Primary Central Nervous System Lymphoma with Waldenström’s Macroglobulinaemia

Noël, Julie; Gille, M.; Garbar, Christian; Martens, Michel; Verhulst, D; Vandeput, Y.

doi:10.1159/000047980

Eur Neurol

2002

DOI: 10.1159/000047980

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Primary Central Nervous System Lymphoma with Waldenström’s Macroglobulinaemia

Julie Noël

M. Gille²,

Christian Garbar³

et al.

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Cited by 4 publications

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“…However, involvement of the peripheral nervous system due to leptomeningeal infiltration by neoplastic cells is very rare and has been reported in few cases only [1,[4][5][6]. Remarkably, in contrast to other non-Hodgkin's lymphomas where meningeal involvement develops in the setting of progressive disease, in our patient meningeal infiltration occurred in a stable partial remission phase of the WM.…”

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confidence: 56%

Cranial and peripheral neuropathy due to leptomeningeal infiltration in a patient with Waldenstrom's macroglobulinemia

et al. 2007

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confidence: 56%

Cranial and peripheral neuropathy due to leptomeningeal infiltration in a patient with Waldenstrom's macroglobulinemia

et al. 2007

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Leptomeningeale Tumorzellinfiltration als Erstmanifestation eines Immunozytoms (M. Waldenström)

et al. 2004

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Immunocytoma (Waldenstrom's macroglobulinemia) is a rare chronic lymphoproliferative disorder of B-cell origin. It is characterized by the presence of large amounts of circulating monoclonal immunoglobulin M (IgM) and lymphoplasmocytoid bone marrow infiltration. Affection of the peripheral nervous system is common and causes polyneuropathy (5-10%). An isolated leptomeningeal infiltration by neoplastic cells is very rare and has been reported in few cases only. The diagnosis is difficult, in particular if cerebrospinal fluid (CSF) cytology is inconclusive. We present the case of a patient who developed a personality disorder and cognitive impairment. Initial CSF findings were compatible with chronic lymphocytic (aseptic) meningitis. The serologic detection of IgM paraproteinemia and bone marrow cytology suggested immunocytoma. The selective analysis of B-cell clonality in both whole CSF cell lysates and individual CSF cells using polymerase chain reaction (PCR) based amplification of the rearranged CDR3 region of the IgH gene revealed the presence of a monoclonal B-cell population and was diagnostic for leptomeningeal tumor cell infiltration by immunocytoma.

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Neurological manifestations of Waldenström macroglobulinemia

et al. 2008

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Waldenström macroglobulinemia, a condition that most commonly occurs in lymphoplasmacytic lymphoma, typically manifests with diffuse lymphadenopathies, cytopenias, and a markedly elevated erythrocyte sedimentation rate. Peripheral neuropathy occurs in nearly half of patients with this condition, and hyperviscosity-related nervous system disorders are encountered in up to a third. Other neurological complications, such as encephalopathy or myelopathy caused by direct tumor infiltration, paraprotein deposition or autoimmune phenomena, are rare. Diagnosis of Waldenström macroglobulinemia requires identification of monoclonal IgM protein in the serum, bone marrow biopsy, and appropriate neurological testing (e.g. imaging studies of affected areas of the central neuraxis, electrophysiological studies). Treatment options, which should address both the paraprotein burden and the lymphoplasmacytic clone, include plasmapheresis and chemotherapy with alkylating agents, nucleoside analogs, and rituximab. As the disease is incurable and its course indolent, these treatments are only provided to symptomatic patients.

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Primary Central Nervous System Lymphoma with Waldenström’s Macroglobulinaemia

Cited by 4 publications

References 3 publications

Cranial and peripheral neuropathy due to leptomeningeal infiltration in a patient with Waldenstrom's macroglobulinemia

Cranial and peripheral neuropathy due to leptomeningeal infiltration in a patient with Waldenstrom's macroglobulinemia

Leptomeningeale Tumorzellinfiltration als Erstmanifestation eines Immunozytoms (M. Waldenström)

Neurological manifestations of Waldenström macroglobulinemia

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