Intravascular large B-cell lymphoma as a mimic of primary vasculitis of the central nervous system

Juan S. Saavedra-Moreno, Sección de Neurología, Universidad de Antioquia/Hospital San Vicente Fundación, Medellín, Colombia
Duvan A. Jiménez-Trespalacios, Programa de Residencia en Neurología, Universidad de Antioquia, Medellín, Colombia
Andrea C. Ibáñez-Acosta, Programa de Residencia en Neurología, Universidad de Antioquia, Medellín, Colombia
Valentina Serna-Loaiza, Programa de Residencia en Neurología, Universidad de Antioquia, Medellín, Colombia
Lisseth Triviño-Charry, Programa de Residencia en Neurología, Universidad de Antioquia, Medellín, Colombia
Reydmar López, Hospital San Vicente Fundación/Universidad de Antioquia, Medellín, Colombia
Gabriel J. Varela-Aguirre, Departamento de Patología, Hospital Pablo Tobón Uribe/Hospital San Vicente Fundación, Rionegro; Facultad de Medicina, Universidad Pontificia Bolivariana, Medellín. Colombia

Intravascular lymphoma (IVL) is a rare variant of non-Hodgkin lymphoma characterized by the proliferation of neoplastic lymphoid cells within small- and medium-caliber blood vessels.This unusual localization makes it a “great imitator”, presenting with nonspecific signs and symptoms that vary depending on the affected organ. Systemic and cutaneous manifestations are not always present, and due to its aggressive, heterogeneous, and infrequent nature, diagnosis is often delayed or made post mortem. We present the case of a patient with IVL isolated to the central nervous system, who presented with multiple cerebrovascular events leading to rapidly progressive dementia, ataxia, and sensorineural hearing loss —the latter not previously reported in the literature. In patients with progressive, multifocal neurological syndromes and imaging findings suggestive of vasculitis, IVL should be considered in the differential diagnosis. Histopathological confirmation is essential for timely diagnosis and to guide appropriate treatment.

Keywords: Intravascular lymphoma. Diffuse large B cell lymphoma. Central nervous system. Primary vasculitis of the central nervous system. Case report.