A 36‐year‐old woman with recurrent high‐grade fevers, hypotension, and hypertriglyceridemia

Silencing the cytokine storm: the use of intravenous anakinra in haemophagocytic lymphohistiocytosis or macrophage activation syndrome

“…Most publications on cytokine storm syndromes describe subcutaneous use of anakinra; however, intravenous dos ing has been reported in eight patients (table 3, appendix pp 1-3) with equal gender distribution and a mean age of 22 years (range 22 months to 71 years). 41,[61][62][63][64][65][66] The reported underlying driver of cytokine storm in these patients included autoimmune or autoinflammatory diseases in three patients (one each with adultonset Still's disease, systemic juvenile idiopathic arthritis, and systemic lupus erythematosus). Underlying infection was diagnosed in three patients (one each with cytomegalovirus, septic arthritis, and human herpesvirus 8); one patient had both underlying auto immunity (vasculitis) and infection (cyto megalovirus); and another had underlying haema tological disease (myelodysplastic syndrome).…”

Diagnosis and Management of Adult Malignancy-Associated Hemophagocytic Lymphohistiocytosis

“…Naymagon, for instance, noted in a retrospective study of outcomes associated with anakinra treatment that those with rheumatologic condition-associated MAS experienced 75% OS, versus only 17% survival in patients with other underlying causes of sHLH [ 63 ]. Publications describing the use of anakinra for mHLH have been varied, ranging from frontline monotherapy use in Hodgkin lymphoma to relapsed/refractory HLH in myelodysplastic syndrome (MDS) and T cell lymphoma (TCL) [ 66 , 67 , 68 ]. The use of anti-IL6 therapy for adult HLH (with tocilizumab; no published data using siltuximab) has been extrapolated from its use in cytokine release syndrome (CRS) and COVID-19.…”