgesia was defaulted to the other agent. Pain was assessed using the Wong-Baker FACES Pain Rating Scale pre-procedure: 30 to 60 minutes post-LP, and 24 hours post-procedure. Results Ten patients were included in the analysis (median age: 5.5 years). Pain ratings at 1 and 24 hours post-LP did not differ between the two strategies ( p = 0.79). No adverse local reactions were reported for either agent. Conclusion Accordingly, both lidocaine and EMLA cream provided effective LP pain control.