Macrophage activation syndrome and other systemic inflammatory conditions after BMT

A. Sreedharan, S. Bowyer, C. A. Wallace, Michael Robertson, K. Schmidt, A. E. Woolfrey, Robert Nelson

Research output: Contribution to journalArticle

35 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)629-634
Number of pages6
JournalBone Marrow Transplantation
Volume37
Issue number7
DOIs
StatePublished - Apr 2006

Fingerprint

Macrophage Activation Syndrome
Hemophagocytic Lymphohistiocytosis
Disseminated Intravascular Coagulation
Cell Transplantation
Pancytopenia
Juvenile Arthritis
Autologous Transplantation
Therapeutics
Ferritins
Cyclosporine
Autoimmune Diseases
Young Adult
Rheumatoid Arthritis
Fever
Wounds and Injuries
Serum

Keywords

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

ASJC Scopus subject areas

  • Hematology
  • Transplantation

Cite this

Macrophage activation syndrome and other systemic inflammatory conditions after BMT. / Sreedharan, A.; Bowyer, S.; Wallace, C. A.; Robertson, Michael; Schmidt, K.; Woolfrey, A. E.; Nelson, Robert.

In: Bone Marrow Transplantation, Vol. 37, No. 7, 04.2006, p. 629-634.

Research output: Contribution to journalArticle

Sreedharan, A. ; Bowyer, S. ; Wallace, C. A. ; Robertson, Michael ; Schmidt, K. ; Woolfrey, A. E. ; Nelson, Robert. / Macrophage activation syndrome and other systemic inflammatory conditions after BMT. In: Bone Marrow Transplantation. 2006 ; Vol. 37, No. 7. pp. 629-634.
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