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• W2318655363 abstract "Introduction: The mainstay of management of Hemophagocytic Lymphohistiocytosis (HLH) and Macrophage Activating Syndrome (MAS) is to decrease systemic inflammation. Therapies such as plasmapharesis, steroids, and chemotherapy leave patients vulnerable to life threatening infections. Here we present our experience of using Anakinra (Kineret®) a recombinant interleukin-1 receptor antagonist as a modality to decrease inflammation. Hypothesis: We hypothesized that Anakinra would be well tolerated and reverse some of the effects of the dysregulated immune system caused by HLH. Methods: A retrospective review of the management of eight children with HLH, treated at the PICU at Helen DeVos Chidren’s Hospital using various databases after IRB approval. Repeated measures ANOVA was used to analyze data (SPSS v. 17, 2008). Results: Eight patients with the median age of 14 years (5 males and 3 females) were administered Anakinra along with systemic steroids. The patients were admitted to the ICU for 9.3 days ±4.9. Indications for ICU admission were respiratory 50% (4/8), cardiovascular 37.5% (3/8) and chest pain 12.5% (1/8). Thirty three percent (3/9) of the patients received 4 courses of mechanical ventilation during their ICU stay. The median PRISM score was 9.5, with 3 patients having MODS on admission to the ICU. Clinical markers of HLH were assessed linearly to determine clinical course. Average serum ferritin at the start of Anakinra and steroids was 8864 ± 10891.8 ng/dL and by 5-7 days decreased to 3210 ± 3331.7 (p<0.173). CRP was 205.5 ± 130.9 mg/L at the start of Anakinra and decreased significantly by 67.1 % to 67.8 ± 94.3, (p<0.010). Fibrinogen increased: 119.2 ± 77.4 mg/dL to 158.4 ± 108.1 (p< 0.304) over the week of Anakinra therapy. Both ANC (p< 0.230), and ALC (p< 0.817) did not decrease significantly. None of the patients had any secondary infections despite surveillance cultures. Seven patients survived to Hospital discharge and 1 died months after stopping Anakinra of disseminated aspergillosis while receiving pre-HSCT chemotherapy. Conclusions: Anakinra administered with steroids was associated with a significant decrease in CRP in this small study and was not associated with immediate secondary infections." @default.
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• W2318655363 date "2012-12-01" @default.
• W2318655363 modified "2023-10-17" @default.
• W2318655363 title "1073" @default.
• W2318655363 doi "https://doi.org/10.1097/01.ccm.0000425286.38504.b2" @default.
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