Background:
Intravascular lymphoma (IVL) is a rare subtype of non-Hodgkin lymphoma with exclusively or predominantly intravascular proliferation. Without therapeutic intervention, the neurologic involvement is rapidly progressive and inevitably fatal. Most of the IVL patients have prominent or exclusive manifestations in the nervous system and there are several reports of patients presenting with spinal symptoms.
Case description:
A 68-year-old male patient admitted with the complaints of progressive paraparesis. T2-weighted magnetic resonance imaging (MRI) of the spinal cord showed hyperintense lesions in the thoracic cord. A diagnosis of myelitis of unknown etiology was assumed, and steroid pulse therapy was administered, which temporarily improved the patient's symptoms. However, the paraparesis recurred, and other symptoms, such as vertigo, psychosis, and seizures, developed 1-month after the initial treatment. Multiple high-intensity lesions were detected in the bilateral subcortical white matter on DW MRI. Based on the patient's clinical course, IVL was suspected; however, obtaining histological confirmation was not possible, as no Gd-enhanced brain or spinal lesions were identified and repeated cerebrospinal fluid examinations were negative for tumor cells. Therefore, a random skin biopsy was performed, and IVL was diagnosed. Obtaining a comparatively favorable outcome was possible owing to the subsequent administration of R-CHOP chemotherapy.
Conclusion:
IVL should be included in the differential diagnosis of atypical case of presumed myelitis. An early diagnosis and chemotherapy is crucial for improving the patient's outcome. When obtaining a diagnosis based on tissues other than skin is difficult, a random skin biopsy should be considered in patients with suspected IVL.