Plasma Cell Myeloma Associated with Epstein-Barr Virus Infection in an 11-Year-Old Girl

Abstract

Multiple myeloma is extremely rare in children and represents fewer than 1% of all patients with myeloma. We report a case of multiple myeloma in an 11-year-old girl, who presented with a well-differentiated immunoglobulin A/kappa plasmacytoma at the base of the skull at 9 years of age; at that time, the bone marrow biopsy was negative. Two years later, the patient experienced generalized bone pain with multiple lytic bone lesions that affected the skull, long bones, ribs, and clavicle. The bone marrow biopsy showed a well-differentiated (Marschalko-type) multiple myeloma that was positive for CD138 and immunoglobulin A, with kappa light chain restriction. Interestingly, the Epstein-Barr virus (EBV) was detected by in situ hybridization for EBV-encoded RNA (EBER) in the majority of the neoplastic cells from both biopsy specimens. The patient responded favorably to treatment with dexamethasone, thalidomide, and zoledronic acid and is scheduled for bone marrow transplantation.