PMC6755279_01
Bone marrow aspiration/biopsy findings. (a, b) Bone marrow aspirate. Wright-Giemsa staining. (a) A large, atypical lymphocyte (x1,000). (b) Hemophagocytosis (x1,000). (c, d) Bone marrow biopsy showing hypercellular marrow. (c) Hematoxylin and eosin staining. Low-magnification image (x40). Inset: high-magnification image (x200). (d) Epstein-Barr virus-encoded RNA (EBER) in situ hybridization. Large atypical lymphocytes are positive. Low-magnification image (x40). Inset: high-magnification image (x200).
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fig1
Bone marrow aspiration demonstrated atypical lymphocytes (3.0% of all nucleated cells) (Figure 1(a)) and hemophagocytosis by macrophages, suggesting lymphoma-associated hemophagocytic syndrome (Figure 1(b)). Bone marrow biopsy revealed hypercellular marrow with diffuse infiltration of large atypical lymphocytes, which were CD20(+), CD79a(+), Bcl-2(-), CD10(-), Epstein-Barr virus-encoded RNA (EBER) in situ hybridization (+), and high Ki-67 index level (100%) (Figures 1(c) and 1(d)).
Histopathology of the angiolipoma. Large atypical lymphocytes in the lumina of the capillaries. Lymphoma cells are indicated by arrowheads. They are CD20(+), CD10(-), Bcl-6(-), and MUM-1(+). Magnification, x400. HE, hematoxylin and eosin stain.
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One of the four samples of random skin biopsy was angiolipoma containing relatively large atypical lymphocytes within the lumina of vessels (CD20(+), CD79a(+), CD10(-), Bcl-6(-), MUM-1(+), and Ki-67(+)) (Figure 2).
Magnetic resonance imaging (MRI) for CNS relapse. (a) Fluid-attenuated inversion recovery image showing a 2.3-cm mass in the infarction lesion in the white matter near the left lateral ventricle. (b) Contrast-enhanced MRI showing an evenly enhanced mass.
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On admission, MRI revealed a new 2.3-cm mass in the infarction lesion in the white matter around the left lateral ventricle (Figures 3(a) and 3(b)). It had low intensity in the T1-weighted image, higher intensity than cerebral parenchyma in the T2-weighted image (T2WI) and fluid-attenuated inversion recovery (FLAIR) (Figure 3(a)), and high intensity in the diffusion weighted image, and its apparent diffusion coefficient value was low. It was evenly enhanced in contrast-enhanced MRI (Figure 3(b)).
Autopsy findings. (a, b) Choroid plexus. (a) Hematoxylin and eosin staining. Magnification, x100. (b) CD20 staining. Magnification, x200. (c, d) Adrenal gland. (c) Hematoxylin and eosin staining. Magnification, x100. (d) CD20 staining. Magnification, x200.
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Lymphoma cells were found in capillaries in the choroid plexus (Figures 4(a) and 4(b)) and pituitary gland. Lymphoma cells were observed in the capillaries of the bilateral adrenal glands and fatty tissue around them (Figures 4(c) and 4(d)), ectopic adrenal cortex tissue around the spleen, and transverse colon.