PMC9148600_01
(A) Axial non-contrast CT on readmission showing acute right parietal intraparenchymal hemorrhage (short white arrow) with mild surrounding edema. (B) Axial T2-FLAIR MRI obtained the next day shows new abnormalities in the left hemisphere (short white arrows). (C) Associated intraparenchymal hemorrhages (short white arrows) on the axial T2* GRE MRI. (D) Abnormal linear enhancement in the conus medullaris (arrowhead). The hematoxylin and eosin (H&E) stained section of brain biopsy shows focal intravascular infiltrate of neoplastic large lymphocytes ((E) and (F), x40); and they are positive for CD20 by immunohistochemistry study (IHC) ((G), x40), which confirms the B cell lineage of the lymphoma.
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CT scan of the head on the day of readmission showed intraparenchymal hemorrhage in the right parietal lobe measuring 7x13x20 mm with surrounding edema causing a mass effect and effacement of the adjacent parietal sulci (Figure 1A). A day later, MRI of the brain showed an additional left-sided hemorrhagic focus in the precentral gyrus that was not seen on the preceding CT scan (Figure 1B and C). A biopsy of the right parietal lobe showed CD20+ large cells inside the blood vessels consistent with IVL (Figure 1). A spinal MRI T2-weighted image (T2WI) showed linear enhancement dorsal to the conus medullaris suggesting leptomeningeal involvement (Figure 1D).
PMC9148600_02
(A) Multiple small subcortical infarcts (short white arrows) on axial DWI MRI at the outside facility. (B) Recurrent small subcortical infarcts (short white arrows) 7 months later on axial DWI MRI. (C) Associated subcortical microhemorrhages (arrowheads) on axial susceptibility-weighted MRI. (D) MR angiography showing focal narrowings in intracranial arteries (arrowheads). The H&E section of spleen demonstrated neoplastic large atypical lymphocytes (black long arrow) filling the sinusoid and small vessels in the red pulp ((E), x40). Immunostains show the neoplastic lymphocytes positive for CD20 (black long arrow) with a high proliferative rate by Ki-67 (90%) ((F), x40). The clusters of large lymphocytes within the sinusoid (black long arrow) are highlighted by immunostain of CD20 and ki-67 ((G), x40).
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MRI of the brain (Figure 2) showed multiple foci of T2 FLAIR hyperintensity within bilateral cerebral hemispheres and subcortical microscopic hemorrhagic foci. Pathologic findings in the spleen were consistent with diffuse large B-cell lymphoma (DLBCL) with infiltrating neoplastic lymphocytes filling the sinusoid and small vessels within the red pulp (Figure 2). The neoplastic lymphocytes were positive for CD79a, CD20, BCL2, and MUM1 (focal and weak) with a high proliferative rate by Ki-67 (90%) (Figure 2); and were negative for CD10, BCL6, MYC, TdT, and cyclin D1 by immunohistochemistry studies.
PMC9148600_03
(A) Axial contrast-enhanced T1-weighted MRI showing multifocal subcortical perivascular intraparenchymal enhancement (short white arrows) and leptomeningeal enhancement (arrowheads). (B) Small cortical and subcortical infarcts corresponding to the distribution of perivascular enhancement (short white arrows). (C) MR angiography showing focal narrowing of several intracranial arteries (arrowheads). The H&E section of brain biopsy shows focal intravascular infiltrate of neoplastic large lymphocytes ((D), x40); and they are positive for CD19 ((E), x40).
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MRI of the brain showed multiple foci of leptomeningeal enhancement involving the supratentorial brain and the cerebellum (Figure 3). MRA of the brain showed multifocal mild narrowing of both anterior and posterior circulation arteries, even though there was no significant vessel wall enhancement to suggest vasculitis (Figure 3). Lymphoma cells were positive for CD19 and negative for programmed death-ligand 1 (PDL-1) (Figure 3).