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Macrophage activation syndrome and other systemic inflammatory conditions after BMT

Bone Marrow Transplantation volume 37, pages 629–634 (2006)Cite this article

Abstract

Autologous hematopoietic cell transplantation (HCT) is being used to treat autoimmune diseases refractory to conventional therapy, including rheumatoid arthritis. Macrophage activation syndrome (MAS) is a descriptive term for a systemic inflammatory disorder that has been described in patients with juvenile rheumatoid arthritis (JRA). This case report describes a young adult with systemic JRA (sJRA) who developed MAS on day # 12 post-autologous transplantation. The patient developed high fever, laboratory evidence of disseminated intravascular coagulation (DIC), hepatocellular injury, pancytopenia and hyper-ferritinemia. All viral, bacterial and fungal studies were negative and the patient improved with high-dose glucorticosteroid and cyclosporine therapy. Extreme elevation of serum ferritin was documented and helpful in monitoring response to therapy. A number of systemic inflammatory syndromes have been described in association with HCT. These include DIC, ‘engraftment syndrome,’ infection-associated hemophagocytic syndrome and familial hemophagocytic lymphohistiocytosis. Macrophage activation syndrome presents with features of DIC and is closely related or identical to infection-associated hemophagocytic syndrome. The diagnosis needs to be established in a timely fashion because early and appropriate treatment may improve outcome.

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Authors and Affiliations

  1. Department of Medicine, Indiana University School of Medicine, Indianapolis, IN, USA

    A Sreedharan

  2. Department of Pediatrics, Section of Rheumatology, Indiana University School of Medicine, Indianapolis, IN, USA

    S Bowyer & K Schmidt

  3. Division of Rheumatology, Department of Pediatrics, University of Washington School of Medicine, Seattle, WA, USA

    C A Wallace

  4. Division of Hematology/Oncology, Indiana University School of Medicine, Indianapolis, IN, USA

    M J Robertson & R P Nelson Jr

  5. Division of Oncology, Department of Medicine, University of Washington School of Medicine, Seattle, WA, USA

    A E Woolfrey

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  1. A Sreedharan
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  2. S Bowyer
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Correspondence to R P Nelson Jr.

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Sreedharan, A., Bowyer, S., Wallace, C. et al. Macrophage activation syndrome and other systemic inflammatory conditions after BMT. Bone Marrow Transplant 37, 629–634 (2006). https://doi.org/10.1038/sj.bmt.1705305

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