Clinicopathological features of neuropathy associated with lymphoma

Abstract

Lymphoma causes various neurological manifestations that might affect any part of the nervous system and occur at any stage of the disease. The peripheral nervous system is one of the major constituents of the neurological involvement of lymphoma. In this study we characterized the clinical, electrophysiological and histopathological features of 32 patients with neuropathy associated with non-Hodgkin's lymphoma that were unrelated to complications resulting from treatment for lymphoma. Nine patients had pathologically-proven neurolymphomatosis with direct invasion of lymphoma cells into the peripheral nervous system. These patients showed lymphomatous cell invasion that was more prominent in the proximal portions of the nerve trunk and that induced demyelination without macrophage invasion and subsequent axonal degeneration in the portion distal from the demyelination site. Six other patients were also considered to have neurolymphomatosis because these patients showed positive signals along the peripheral nerve on fluorodeoxyglucose positron emission tomography imaging. Spontaneous pain can significantly disrupt daily activities, as frequently reported in patients diagnosed with neurolymphomatosis. In contrast, five patients were considered to have paraneoplastic neuropathy because primary peripheral nerve lesions were observed without the invasion of lymphomatous cells, with three patients showing features compatible with chronic inflammatory demyelinating polyneuropathy, one patient showing sensory ganglionopathy, and one patient showing vasculitic neuropathy. Of the other 12 patients, 10 presented with multiple mononeuropathies. These patients showed clinical and electrophysiological features similar to those of neurolymphomatosis rather than paraneoplastic neuropathy. Electrophysiological findings suggestive of demyelination were frequently observed, even in patients with neurolymphomatosis. Eleven of the 32 patients, including five patients with neurolymphomatosis, fulfilled the European Federation of Neurological Societies/Peripheral Nerve Society electrodiagnostic criteria of definite chronic inflammatory demyelinating polyneuropathy. Some of these patients, even those with neurolymphomatosis, responded initially to immunomodulatory treatments, including the administration of intravenous immunoglobulin and steroids. Patients with lymphoma exhibit various neuropathic patterns, but neurolymphomatosis is the major cause of neuropathy. Misdiagnoses of neurolymphomatosis as chronic inflammatory demyelinating polyneuropathy are frequent due to a presence of a demyelinating pattern and the initial response to immunomodulatory treatments. The possibility of the concomitance of lymphoma should be considered in various types of neuropathy, even if the diagnostic criteria of chronic inflammatory demyelinating polyneuropathy are met, particularly in patients complaining of pain.

Neurolymphomatosis: A Rare Cause of Multiple Mononeuropathy

“…MRI reveals enlargement and enhancement of nerves, roots or plexus. FDG-PET is a useful adjunct to the diagnosis and is most sensitive and specific tool currently available for diagnosis of NL [4]- [7]. It may be positive even when all other modalities fail to yield the diagnosis as was in our case [5].…”

Atypical presentation of Non-Hodgkin Lymphoma (NHL): a case report

“…La simmetria delle manifestazioni cliniche, la natura prevalentemente sensitiva e l'interessamento delle fibre di piccolo calibro (assenza di potenziali corticali registrabili dopo stimolo laser) potrebbero deporre per un quadro di polineuropatia sensitiva [3,4]. Tuttavia le neuropatie associate a NHL presentano più frequentemente caratteristiche similari a quelle osservabili nelle neuropatie di natura demielinizzante/infiammatoria (con riduzione delle velocità di conduzione) [5,6], con una minore probabilità di presentare le caratteristiche di una neuropatia assonale sensitiva o una neuronopatia (danno a carico del ganglio sensitivo) [5,6]. L'alternativa di una più estesa diffusione del processo neoplastico (esordio di un coinvolgimento del processo neurolinfomatosico al plesso brachiale [7] o una multineuropatia), scarsamente rilevabile mediante le indagini strumentali effettuate, è un meccanismo fisiopatologico ragionevolmente ipotizzabile.…”

Clinical Profile and Differential Diagnosis of Classical Paraneoplastic Neurologic Syndromes in Lymphomas

“…К другим причинам нейропатий при лим-фомах можно отнести химио-и лучевую терапию, инфекционные заболевания, повышенную вяз-кость крови, вторичный амилоидоз, компрессию нервных стволов [26]. Некоторые авторы выде-ляют следующие классы нейропатий, ассоцииро-ванных с лимфомой: нейролимфоматоз; демие-линизирующая нейропатия, маскирующаяся под болезнь Гийена-Барре (хроническая воспалитель-ная демиелинизирующая нейропатия); сенсорная нейропатия; нейропатия, связанная с развитием васкулита; паранеопротеинемическая нейропатия [27].…”

“…описано 14 пациентов с НХЛ, у которых был выяв-лен нейролимфоматоз. Скрининг на антинейро-нальные антитела был проведен у 7 пациентов: исследованы анти-Hu, анти-Ri, ANNA-3, анти-Yo, анти-PCA2, анти-PCA-Tr, анти-CV2, антиамфифи-зин, антитела к P/Q типу кальциевых каналов, но ни одно из представленных антител выявлено не было [27]. Паранеопластические нейропатии так-же не ассоциированы с онконейрональными анти-телами [27].…”