Abstract:
Epstein-Barr virus-associated B-cell lymphoproliferative disorders (EBV-LPDs) are a heterogeneous spectrum of hematologic disorders. Defects in immune surveillance are linked to pathogenesis of EBV-LPD. Lymphomatoid granulomatosis (LYG) is a unique, extra-nodal EBV-LPD that can involve organs like the lungs, central nervous system (CNS), skin, kidneys and liver. Primary CNS-LYG is extremely rare and can masquerade as primary brain tumors like glioblastomas. We describe a challenging case of a 59-year-old gentleman with isolated primary CNS-LYG and a discordant low-grade B-cell lymphoma involving the bone marrow causing chronic immune thrombocytopenia. Clinical course was complicated with disseminated zoster and sub-massive pulmonary embolism. There are no established standards-of-care in managing LYG. Treatment strategies that have been used for LYG include expectant observation, surgical resection, corticosteroids, radiation-, chemo- and immuno-therapies. Our patient was successfully managed with a combination radioimmunotherapy strategy with rituximab and ultra-low-dose radiation therapy (ULD-RT) of 2 Gy x 2. The biologic rationale for synergy with this combination, published literature and future directions in managing EBV-LPDs and LYG are discussed.
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