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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