Journal
JOURNAL OF CANCER RESEARCH AND CLINICAL ONCOLOGY
Volume 146, Issue 4, Pages 1065-1077Publisher
SPRINGER
DOI: 10.1007/s00432-020-03139-4
Keywords
HLH; Hemophagocytic lymphohistiocytosis; Sepsis; Inflammation; Cytokine storm
Categories
Funding
- Projekt DEAL
- Jena Interdisciplinary Centre of Clinical Research (IZKF)
- Thuringer Aufbaubank [2014 KN 0024]
Ask authors/readers for more resources
Purpose Hemophagocytic lymphohistiocytosis (HLH) is a severe hyperinflammatory syndrome emerging from a deregulated immune response due to various triggers. In adults, systematic data are sparse, which is why recommendations on diagnosis and management have been adopted from pediatric guidelines. 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. Methods Patients with proven or suspected HLH were registered by 44 institutions. Both HLH-2004 diagnostic criteria and the HScore () were used to confirm HLH diagnosis. Data referring to underlying disease, treatment, outcome, clinical presentation and laboratory findings were recorded. Results The study included 137 patients and provides the first systematic data on adult HLH in Germany. Median age was 50 years with a wide range (17-87 years), 87 patients (63.5%) were male. Most common triggering diseases were infections in 61 patients (44.5%) and malignancies in 48 patients (35%). Virtually all patients had elevated ferritin concentrations, and 74% had peak concentrations greater than 10,000 mu g/l. At time of analysis, 67 of 131 patients (51%) had died. Patients with malignancy-associated HLH had the shortest median survival (160 days), however no statistically significant difference between subgroups was observed (p = 0.077). Platelets under 20*10(9)/l and low albumin concentrations (< 20 g/l) were associated with poor overall and 30-day survival. Conclusion Close multidisciplinary case consultation and cooperation is mandatory when treating adult HLH patients. Early contact with reference centers is recommended, especially in relapsing or refractory disease.
Authors
I am an author on this paper
Click your name to claim this paper and add it to your profile.
Reviews
Recommended
No Data Available