Benefit of Anakinra in Treating Pediatric Secondary Hemophagocytic Lymphohistiocytosis.

Journal: 2019/September - Arthritis and Rheumatology

ISSN: 2326-5205

Abstract:

To assess the benefit of the recombinant human interleukin-1 receptor antagonist, anakinra, in treating pediatric patients with secondary hemophagocytic lymphohistiocytosis (sHLH) / macrophage activation syndrome (MAS) associated with rheumatologic and non-rheumatologic conditions.We performed a retrospective chart review of all anakinra-treated sHLH/MAS patients at Children's of Alabama from January 2008 through December 2016. Demographic, clinical, laboratory, genetic, concurrent treatment, and outcome data were collected and analyzed by appropriate univariate statistical approaches.Forty-four sHLH/MAS patients treated with anakinra were identified in the electronic medical record. Median duration of hospitalization was 15 days. The mean pre-treatment serum ferritin level was 33,316 ng/mL and dropped to 14,435 (57% decrease) within 15 days of starting anakinra. The overall mortality for the cohort was 27%. Earlier initiation of anakinra (≤5 days hospitalization) was associated with reduced mortality (p=0.046), whereas thrombocytopenia (<100,000/μL) and STXBP2 mutations were both associated with increased mortality (p=0.008 and p=0.012, respectively). Considering the underlying diagnosis, systemic juvenile idiopathic arthritis (sJIA) had the lowest mortality rate, with no deaths among the 13 sJIA patients included in the study (p=0.006). In contrast, underlying hematologic malignancy had the highest mortality rate at 100% (n=3).These findings suggest anakinra appears to be effective in non-malignancy associated pediatric sHLH, especially when given early in disease course and in patients with an underlying rheumatic disease etiology. This article is protected by copyright. All rights reserved.

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