Abstract
Primary leptomeningeal lymphoma (PLML) is a rare disease, comprising less than 1% of all lymphomas. Clinical manifestations include headache, encephalopathy, ataxia, cranial nerve palsy, and myelitis. Diagnosis requires a combination of magnetic resonance images (MRI), cytology, flow cytometry of cerebrospinal fluid (CSF), and an extensive workup to rule out systemic lymphoma.We describe the case of a 49-year-old man who developed subacute onset headache, encephalopathy, and blindness. Whole-body examinations, including a bone marrow trephine biopsy, excluded systemic lymphoma. Brain MRI showed leptomeningeal enhancement. Cytology and flow cytometry of CSF found a clonal B-cell population making a diagnosis of PLML. He began treatment with rituximab and high-dose methotrexate (HD-MTX), with progressive clinical improvement. CSF analysis after two cycles and one intrathecal methotrexate dose was normal.Brain and spinal MRI images plus CSF analysis, along with an extensive workup to exclude systemic lymphoma, are necessary to diagnose PLM. Early treatment with HD-MTX alone or in combination with rituximab improves clinical outcomes.
Highlights
Lachance et al coined the term primary leptomeningeal lymphoma (PML) in 1991 to describe a malignant disease characterized by meningeal infiltration of tumoral lymphoid cells, without cerebral parenchyma or systemic evidence of lymphoma [1]
Diagnosis requires a combination of magnetic resonance images (MRI), cytology, flow cytometry of cerebrospinal fluid (CSF), and an extensive workup to rule out systemic lymphoma
We describe the case of a 49-year-old man who developed subacute onset headache, encephalopathy, and blindness
Summary
Lachance et al coined the term primary leptomeningeal lymphoma (PML) in 1991 to describe a malignant disease characterized by meningeal infiltration of tumoral lymphoid cells, without cerebral parenchyma or systemic evidence of lymphoma [1]. Medical history included a depressive disorder and occasional alcohol consumption He reported episodic night sweats and weight loss of less than 10% of his usual weight. The first brain MRI was considered normal, but the initial cerebrospinal fluid (CSF) sample was abnormal, with lymphocytic pleocytosis, high protein, and hypoglycorrhachia (Table 1, Column A). Flow cytometry found pathological large CD19/CD20 and lambda monoclonal B lymphocytes suggestive of lymphoma (Table 2, Column A).
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