A Case of Acute Lupus Hemophagocytic Syndrome: A Case Report / 대한류마티스학회지

ABSTRACT

A 45-year-old woman was admitted to our hospital because of a high fever, dyspnea, and myalgia. At the time of admission, a diagnosis of systemic lupus erythematosus(SLE) was made by fulfilling four of the 1982 American College of Rheumatology criteria with increasing levels of anti-nuclear antibody titer(speckled pattern). Prednisolone given orally in an initial dosage of 30mg/day was not effective and she was expired by respiratory failure due to disseminated intravascular coagulation. A diagnosis of hemophagocytic syndrome was made because of the increased number of unusual hemophagocytic cells in the bone marrow. High levels of serum ferritin which are known to reflect macrophage activition, supported the diagnosis of hemophagocytic syndrome. Hemophagocytic syndrome is characterized by activated phagocytosis presumably induced by hypersecretion of cytokines. Malignant lymphoma and infection are the two representative diseases which may cause hemophagocytic syndrome. Recently several acute lupus hemophagocytic syndromes were reported in patients with SLE. Here we report a case of acute lupus hemophagocytic syndrome observed in a patient with SLE with brief review of literatures.