A 32-year-old woman in the 16th week of pregnancy was admitted to our hospital because of high
fever. Laboratory findings disclosed
pancytopenia and extremely elevated serum LDH and
ferritin levels. Coagulation tests showed
disseminated intravascular coagulation. Serum soluble
interleukin-2 receptor,
tumor necrosis factor-alpha, and
interleukin-6 levels were high, but serum
interferon-gamma was below the detectable limit. Reactive Epstein-Barr virus (
EBV) infection was diagnosed on the basis of a high titer of
IgG antibodies to the
EBV capsid antigen and early
antigen. EBV was demonstrated in the peripheral blood and bone marrow cells by polymerase chain reaction. Mature
histiocytosis and hemophagocytosis were detected in the bone marrow. A diagnosis of EBV-associated
hemophagocytic syndrome (EBV-AHS) was made. Neither
prednisolone (PSL 30 mg/day, P.O.) nor
methylprednisolone (m-PSL) pulse
therapy (1,000 mg/day for 3 days) induced a response. Thereafter, treatment with m-PSL pulse
therapy (1,000 mg/day for 3 days) and i.v. administrations of high-dose
immunoglobulin (20 g/day for 3 days) in combination with
acyclovir (750 mg/day) and
gabexate mesilate (2 g/day) induced remission of the disease. Maintenance
therapy consisted of PSL (5 mg/day, P.O.) and
camostat mesilate (600 mg/day, P.O.). The patient delivered a healthy male infant in the 35th week of pregnancy via natural birth. Reports of pregnant women with EBV-AHS are rare, and the choice of
therapy has not yet been established. The present case study suggested the above combination treatment is useful and safe, and capable of changing the fulminant course of EBV-AHS during pregnancy without the use of anticancer drugs.