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Clinical Features and Treatment for Neurolymphomatosis in the Rituximab Era: Single Institution Experience

Background: Neurolymphomatosis (NL) is a highly rare disorder defined as infiltration of peripheral nervous system by a lymphoproliferative neoplasm, both presented as the first manifestation of the malignancy (primary NL) or as a site of disease progression or relapse (secondary NL). Due to rarity...

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Bibliographic Details
Published in:Blood 2019-11, Vol.134 (Supplement_1), p.4129-4129
Main Authors: Novo, Mattia, Nowakowski, Grzegorz, Micallef, Ivana, King, Rebecca L., Spinner, Robert, Ristow, Kay M., Habermann, Thomas M., Johnston, Patrick
Format: Article
Language:English
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Summary:Background: Neurolymphomatosis (NL) is a highly rare disorder defined as infiltration of peripheral nervous system by a lymphoproliferative neoplasm, both presented as the first manifestation of the malignancy (primary NL) or as a site of disease progression or relapse (secondary NL). Due to rarity of this disorder data on clinical features, best approach and outcome are limited. Furthermore diagnosis of this disease is often uncertain and based on radiological images only. Methods: We retrospectively reviewed clinical records of all histologically confirmed-NL, diagnosed and treated at the Division of Hematology of Mayo Clinic in Rochester, MN between January 2002 and December 2018. Clinical features and outcome were analyzed. Responses to therapy were assessed by post-treatment radiological changes and clinical symptoms improvement. Survival analysis such as progression free survival (PFS) and overall survival (OS) were estimated using Kaplan Meier curves with log-rank test. Comparison between subgroups was investigated using Fisher's exact test and Wilcoxon test for categorical and continuous variables, respectively. Results: Over a 16-year period 40 patients with biopsy-proven NL were included in study. Primary and secondary NL were 19 (48%) and 21 (52%) respectively, with a median age of 60.5 years (range 37-83) and male sex 24/40 (60%). The predominant histology was represented by B-cell lymphomas (39/40, 97%) with a vast majority large B-cell lymphoma (LBCL) 27/40 (67%) and only 1 case of T-cell lymphoma. The affected structures included brachial plexus (12/40, 35%), lumbosacral plexus (5/40, 12%), cauda equine and nerve roots (14/40, 35%), sciatic nerve (12/40, 30%), femoral nerve (4/40, 10%), cranial nerves (8/40, 20%), multiple nerves involved in 23/40 (57%). Four out of 40 cases had brain involvement and cerebrospinal fluid (CSF) was positive in 7/29 (24%). NL clinical manifestations included muscular weakness (33/4, 82%), sensory deficit (32/40, 80%), pain (28/40, 70%) and autonomic dysfunction (3/40, 7%). Magnetic resonance imaging (MRI) detected neural abnormalities in 35/36 cases (97%), CT imaging identified disease in 3/8 (37%) and FDG-PET scan was positive in 22/31 (71%); electromyography showed some grade of neuropathy in 28/29 (97%). Treatment included systemic chemotherapy in 38/40 (95%) cases, containing high dose methotrexate (HD-MTX) in 26/40 (65%) and rituximab in 20/40 (50%); 11/39 (28%) patients underwent consolidative autologous
ISSN:0006-4971
1528-0020
DOI:10.1182/blood-2019-131378