In the last 10-15 years, secondary HLH has been increasingly recognized in adults, where it is frequently associated with lymphoid malignancy, infection, or autoimmune disease. This relatively recently recognized diagnosis and the treatment of adult HLH have been largely shaped by observations in ...
Conclusions The main differences reside in the better prognosis of the patients with MAS and the lesser proportion associated with severe illness (p<0.005). The presence of autoimmune disease seems to act as a trigger factor per se, and the infection does not appear to play an important role ...
Hemophagocytic lymphohistiocytosis in adults: diagnosis and treatment. Joint Bone Spine. 2012;79(4):356–61. https://doi.org/10.1016/j. jbspin.2011.10.015. 6. Debaugnies F, Mahadeb B, Ferster A, Meuleman N, Rozen L, Demulder A, et al. Performances of the H-score for diagnosis of ...
Finally, a novel 1 bp deletion in gene SH2D1A was discovered. The result was also confirmed by Sanger sequencing. The result of the genetic test served as a good basis for further diagnosis of HLH. Conclusion: This is the first case that the disease-causing genetic defect of HLH was ...
HLH reflects a disbalanced immune system in response to infectious, malignancy, or autoinflammatory/autoimmune mediated triggers(1). The latter group of patients are regarded as having Macrophage Activation Syndrome (MAS-HLH)() In adults MAS-HLH comprises 12.5% of all HLH causing triggers(4). ...
Serum soluble interleukin-2 receptor (sIL-2r) is an important disease marker in hemophagocytic lymphohistiocytosis (HLH), but there are no published data on its diagnostic value in adults. We conducted a single-center retrospective study of 78 consecutive adults who had sIL-2r measured for ...