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• W2149208315 abstract "An 82-year-old man presented with progressive cognitive decline over a 2-month period. Fifteen years previously, he had been diagnosed with Waldenström macroglobulinemia, and he had received immunosuppressive chemotherapy intermittently. During the past 2 years, he had noted gradually progressive weakness in the arms and legs, difficulty with fine-motor movements, and trouble getting out of a chair. Clinically, there was no muscle atrophy, pain, or fasciculation; a muscle biopsy showed only mild myofiber atrophy. The remainder of his medical history was unremarkable. He received gamma globulin infusions for 2 years, but his immunoglobulin (Ig) M gammopathy (levels >2000 mg/dL; normal, <250 mg/dL) persisted. Because of persistent weakness, he was treated with 4 cycles of rituximab (375 mg/m2), and his IgM levels fell to less than 2000 mg/dL. One month later, his family noted a cognitive decline, confusion, and irritability. Analysis of cerebrospinal fluid indicated a slight increase in the protein level, but glucose levels and cell counts were normal; cytology was also normal. Cultures, serologies, and polymerase chain reaction for Epstein-Barr virus were all negative in the cerebrospinal fluid.Magnetic resonance imaging (Figure, A and B) demonstrated multiple lesions in the white matter that were isointense to brain on T1-weighted images, took up contrast poorly with gadolinium (Figure, A), and were associated with white matter edema on T2-weighted and fluid-attenuated inversion recovery images (Figure, B). Positron emission tomography showed these lesions to be hypometabolic. Stereotactic biopsies failed to reveal a diagnosis. His postoperative course was complicated by a large left-sided bronchial plug and declining respiratory status. One day prior to his death, he suffered acute development of a negative corneal reflex, a negative response to deep pain, and a negative gag reflex.At autopsy, gross examination of the brain showed a cystic lesion at the site of the previous biopsy (arrow in Figure, B) but no other obvious gross lesions. Microscopic examination showed prominent perivascular infiltrates (Figure, C) that were immunohistochemically positive for CD20 (Figure, D). These infiltrates were found not only at the grossly visible site, but also throughout the brain, corresponding not only to the lesions seen on magnetic resonance imaging, but elsewhere as well, including the cerebrum, cerebellum, midbrain, and pons.What is your diagnosis?Postmortem findings were consistent with central nervous system (CNS) involvement by Waldenström macroglobulinemia (WM). Coronal sections of the cerebral hemispheres revealed a solitary 2.0 × 2.0 × 1.0-cm lesion in the right centrum semiovale with surrounding hyperemia at the site of the recent biopsy. Horizontal sections of the brainstem and cerebellum showed no gross abnormalities. Histologic preparations from the gross lesion, as well as the striatum, midbrain, cerebellum, pons, and medulla, showed a prominent perivascular large-cell lymphoplasmacytoid infiltrate with minimal invasion of the neuropil (Figure, C). Additionally, all sampled sections of the leptomeninges showed a similar cellular accumulation. Immunohistochemical staining demonstrated that the perivascular and leptomeningeal cells were predominantly CD20-positive with κ-light chain–restricted cytoplasmic IgM positivity (Figure, D). There was no significant staining with CD3, IgG, IgA, or CD68.Waldenström macroglobulinemia was described in 1944 in 2 patients with signs that included systemic lymphadenopathy, elevated serum viscosity, and increased serum IgM levels.1 Waldenström macroglobulinemia is a disease of elderly white men, with a peak incidence in the sixth to eighth decades. Clinical symptoms are a function of tumor mass location and the properties of elevated serum IgM. Symptoms and signs are protean and may include weakness, fatigue, splenomegaly, lymphadenopathy, visual disturbances, gingival or nasal bleeding, peripheral polyneuropathy, multifocal leukoencephalopathy, sudden sensorineural hearing loss, headache, and impaired mental status.1,2 The abnormal cell in WM is of B-cell origin, which expresses the immunophenotypic markers CD19, CD20, and CD22, but not CD5 or CD23. These features, plus IgM expression, distinguish WM from chronic B-cell lymphocytic leukemia.3Central nervous system involvement by WM is rare. The combination of IgM macroglobulinemia with CNS symptoms and pathology is known as the Bing-Neel syndrome.4 Autopsy evaluation of the first reported cases in 1937 showed perivascular plasma cell and lymphocytic infiltrates surrounded by proliferated astrocytes.5,6 In 1984, Scheithauer et al6 compiled a comprehensive review of 15 histologically confirmed cases. All cases showed variations on the theme of perivascular and leptomeningeal lymphoplasmacytoid infiltrates, with or without extravasation of perivascular periodic acid-Schiff–positive material, and variable neuropil and vascular infiltration by cellular infiltrates.6 While cytologic variations of the cells make morphology alone an imperfect diagnostic tool, immunohistochemical staining for the panel of monoclonal antibodies noted above allows confirmation of CNS involvement by WM and excludes other lymphoproliferative or neoplastic disorders.In WM, therapy is generally withheld until the patient develops symptoms such as weakness, fatigue, or night sweats, or signs such as weight loss or anemia, hyperviscosity, hepatosplenomegaly or lymphadenopathy, weakness, headaches, or mental status changes. Oral chlorambucil, with or without corticosteroids, is tried first; additional therapies may include cyclophosphamide, alkylating agents such as vincristine or melphalan, gamma globulin, and plasmapheresis. A newly developed therapy recently advocated is rituximab (Rituxan), a chimeric murine-human anti-CD20 antibody developed to treat non-Hodgkin lymphoma.7,8 CD20 appears to be involved in regulating B-cell development and differentiation. CD20 is expressed in most mature B cells, appearing at the late pre–B-cell stage; most importantly, it is expressed at high levels in more than 95% of B-cell lymphomas, which makes it an attractive target for therapy. Rituximab has shown promise in treating non-Hodgkin lymphoma and other B-cell lymphoproliferative disorders and appears to have a favorable side-effect profile, the most serious CNS side effect being increased susceptibility to herpes encephalitis. Rituximab has been advocated recently to treat WM, and 50% to 75% of patients have responded.7,8 To our knowledge, this case represents the first reported case of Bing-Neel syndrome developing in spite of rituximab therapy." @default.
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• W2149208315 date "2002-10-01" @default.
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• W2149208315 title "Pathologic Quiz Case: A Man With Long-Standing Monoclonal Gammopathy and New Onset of Confusion" @default.
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