Abstract
We present a case of successful bone marrow transplantation (BMT) in a 15-year-old girl with T-cell acute lymphocytic leukemia (T-ALL) after treatment for hemophagocytic syndrome (HPS). The patient presented with progressive fever, pancytopenia and erythema after reinduction therapy. A bone marrow biopsy specimen demonstrated intense hemophagocytosis and no lymphoblasts. Dexamethasone and VP-16 were initiated, and symptoms of HPS resolved. Two weeks later, however, fever and pancytopenia relapsed, followed by disseminated vesicles of the skin. A biopsy specimen taken from the skin and vesicles showed varicella-zoster virus (VZV) infection, even though the patient had a past history of chickenpox at four years of age. HPS improved after antiviral therapy with acyclovir, but recurred with neurological manifestations after cessation of antiviral therapy. Computed tomography and magnetic resonance imaging of the brain indicated multiple lesions in the central nervous system. HPS was well-controlled after initiation of anti-HPS therapy with aciclovir and anti-VZV high titer immunoglobulin. Allogeneic BMT was performed with pretreatment without irradiation to the brain after improvement of neurological symptoms. The patient remains in complete remission 84 months following BMT. Hemophagocytic syndrome is an uncommon condition during chemotherapy and the underlying cause is sometimes difficult to determine. The possibility of VZV infection should be considered in immunosuppressed HPS patients, even in those lacking characteristic skin lesions. This condition is often misdiagnosed because clinicians are unfamiliar with it. Magnetic resonance imaging of the brain appears helpful in diagnosing central nervous involvement with HPS.
