CENTRAL NERVOUS SYSTEM INVOLVEMENT IN HODGKIN'S LYMPHOMA ASSOCIATED WITH EPSTEIN-BARR VIRUS IN A PATIENT WITHOUT HIV INFECTION IN PREVIOUS HEALTHY CHILDREN

Abstract

Introduction: Hodgkin lymphoma (HL), unlike non-Hodgkin lymphoma, rarely involves the central nervous system (CNS). The incidence has been reported as 0.2% to 0.5% of all HL cases, but a recent review of 14 868 patients identified only 2 cases of HL involving the CNS. Consequently, there is no consensus to guide therapeutic decision-making. Involvement of the brain or spinal cord by HL can be primary or, more frequently, secondary to the compromise of adjacent lymph nodes. Lesions are more frequently intraspinal than intracranial. We describe a patient with systemic HL who presented with involvement of the CNS at diagnosis. Case Report: A six year -old female with a history of progressive cervical lymphadenopathy, presented in October 2023 with isolated cervical lymphadenopathy on the right, in addition to fever, pruritus in the upper limbs and night sweats. Initial pediatric evaluation did not conclude that the cause was infectious. In December 2023 cervical lymphadenomeglia increased in size (2x2cm) but in January 2024 with a progressive increase of 3x2 cm in addition to fever, night sweats and worsening itching, a biopsy was performed which defined as Hodgkin's Lymphoma subtype nodular sclerosis. Admitted to our service in March 2024, in addition to enlarged lymph nodes, fever, night sweats and pain in the lower limbs. After 48 hours of admission to this hospital to start the staging, she developed loss of strength in the left lower limbs. She mainly started Dexamethasone 10mg/m2/day and underwent an MRI. Magnetic core scan of the total spine and brain that showed: Solid neoplastic lesion inside the vertebral and sacral canal, from L4 to S3, involving the roots of the cauda equina, infiltrating the neuroforamens L5-S1, S1-S2 and S2-S3 on the left and compressing the corresponding roots. Solid paravertebral neoplastic lesion on the left, infiltrating the spinal canal at the level of T1-T2 and T6-T7 on the left. Diffuse heterogeneous change in the bone marrow signal of the vertebral bodies, which may represent neoplastic involvement. Solid neoplastic lesions with a dural base, one in the left temporal lobe, measuring 4.3 x 3.8 x 1.7 cm and another in the right frontal lobe, measuring 3.9 x 2.3 x 1.7 cm (CC x TT x AP), showing intense homogeneous post-contrast enhancement and diffusion restriction, the latter associated with vasogenic edema in the frontal white matter on the right. These findings may be related to the spread of the underlying neoplastic lesion. PET-CT with 18F-FDG showed - Metabolism in supra and infradiaphragmatic lymphadenopathy, spleen and bones compatible with active lymphoproliferative disease. Twenty-four hours after starting Dexamethasone, the patient experienced improvement in neurological symptoms, then underwent the 1st cycle of chemotherapy with improvement in neurological and systemic symptoms. She showed an excellent response after two cycles and is currently undergoing chemotherapy with no evidence of disease progression. Conclusion: Finally, unusual and aggressive extranodal presentations of HL should alert to the possibility of HIV infection. Whole brain irradiation and systemic chemotherapy remain the treatment of choice for CNS involvement in patients with HL.