By convention, and with impact on differential treatment, HLH in patients with autoimmune/autoinflammatory diseases is also called macrophage activation syndrome (MAS-HLH) (Emile et al. 2014), as are patients with autoimmune/autoinflammatory disorders. Patients with long-term immunosuppression are at increased risk to develop HLH (Ramos-Casals et al. In adults, this often fatal aberrant immune response most frequently is triggered by infections and malignancies, or a combination of these. Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome driven by excessive activation and stimulation of cytotoxic T-lymphocytes, natural killer T-cells and macrophages with subsequent cytokine storm and organ damage (Janka and Lehmberg 2014). Early contact with reference centers is recommended, especially in relapsing or refractory disease. ConclusionĬlose multidisciplinary case consultation and cooperation is mandatory when treating adult HLH patients. Platelets under 20*10 9/l and low albumin concentrations (< 20 g/l) were associated with poor overall and 30-day survival. Patients with malignancy-associated HLH had the shortest median survival (160 days), however no statistically significant difference between subgroups was observed ( p = 0.077). At time of analysis, 67 of 131 patients (51%) had died. Virtually all patients had elevated ferritin concentrations, and 74% had peak concentrations greater than 10,000 µg/l. Most common triggering diseases were infections in 61 patients (44.5%) and malignancies in 48 patients (35%). Median age was 50 years with a wide range (17–87 years), 87 patients (63.5%) were male. The study included 137 patients and provides the first systematic data on adult HLH in Germany. Data referring to underlying disease, treatment, outcome, clinical presentation and laboratory findings were recorded. Both HLH-2004 diagnostic criteria and the HScore ( were used to confirm HLH diagnosis. Patients with proven or suspected HLH were registered by 44 institutions. A nationwide clinical registry with associated consulting service as collaborative initiative of HLH-specialized pediatricians and hematologists was initiated to better characterize HLH in adults. In adults, systematic data are sparse, which is why recommendations on diagnosis and management have been adopted from pediatric guidelines. Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers.
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