Rafael Bragança Rodrigues Matias
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Bruna Cardoso de Mattos Boccalini
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Renata de Oliveira Costa
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Maria Fernanda Mélega Mingossi
Introduction: Mantle cell lymphoma (MCL) is a subtype of uncommon nonHodgkin lymphoma. The involvement of the central nervous system (CNS) is uncommon in the course of the disease. Objective: To report a case of recurrence of MCL in the CNS as the first manifestation, after chemotherapy and bone marrow transplantation. Case report: Male patient, 49 years old, with no previous comorbidities diagnosed with stage IV MCL (bone marrow), submitted to chemotherapy and autologous transplantation. After two years, he sought out the neurology clinic with a complaint of blurred vision. Neurological examination: without motor deficit; bilateral partial ptosis, bilateral divergent strabismus, tongue shift to the right. CSF with 230 leukocytes/mm³, 70% of lymphocytes, glucose of 71 mg /dl and protein of 85 mg /dl; Skull MRI demonstrated bilateral and symmetrical enhancement of segments of the cisterns of the optic and oculomotor nerves; Trigeminal, facial, vestibulocochlear and glossopharyngeal, vagus and accessory nerves more exuberant on the left. CSF immunophenotyping showed CD19, CD5 and Kappa positive monoclonal, compatible with MCL recurrence. Intrathecal and systemic chemotherapy with methotrexate were initiated. Discussion: Risk of recurrence of MCL and infiltration of the CNS is uncommon (3.9 - 5%). The patient did not show any signs of systemic involvement, only the neurological findings, which is atypical since the neurological presentation is more associated with recurrence of MCL with a course of systemic findings. Conclusion:The authors point out that in patients with treated MCL who have neurological manifestations without systemic findings, tumor recurrence should be considered.
Farber's disease without central nervous system involvement: bone-marrow transplantation provides a promising new approach
