A Case Report of Successful Treatment With Plasma Exchange for Hemophagocytic Syndrome Associated With Severe Systemic Juvenile Idiopathic Arthritis in an Infant Girl

Abstract

An infantile case of hemophagocytic syndrome (HPS) with systemic juvenile idiopathic arthritis (s-JIA), refractory to methylprednisolone pulse therapy and cyclosporine A administration, was successfully treated by plasma exchange. The patient was a one-year-old Japanese girl who had developed recurrent steroid-dependent signs, including fever, skin eruption, and hepatopathy, while in France, where she had been diagnosed as having s-JIA at eight months of age. As a high fever and rheumatoid rash were evident on arrival at our hospital, she was admitted and given intravenous methylprednisolone pulse therapy and cyclosporine A. She developed pancytopenia with a generalized clonic seizure, high fever, and liver dysfunction after her cytomegalovirus (CMV) titer became positive during the course of treatment; therefore, she was treated with ganciclovir. She was subsequently diagnosed as having HPS complicating s-JIA from the findings of a bone marrow aspirate. At this time, her blood examination data including a high level of C-reactive protein and hyperferritinemia, suggested that her s-JIA was very active, and the pancytopenia continued after her CMV titer became negative. Therefore, CMV infection against a background of active s-JIA could have triggered the HPS in this case. Because the HPS was resistant to an immunosuppressive regime of methylprednisolone pulse therapy and cyclosporine A, plasma exchange therapy was started. After three sessions of this therapy, the patient's symptoms and laboratory data were markedly improved. Our experience suggests that plasma exchange should be considered as a therapeutic tool for HPS refractory to conventional therapy.

Hyperferritinemia in the critically ill child with secondary hemophagocytic lymphohistiocytosis/sepsis/multiple organ dysfunction syndrome/macrophage activation syndrome: what is the treatment?

“…PE therapy reverses HLH, possibly by decreasing circulating inflammatory cytokines, including macrophage colony-stimulating factor (M-CSF) [30,31]. There have also been anecdotal reports of patients with secondary HLH/MAS successfully treated with PE [30-32]. In the present study, PE was used as standard care for patients with secondary HLH/MAS to control hypercytokinemia.…”

“…PE is an extracorporeal blood purification technique designed to remove various toxic and inflammatory mediators and to replenish essential compounds via the replacement plasma. PE therapy reverses HLH, possibly by decreasing circulating inflammatory cytokines, including macrophage colony-stimulating factor (M-CSF) [30,31]. There have also been anecdotal reports of patients with secondary HLH/MAS successfully treated with PE [30-32].…”

The Hyperferritinemic Syndrome: macrophage activation syndrome, Still’s disease, septic shock and catastrophic antiphospholipid syndrome

“…Plasma exchange is an extracorporeal blood purification technique designed to remove various toxic and inflammatory mediators and to replenish essential compounds via the replacement plasma, which is known also to decrease ferritin levels [16]. It is a successful therapy in all four clinical conditions discussed, although in the case of the AOSD, there are only anecdotal cases [59,73-75,77,78,81,85-88]. …”

Management of the critically ill child with the sepsis/hemophagocytic lymphohistiocytosis/macrophage activation syndrome overlap syndrome

“…The overall concept of PE is in these diseases, therefore, is to attenuate the overwhelming systemic overflow of pro-and anti-inflammatory mediators released at the early phase of disease and to restore a broad-based humoral homeostasis. PE was found as a successful therapy in MAS, Still's disease, catastrophic antiphospholipid syndrome and sepsis [84][85][86][87][88]. Demirkol et al [51] investigated the efficacy of PE treatment in hyperferritinemia associated acquired HLH/SIRS/MODS/ MAS patients.…”