case_images: PMC9635027_01
case_images: Histological findings of an axillary lymph node (hematoxylin and eosin staining and immunostaining) at diagnosis of lymphoplasmacytic lymphoma/Waldenstrom's macroglobulinemia.. There is diffuse proliferation of small abnormal lymphocytes with plasmacytoid differentiation. Immunohistochemically, abnormal lymphocytes were positive for CD20, IgM, and kappa; and negative for CD5, CD10, CD23, and lambda.
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case_images: Immunohistochemically, abnormal lymphocytes were positive for CD20, IgM, and kappa; and negative for CD5, CD10, CD23, and lambda (Figure 1).
case_images: Magnetic resonance imaging (MRI) and 18F-fluorodeoxyglucose positron emission tomography/computed tomography images.. a) T2-weighted MRI reveals areas of hyperintensity that show contrast enhancement in the medulla, surface of the right temporal lobe, bilateral basal ganglia, parietal cortex, and spinal cord at the Th2-11 levels, suggesting a diagnosis of Bing-Neel syndrome. 18F-fluorodeoxyglucose positron emission tomography/computed tomography shows abnormal 18F-fluorodeoxyglucose accumulation in the medulla and spinal cord at the Th5-8 levels.. b) The areas of signal hyperintensity show improvement after craniospinal irradiation.. c) There is progression of the areas of hyperintensity and contrast enhancement in the medulla and bilateral basal ganglia at two years after craniospinal irradiation.. d) Areas of signal hyperintensity appear indistinct at two months of treatment with tirabrutinib.
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case_images: Th2-11 levels. 18F-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography also showed abnormal FDG accumulation in the medulla and thoracic spinal cord at the Th5-8 levels (Figure 2a). The cerebrospinal fluid (CSF) cell count was 14/mul, all of which were mononuclear cells. Craniospinal irradiation of 30.6 Gy improved the neurological symptoms and MRI findings (Figure 2b). Three years after craniospinal irradiation, serum IgM level, CRP, and soluble IL-2 receptor increased to 1032 mg/dl, 18.5 mg/dl, and 2354 U/ml, respectively, and T2-weighted MRI showed recurrence of the areas of hyperintensity that exhibited contrast enhancement in the medulla and bilateral basal ganglia (Figure 2c). The areas of hyperintensity in the medulla and bilateral basal ganglia on MRI became indistinct (Figure 2d) at two months, with disappearance of the myoclonus of lower limbs due to basal ganglion lesions, and improvement in the Manual Muscle Test (MMT) of lower limbs from MMT 4 to MMT 5.
case_images: Histological findings of cerebrum with hematoxylin and eosin staining and immunostaining at diagnosis of Bing-Neel syndrome.. There is diffuse proliferation of small abnormal lymphocytes with plasmacytoid differentiation. Immunohistochemically, abnormal lymphocytes were positive for CD20, IgM, and kappa; and negative for CD10 and lambda.
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case_images: Immunohistochemically, abnormal lymphocytes were positive for CD20, IgM, and kappa; and negative for CD10, CD23, and lambda (Figure 3).
case_images: Clinical course following treatment with tirabrutinib.. Tirabrutinib was started at 480 mg/day. Serum IgM levels, soluble IL-2 receptor, and CRP had decreased at one month after the initiation of tirabrutinib treatment. At two months, myoclonus of lower limbs due to basal ganglion lesions disappeared along with improvement in the Manual Muscle Test of lower limbs.
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case_images: T-cell phenotype by flow cytometry. Tirabrutinib was started at 480 mg/day for the second recurrence of WM and progression of BNS (Figure 4).