Ataxic gait and hearing loss in a patient with a history of Mantle Cell Lymphoma

Abstract

Objective: Reporting a case of Mantle Cell Lymphoma (MLC) with rapidly progressive envolvement of the central nervous system. Background: Mantle Cell Lymphoma is a B-cell malignancy, which comprises approximately 3–10% of all non-Hodgkin lymphomas with an incidence of approximately 4 to 8 cases per million people per year. It is characterized by the chromosomal translocation t(11;14)(q13;q32), which results in overexpression of the cell cycle protein cyclin D1. MCL arises in older adults (median age of 60–65 years) and has a male and caucasian predominance. Case report: JBJSM, 66 years old, female, caucasian, was referred to Internal Medicine in November 2010 for splenomegaly and thrombocytopenia. There were no complaints of fever, sweat, or loss of weight. Physical examination revealed splenomegaly and cervical lymphadenopathy. Diagnostic tests: Abdominal ultrasound: marked splenomegaly of approximately 20 cm, Upper GI: micropolyp at stomach fundus. TC Cervico Facial, thoraco-abdominal-pelvic: lymphadenopathy involving several chains: facial, axillar, mediastinal, hilar, intra-abdominal, retroperitoneal and inguinal-femoral bilaterally. Adenopathy biopsy and Immunophenotyping and immunohistochemistry studies have shown morphology compatible with replacement by non-Hodgkin lymphoma of low grade. Atypical cells expressing CD20, CD5 and cyclin D1 — histopathological diagnosis of lymphoma Mantle. The patient was treated with RCHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, prednisolone) with relapse 15 months later and was again given RCHOP (6 cycles) — Last cycle in November 2012. In January of 2013, we confirmed total remission with bone biopsy and CT of the body. On February 1, 2013 the patient presented ataxic gait, paraparesis, marked asthenia and polyarthralgia most intense in the lombar spine and pelvis for 2 months and headache and tinnitus for 6 months. Physical examination: alert, ataxic gait with no preferential drop, dysmetria to the right, marked decrease in hearing acuity, cranial nerves normal, with the exception of III bilaterally. Tone maintained in the upper limbs, but hypotonia below. Muscle strenght grade V in the and II in the lower. Sensory level D6-D7. Liver and spleen not palpable. The magnetic resonance shows thickening of both trigeminal nerves, predominantly the right, and complete obliteration of the cisterns of the ganglion Gasser. Pumping of the wall of the left cavernous sinus, envolvement of the pre-chiasmatic optic nerves and the proximal portion of the acoustic nerve was also shown. In the lombar spine there was a mass extending from D12–L1 to L5–S1, circling the terminal segment of the spinal cord with consequent compression. The patient started intrathecal chemotherapy with methotrexate, cytarabine and dexamethasone 3 times per week with improved hearing loss and analytic response. On March 27, 2013, the patient initiated productive cough and asthenia and died on 10 April 2013. Discussion/conclusion: Most patients with MCL present with advanced stage disease. Approximately 75% of patients initially present with lymphadenopathy. Central nervous system involvement is rare (<5% of cases), and is usually associated with a leukemic phase. The course of MCL is moderately aggressive and variable. Median overall survival is three to four years.