Macrophage activation syndrome: serological markers and treatment with anti-thymocyte globulin.
Two patients presented at the University of Rochester Medical Center with a febrile illness, cytopenias, organ failure (liver failure or respiratory failure), and markedly elevated serum ferritin and sIL-2R. A diagnosis of probable macrophage activation syndrome was made. Both patients failed initial therapy with steroids and cyclosporine, either due to toxicity or lack of efficacy. Both patients responded dramatically to rabbit anti-thymocyte globulin (ATG).
Coca
Andreea
A
University of Rochester, Department of Medicine, Allergy Immunology Rheumatology Division, Room G-6427C, 601 Elmwood Avenue, Rochester, New York 14642, USA.
Bundy
Kemp W
KW
Marston
Bethany
B
Huggins
Jennifer
J
Looney
R John
RJ
D015703:Antigens, CD / Q000097:blood; D015214:Antigens, Differentiation, Myelomonocytic / Q000097:blood; D000961:Antilymphocyte Serum / Q000627:therapeutic use*; D015415:Biomarkers / Q000097:blood*; D005260:Female; D005293:Ferritins / Q000097:blood; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D055501:Macrophage Activation Syndrome / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy*; D011956:Receptors, Cell Surface / Q000097:blood; D015375:Receptors, Interleukin-2 / Q000097:blood; D055815:Young Adult; D000098902:CD163 Antigen
D015703:Antigens, CD; D015214:Antigens, Differentiation, Myelomonocytic; D000961:Antilymphocyte Serum; D015415:Biomarkers; D000098902:CD163 Antigen; D007166:Immunosuppressive Agents; D011956:Receptors, Cell Surface; D015375:Receptors, Interleukin-2; D005293:Ferritins