INFECTION-ASSOCIATED HEMOPHAGOCYTIC SYNDROME COMPLICATED BY INFECTIOUS LYMPHOPROLIFERATION - A CASE-REPORT

The case of a 7-year-old boy with virus-associated hemophagocytic synd rome (VAHS) and serologically proven parvovirus B-19 infection is desc ribed. The patient with VAHS presented with fever, hepatosplenomegaly, pancytopenia, and hyperlipidemia type IV. After induction therapy wit h VP-16 and prednisone, partial remission was achieved. Despite mainte nance therapy, reinductions, and the addition of cyclosporine A for 3 months, several relapses occurred. The therapy was stopped because of life-threatening complications (Klebsiella sepsis, neutropenic enteroc olitis, and stercoral peritonitis). The complication were treated succ essfully. The patient status was stabilized after splenectomy. However , hepatomegaly progressed slowly and the hyperlipidemia endured. Ten m onths after the diagnosis, leukocytosis with absolute T lymphocytosis appeared. Reactivation of VAHS was suspected and intravenous immunoglo bin and then antilymphocyte immunoglobulin ALG therapy were started. T he resultant decrease in leukocytosis was prompt, but lymphopenia did not occur. Virostatic treatment with foscarnet was introduced based on human herpesvirus-6 seroconversion. Twenty-six months after the diagn osis,the patient is well, without any sign of VAHS or lymphoproliferat ion.