Abstract
BackgroundHyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course.Case presentationWe describe two patients of 11 and 9 years of age with hyperferritinemic syndromes, one with impending macrophage activation syndrome (MAS) and one with overt MAS, who were refractory or intolerant to conventional therapies, but improved dramatically with canakinumab.ConclusionsOur report indicates that canakinumab may be efficacious in the management of hyperferritinemic syndromes, including MAS.
Highlights
Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course
Hyperferritinemic syndromes (HFS) are systemic inflammatory disorders characterized by a dysfunctional immune response, leading to excessive activation of the monocyte-macrophage system with hypercytokinemia, and pronounced hemophagocytosis [1]
Patient 2 A 9-year-old girl was diagnosed with systemic juvenile idiopathic arthritis (sJIA) and macrophage activation syndrome (MAS) at her local hospital based on the presence of high-spiking fever, erythematous rash and polyarthritis, together with increased acute phase reactants, AST, triglycerides and ferritin, and decreased platelet count and fibrinogen
Summary
Hyperferritinemic syndromes are systemic inflammatory disorders characterized by a dysfunctional immune response, which leads to excessive activation of the monocyte-macrophage system with hypercytokinemia and may pursue a rapidly fatal course. Three patients with sJIA associated MAS which was refractory to conventional therapies or could not be controlled with standard doses of ANK or CNK, but responded dramatically to one single injection of CNK at higher doses (7,5 to 15 mg/kg) have been reported in a meeting abstract [22].
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
