PMID: 15239401Jul 9, 2004Paper

Fulminant hemophagocytic syndrome with a high interferon gamma level diagnosed as macrophage activation syndrome

International Journal of Hematology
Tsuyoshi Muta, Yujiro Yamano

Abstract

A 26-year-old woman presented with general fatigue, persistent fever, nuchal lymphadenitis, thrombocytopenia, and liver damage. From the bone marrow finding, we diagnosed her condition as hemophagocytic syndrome. Steroid pulse therapy, cyclosporin A treatment, and combined chemotherapy generated no response. The patient showed severe mucosal bleeding, rapidly experienced multiple organ failure, and finally died of a brain hemorrhage on the 13th hospital day. Epstein-Barr virus, cytomegalovirus, human herpes virus type 6, human parvovirus B19, and herpes simplex virus were not detected. Autopsied samples of the spleen, bone marrow, and liver showed extreme proliferation of activated macrophages, so-called histiocytes, without lymphoid malignancy. The interferon gamma level at presentation was prominently high. The continuously elevated levels of ferritin and soluble interleukin 2 receptor were correlated with the catastrophic outcome. The disease in our case mimicked infantile hemophagocytic lymphohistiocytosis. However, there was neither a family history of the disease nor a mutation in the perforin gene. So, it is reasonable to categorize our case as macrophage activation syndrome. Although our patient lacked arthritis or erup...Continue Reading

References

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Apr 29, 2003·British Journal of Haematology·Ikuyo UedaShinsaku Imashuku

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Citations

May 12, 2010·Journal of Microbiology, Immunology, and Infection = Wei Mian Yu Gan Ran Za Zhi·Erol ErduranMehmet Mutlu
Dec 27, 2005·Clinics in Liver Disease·John BucuvalasRobert H Squires
Jan 15, 2008·International Journal of Hematology·Anastasios AndreopoulosGeorge Vaiopoulos

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