Macrophage activation syndrome and other systemic inflammatory conditions after BMT

A. Sreedharan, S. Bowyer, C. A. Wallace, M. J. Robertson, K. Schmidt, A. E. Woolfrey, R. P. Nelson

Research output: Contribution to journalReview articlepeer-review

37 Scopus citations


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.

Original languageEnglish (US)
Pages (from-to)629-634
Number of pages6
JournalBone marrow transplantation
Issue number7
StatePublished - Apr 2006


  • Arthritis
  • Autoimmunity
  • Engraftment syndrome
  • Macrophage activation syndrome
  • Stem cell transplantation

ASJC Scopus subject areas

  • Hematology
  • Transplantation

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