A 66-year-old man was admitted to our hospital for
fever on January 19, 1998. He began showing periodic high
fever in June 1997 and an increased serum LDH in August 1997. His history included surgery for
esophageal cancer in 1993. On admission, the patient's body temperature was 38.5 degrees C. Physical examination was negative for
lymphadenopathy, hepatosplenomegaly, and
skin rash. Peripheral blood revealed a
hemoglobin level of 8.6 g/dl and a platelet count of 7.9 x 10(4)/microliter. Bone marrow examination showed hypocellularity with marked histiocytic hemophagocytosis. The various bacterial cultures were negative. Serum LDH was elevated to 1,606 IU/l, and
ferritin was greater than 3,000 ng/ml.
Antinuclear antibodies were negative. No significant elevation of viral antibody titers including that to Epstein-Barr virus was found.
Hemophagocytic syndrome (HPS) was diagnosed, but no underlying diseases was identified. The patient's condition was complicated by
interstitial pneumonia and
pleural effusion.
gamma-globulin and pulse
methylprednisolone both proved ineffective for the HPS; however, complete remission was achieved with cyclic
intravenous administration of
etoposide (
VP-16, 150 mg/day). Interestingly, the
interstitial pneumonia resolved promptly with
etoposide therapy. The patient relapsed, in July 2001, exhibiting high
fever,
cytopenia, and marrow hemophagocytosis. His condition was ameliorated by administration of
etoposide. This was a rare case of chronic and recurrent HPS of unknown etiology accompanied by
interstitial pneumonia.
Etoposide should be considered as a primary
therapy for HPS and its complications in cases such as our patients.