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Primary malignant lymphoma of the CNS and polyneuropathy in a patient with necrotizing vasculitis treated with immunosuppression

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Summary

A woman, aged 58, who had undergone prolonged treatment with corticosteroids for generalized necrotizing vasculitis, and had received azathioprine (Imurel) for 18 months, 4 years after discontinuing the latter treatment, developed peripheral neuropathy and a rapidly progressing cerebral disorder suggesting a basal meningeal process. CSF cytology suggested malignant lymphoma with meningeal involvement. Immunological studies showed an increase of Null-lymphocytes in the peripheral blood. Autopsy disclosed a primary malignant lymphoma of the CNS with the histological appearance of a multilocular immunoblastoma showing almost ubiquitous meningocerebral involvement. Clinical and postmortem examinations failed to demonstrate any systemic extraneural lymphoproliferative disorder. In addition, there was peripheral polyneuropathy of the axonal type with denervation atrophy of skeletal muscle, but without lymphomatous involvement of the neuromuscular system. The possible relations between primary malignant lymphomas of the CNS and previous immunosuppressive treatment of immunoinflammatory disease are discussed in view of the concept of impaired immunoregulation. The pathogenetic background of peripheral polyneuropathy is unknown.

Zusammenfassung

Eine 58jährige Frau, die wegen generalisierter Purpura necroticans (nekrotisierender Vaskulitis) eine Langzeitbehandlung mit Kortikosteroiden sowie durch 18 Monate hindurch Azathioprine (Imurel) erhalten hatte, entwickelte 4 Jahre nach Absetzen der Azathioprinbehandlung eine periphere Polyneuropathie sowie ein rasch progredientes neurologisches Syndrom im Sinne eines basalen Meningealprozesses. Die Liquorcytologie ergab ein malignes Lymphom mit Meningealbefall. Immunologische Untersuchungen ergaben im peripheren Blut eine Zunahme der Null-Zellpopulation. Autoptisch fand sich ein primäres malignes Lymphom des ZNS vom histologischen Aufbau eines diffusen multilokulären Immunoblastoms mit nahezu ubiquitärer meningozerebraler Ausbreitung. Klinisch und autoptisch ergaben sich keine Hinweise auf einen generalisierten lymphoproliferativen Prozeß. Daneben bestand eine periphere Polyneuropathie vom axonalen Typ mit Denervationsatrophie der Skelettmuskulatur, aber ohne Lymphombefall der neuromuskulären Peripherie. Die möglichen Beziehungen zwischen primärem malignem Lymphom des ZNS und vorangegangener Immunsuppressivbehandlung eines Autoimmunprozesses werden im Hinblick auf das Konzept der gestörten Immunregulation diskutiert. Die Pathogenese der Polyneuropathie ist ungeklärt.

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Authors and Affiliations

  1. Ludwig Boltzmann Institute of Clinical Neurobiology and Department of Neurology, Lainz Hospital, Vienna, Austria

    K. Jellinger, P. Kothbauer, R. Weiss & E. Sunder-Plassmann

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  1. K. Jellinger
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  2. P. Kothbauer
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  3. R. Weiss
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  4. E. Sunder-Plassmann
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Jellinger, K., Kothbauer, P., Weiss, R. et al. Primary malignant lymphoma of the CNS and polyneuropathy in a patient with necrotizing vasculitis treated with immunosuppression. J. Neurol. 220, 259–268 (1979). https://doi.org/10.1007/BF00314150

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