We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up-regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities with hypofibrinogenemia, and rapidly evolving pancytopenia should alert the clinician to the possible diagnosis of macrophage activation syndrome, even in autoinflammatory diseases characterized basically by the periodic recurrence of unprovoked inflammatory attacks. Bone marrow aspiration showing well-differentiated macrophages phagocytosing hematopoietic elements remains the main tool for a final diagnosis, and cyclosporine is the best strategy for treatment.

Rigante, D., Capoluongo, E. D., Bertoni, B., Ansuini, V., Chiaretti, A., Piastra, M., Pulitano', S. M. M., Genovese, O., Compagnone, A., Stabile, A., First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome, <<ARTHRITIS AND RHEUMATISM>>, 2007; 2007 (56(2)): 658-661 [http://hdl.handle.net/10807/3667]

First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome

Rigante, Donato;Capoluongo, Ettore Domenico;Ansuini, Valentina;Chiaretti, Antonio;Piastra, Marco;Pulitano', Silvia Maria Modesta;Genovese, Orazio;Stabile, Achille
2007

Abstract

We describe for the first time a case of macrophage activation syndrome in a child with hyperimmunoglobulinemia D with periodic fever syndrome who required intensive care support. Up-regulated monokine production, high serum levels of triglycerides and ferritin, clotting abnormalities with hypofibrinogenemia, and rapidly evolving pancytopenia should alert the clinician to the possible diagnosis of macrophage activation syndrome, even in autoinflammatory diseases characterized basically by the periodic recurrence of unprovoked inflammatory attacks. Bone marrow aspiration showing well-differentiated macrophages phagocytosing hematopoietic elements remains the main tool for a final diagnosis, and cyclosporine is the best strategy for treatment.
Inglese
Rigante, D., Capoluongo, E. D., Bertoni, B., Ansuini, V., Chiaretti, A., Piastra, M., Pulitano', S. M. M., Genovese, O., Compagnone, A., Stabile, A., First report of macrophage activation syndrome in hyperimmunoglobulinemia D with periodic fever syndrome, <<ARTHRITIS AND RHEUMATISM>>, 2007; 2007 (56(2)): 658-661 [http://hdl.handle.net/10807/3667]
File in questo prodotto:
Non ci sono file associati a questo prodotto.

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: http://hdl.handle.net/10807/3667
Citazioni
  • ???jsp.display-item.citation.pmc??? ND
  • Scopus 56
  • ???jsp.display-item.citation.isi??? 55
social impact