Abstract Details

Title Unusual Presentation of Mantle Cell Lymphoma Revealed by Central Nervous System Involvement: A Case Report

Topic Cerebrovascular Disease and Interventional Neurology

Presentation(s) S22 - (-)

Poster/Presentation
Number 005

Objective

Background CNS involvement in Non-Hodgkin Lymphoma (NHL) is not uncommon, can either develop at the beginning of the disease or at relapse and is exceptionally the only symptom leading to the diagnosis. Also, CNS involvement can correspond to leptomeningeal infiltration or brain metastases and MRI show cerebral hemisphere, basal ganglia or periventricular lesions similar to those observed in Primary CNS Lymphoma, with homogeneous contrast enhancement.

Design/Methods The patient presented with cognitive impairment, ataxia, weight loss and back pain. First, brain and spine MRI showed T2 and FLAIR-weighted hyper-intensity in frontal cortex, basal ganglia, brainstem and spinal cord with heterogeneous linear and punctiform contrast enhancement, which could have been compatible with the diagnosis of Clippers syndrom or neurosarcoidosis. In addition, the lumbar puncture revealed a high level of Cerebro-Spinal Fluid (CSF) protein with 14 lymphocytes. Also, note that neither peripheral lymph node nor neoplasm was found on the Chest/Abdomen/Pelvis Computed Tomography. Finally, flow cytometry and Polymerase Chain Reaction (PCR) for clonal immunoglobulin gene rearrangements of CSF and plasmatic lymphocytes revealed CD5+monotypal B lymphocytes with t(11;14) translocation which was highly suggestive of MCL.

Results Moreover, the patient partially improved with steroids as the MRI lesions and contrast enhancement also regressed but relapsed several months later while steroids were decreased.

Conclusions Multiple cerebral, spinal and leptomeningeal T2-weighted hyper-intense images on MRI with linear and punctiform perivascular contrast enhancement can be features of Mantle Cell Lymphoma and should lead up to perform specific CSF studies (flow cytometry and PCR) because MRI features may be misleading. Furthermore, CNS involvement in MCL can be isolated, responsible for misdiagnosis.

Authors/Disclosures
Geraldine Faivre, MD (The Queens' Medical Center- West O'ahu) PRESENTER	Dr. Faivre has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
	No disclosure on file
Jean-Yves Delattre, MD (Hopital de la Salpetriene)	No disclosure on file
Catherine Lubetzki (Hopital De La Salpetriere)	Catherine Lubetzki has received personal compensation in the range of $500-$4,999 for serving as a Consultant for ROCHE. Catherine Lubetzki has received personal compensation in the range of $500-$4,999 for serving as a Consultant for MERCK SERONO. Catherine Lubetzki has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for REWIND.
Edgardo Cristiano, MD (Hospital Italiano De Buenos Aires)	Dr. Cristiano has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Cristiano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Cristiano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Genzyme. Dr. Cristiano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Cristiano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Merck.

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