title: Macrophage activation syndrome and other systemic inflammatory conditions after BMT.
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 glucocorticosteroid 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.
authors.affiliation: Department of Medicine, Indiana University School of Medicine, Indianapolis, IN 46202, USA.
authors.forename: A
authors.initials: A
authors.lastname: Sreedharan
authors.forename: S
authors.initials: S
authors.lastname: Bowyer
authors.forename: C A
authors.initials: CA
authors.lastname: Wallace
authors.forename: M J
authors.initials: MJ
authors.lastname: Robertson
authors.forename: K
authors.initials: K
authors.lastname: Schmidt
authors.forename: A E
authors.initials: AE
authors.lastname: Woolfrey
authors.forename: R P
authors.initials: RP
authors.lastname: Nelson
mesh_terms: D000328:Adult; D001171:Arthritis, Juvenile / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D001327:Autoimmune Diseases / Q000209:etiology / Q000276:immunology* / Q000628:therapy; D018380:Hematopoietic Stem Cell Transplantation / Q000009:adverse effects*; D006801:Humans; D007249:Inflammation / Q000276:immunology; D008262:Macrophage Activation / Q000276:immunology*; D008264:Macrophages / Q000276:immunology*; D008297:Male; D012074:Remission Induction; D013577:Syndrome