Effect of Plasma Exchange on the Circulating IL‐6 Levels in a Patient With Fatal Hemophagocytic Syndrome Associated With Bile Ductopenia

Song, Kyung Soon; Sung, Hyun Joo

doi:10.1111/j.1744-9987.2006.00347.x

Cited by 20 publications

(11 citation statements)

References 12 publications

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“…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].…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2012

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IntroductionHyperferritinemia is associated with increased mortality in pediatric sepsis, multiple organ dysfunction syndrome (MODS), and critical illness. The International Histiocyte Society has recommended that children with hyperferritinemia and secondary hemophagocytic lymphohistiocytosis (HLH) or macrophage activation syndrome (MAS) should be treated with the same immunosuppressant/cytotoxic therapies used to treat primary HLH. We hypothesized that patients with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS can be successfully treated with a less immunosuppressant approach than is recommended for primary HLH.MethodsWe conducted a multi-center cohort study of children in Turkish Pediatric Intensive Care units with hyperferritinemia associated secondary HLH/sepsis/MODS/MAS treated with less immunosuppression (plasma exchange and intravenous immunoglobulin or methyl prednisolone) or with the primary HLH protocol (plasma exchange and dexamethasone or cyclosporine A and/or etoposide). The primary outcome assessed was hospital survival.ResultsTwenty-three children with hyperferritinemia and secondary HLH/sepsis/MODS/MAS were enrolled (median ferritin = 6341 μg/dL, median number of organ failures = 5). Univariate and multivariate analyses demonstrated that use of plasma exchange and methyl prednisolone or intravenous immunoglobulin (n = 17, survival 100%) was associated with improved survival compared to plasma exchange and dexamethasone and/or cyclosporine and/or etoposide (n = 6, survival 50%) (P = 0.002).ConclusionsChildren with hyperferritinemia and secondary HLH/sepsis/MODS/MAS can be successfully treated with plasma exchange, intravenous immunoglobulin, and methylprednisone. Randomized trials are required to evaluate if the HLH-94 protocol is helpful or harmful compared to this less immune suppressive and cytotoxic approach in this specific population.

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Section: Discussionmentioning

confidence: 99%

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

et al. 2012

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“…These may explain the variable therapeutic responses observed in dengue associated IAHS with supportive therapy, corticosteroids and IVIG. Role of plasma exchange in steroid refractory IAHS have been described [36][37][38].…”

Section: Discussionmentioning

confidence: 98%

Dengue infection associated hemophagocytic syndrome: Therapeutic interventions and outcome

Jamaludin

Periyasamy

Mat

et al. 2015

Journal of Clinical Virology

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“…After three sessions of plasma exchange with immunosuppressive therapy, the patient's symptoms and laboratory data were markedly improved. As reported previously (10–13), plasma exchange therapy was effective for HPS and would not only have corrected the high plasma cytokine levels, but also ameliorated the response to the corticosteroid by decreasing the levels of circulating inflammatory cytokines.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2009

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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.

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Effect of Plasma Exchange on the Circulating IL‐6 Levels in a Patient With Fatal Hemophagocytic Syndrome Associated With Bile Ductopenia

Cited by 20 publications

References 12 publications

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

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

Dengue infection associated hemophagocytic syndrome: Therapeutic interventions and outcome

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

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