Leptomeningeale Tumorzellinfiltration als Erstmanifestation eines Immunozytoms (M. Waldenström)

Abstract

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.

Waldenstrom's macroglobulinemia presenting with spinal cord compression: A case report

“…Patients with a tumoral infiltrate usually present with seizures or focal neurological signs related to the site of infiltration. In the diffuse form, infiltration of the leptomeningeal spaces, peri-ventricular white matter, pons, and medulla are common; and patients frequently demonstrate symptoms of confusion and encephalopathy [14,15,17]. Spinal cord or cauda equina complications of WM are rare.…”

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

“…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.…”