Neurolymphomatosis

Pavan Tummala MD MS (Dr. Tummala of Saint Louis University has no relevant financial relationships to disclose.)
Niranjan N Singh MD (Dr. Singh of the University of Missouri - Columbia has no relevant financial relationships to disclose.)
Florian P Thomas MD MA PhD MS (Dr. Thomas, Chair, Department of Neurology, Seton Hall-Hackensack Meridian School of Medicine, has no relevant financial relationships to disclose.)
Louis H Weimer MD, editor. (Dr. Weimer of Columbia University has received consulting fees from Roche.)
Originally released January 22, 2002; last updated October 31, 2016; expires October 31, 2019

This article includes discussion of neurolymphomatosis, Marek disease, peripheral lymphomatosis, and reticulosis of nervous system. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Historical note and terminology

The term neurolymphomatosis is used to describe peripheral nervous system or spinal root disease resulting from infiltration by malignant lymphoma cells (Diaz-Arrastia et al 1992; Jellinger and Grisold 2002). This phenomenon was originally recognized by Marek, who observed a neuropathy in 4 chickens and found demyelination and lymphomatous nerve infiltrates with sparing of internal organs in 1 autopsy (Marek 1907). Now referred to as Marek disease, it is recognized as a T-cell lymphoma in fowl associated with a Herpes virus (Lee et al 1969). Pappenheimer and colleagues attempted to reproduce this condition in experimental chickens in order to identify its etiology; they coined the term “neurolymphomatosis gallinarum” (Pappenheimer et al 1929). Association with leukemia was described in a report of a young man who died 1 month after presenting with sensorimotor polyneuropathy, anemia, and splenomegaly; autopsy showed leukemic involvement of nerves, liver, kidney, and spleen (Harris 1922). The term "peripheral lymphomatosis" was proposed by Lhermitte and Trelles for the case of 67-year-old woman with malignant neuropathy and lymphoid nerve infiltrates but no systemic or brain involvement (Lhermitte and Trelles 1934).

A histologically similar case of multiple relapsing-progressive mononeuropathies was described by Garcin and colleagues (Garcin et al 1953). They report that an autopsy 7 months after onset demonstrated generalized nerve enlargement and diffuse lymphomatous infiltration of essentially all nerves, dural spaces, and central nervous system with sparing of internal organs. Radnai and Takacs Nagy described a 36-year-old woman with multiple mononeuropathies and lymphomatous involvement of the brain and peripheral nervous system but not the spinal cord, and of internal organs but not lymphoid organs (Radnai and Takacs Nagy 1952).

Allison and Gordon used the term "reticulosis of nervous system" for the case of a 56-year-old woman with progressive sensorimotor peripheral and cranial neuropathies, characterized by lymphomatous nerve infiltrates (Allison and Gordon 1955). Barron and colleagues described a case associated with Hodgkin disease (Barron et al 1960). Gherardi and colleagues presented 2 patients with T cell lymphomatous infiltrates (Gherardi et al 1986). Diaz-Arrastia and colleagues presented a literature review dedicated to neurolymphomatosis (Diaz-Arrastia et al 1992). Vital and colleagues detected retrovirus-like particles consistent with HTLV-1 on electron microscopy of peripheral nerves with T-cell lymphomatous infiltrates (Vital et al 1993). Abad and colleagues described 4 cases of neurolymphomatosis with malignant infiltration in Waldenstrom macroglobulinemia (Abad et al 1999).

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