A Rare Case of Peripheral Neurolymphomatosis in Cutaneous T-cell Lymphoma (P1.9-040)

Objective: NA Background: Cutaneous T cell Lymphoma (CTCL) rarely involves the nervous system. In a retrospective study, 3% cases were found to have neurological complications , half of them being due to direct involvement. Neurolymphomatosis (lymphoma involving peripheral nerve) has been very rarely described for T-cell lymphomas. Design/Methods: Case report Results: Case: A 66 year old gentleman with h/o diabetes mellitus presented to the neurology clinic with c/o bilateral hand paresthesia and weakness progressing over several weeks. His past medical history was significant for cutaneous T-cell lymphoma diagnosed in 1999 followed by multimodality treatment including topical therapies, chemotherapy and palliative radiation therapy. Neurological examination was suggestive of right median neuropathy at wrist and proximal left ulnar neuropathy and these were confirmed by NCS/EMG. MRI showed evidence of heterogeneous nodular enhancement of the left ulnar nerve and right median nerve. CT/PET scan showed increased FDG (flurodeoxyglucose) activity corresponding to the areas of enhancement of the median nerve. With strong suspicion for neurolymphomatosis, the patient underwent a fascicular biopsy of the median nerve. Histopathology showed severely distorted architecture of nerve tissue with CD-3 and CD-8 positive T cell infiltrating neural tissue and blood vessel wall. The patient was treated with radiation and chemotherapy. Unfortunately, the disease progressed and the patient died within two months. Conclusions: Our case adds to the rarely documented neurological complication of CTCL. Clinical courses varying from recovery to fatality have been seen in the very few reported cases of neurolymphomatosis due to CTCL. Diagnosis can be challenging but one would need to distinguish peripheral neuropathy due to direct lymphocytic infiltration, from autoimmune or paraneoplastic processes as treatments would vary. MRI, PET scan and finally targeted nerve biopsy can help in the diagnosis as in our case. Although rare, neurolymphomatosis should be considered in the differential for acute onset peripheral neuropathy in the setting CTCL. Disclosure: Dr. Paul has nothing to disclose. Dr. Engelstad has nothing to disclose. Dr. Spinner has nothing to disclose. Dr. Pupillo has nothing to disclose. Dr. Dyck has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Ionis Pharmaceuticals.