Isolated Mononeuropathy Multiplex—A Rare Manifestation of Intravascular Large B-Cell Lymphoma

Abstract

Intravascular large B-cell lymphoma, also known as angiotrophic large cell lymphoma, is a rare disorder where neoplastic lymphoid cells proliferate within the walls of small- to medium-sized blood vessels. Peripheral neuropathy and other neurological manifestations, including stroke and dementia, are common, but cases of isolated multiple mononeuropathies in the absence of systemic symptoms are distinctly rare. We present an unusual case of biopsy-proved angiotrophic large cell lymphoma presenting exclusively with multiple mononeuropathies.

Peripheral nervous system involvement in lymphomas

“…Other manifestations include sensory and motor deficits or neuropathies, meningoradiculitis, paraesthesia, hyposthenia, aphasia, dysarthria, hemiparesis, seizures, myoclonus, transient visual loss, vertigo, and altered conscious state . Peripheral nervous system manifestations have been described with a wide spectrum of clinical pictures from acute polyradiculoneuropathy to multiple mononeuropathy.…”

“…43 7 | INTRAVASCULAR LYMPHOMA Intravascular large cell lymphoma, formerly known as angiotropic large cell lymphoma or malignant angioendotheliomatosis, 44 is a rare subtype of aggressive large cell lymphoma characterized by invasion of lymphoma of the blood vessel lumen. 45 46 Peripheral nervous system manifestations have been described with a wide spectrum of clinical pictures from acute polyradiculoneuropathy 47 to multiple mononeuropathy 48 .…”

Demyelinating Neuropathy Due to Intravascular Large B-cell Lymphoma

“…Acute sensorimotor neuropathy, such as Guillain-Barré syndrome, may occur as a paraneoplastic syndrome usually in association with lymphoma (10). Peripheral neuropathy due to IVLBCL is usually believed to be multiple mononeuropathies with axonal degeneration due to ischemia, resulting from intravascular occlusion of small blood vessels by lymphoma cells (11). However, the sural nerve biopsy findings in our case indicated the presence of a demyelinating process that was unrelated to ischemic damage (8).…”

Clinical Neuropathology practice guide 3-2014: Combined nerve and muscle biopsy in the diagnostic workup of neuropathy – the Bordeaux experience

“…Unpredicted diagnosis of intra-vascular lymphoma may be established when numerous malignant B lymphocytes are visible within the lumen of several small vessels on nerve and muscle specimens (Figure 2H, I) [12, 51, 52]. Other lymphomas may infiltrate peripheral nerve [53, 54], but infiltration of muscle is occasional [55].…”