Long Spinal Cord Lesions Caused by Venous Congestive Myelopathy Associated with Intravascular Large B-cell Lymphoma

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a subtype of B-cell lymphoma, characterized by lymphoma cell proliferation within small blood vessels. We herein describe a rare case with long spinal cord lesions caused by venous congestive myelopathy associated with IVLBCL. An 81-year-old man presented with paraplegia of the lower limbs and sensory disturbances. Magnetic resonance imaging revealed intramedullary longitudinal T2-hyperintensity lesions in the thoracic cords. The patient died three months after disease onset, and a neuropathological analysis revealed predominantly atypical B-lymphocytes located sparsely in the veins of the spinal cord. IVLBCL should be considered in the differential diagnoses of long spinal cord lesions.

Keywords: IVL; IVLBCL; MALT lymphoma; intravascular lymphoma; long spinal cord lesion; venous congestive myelopathy.

Figure 1.

1.5 Tesla magnetic resonance imaging (MRI) of the spinal cord. a: On the first hospital day, T2-weighted image (T2WI, the left panel) shows an intramedullary lesion with high signal intensity at the T3 to T6 level. b: The second MRI 10 days after admission. T2WI hyperintensity lesions tend to expand and split between T3 to T4 and T5 to T6. c: The third MRI on the 18th day after admission. T2WI (the left panel) reveals that the hyperintensity lesions remain unchanged and gadolinium-enhanced T1WI (the right panel) demonstrates abnormal mild gadolinium enhancement on the dorsal edge of T4 to T5. d: The fourth MRI on the 44th day after admission. The T2WI (the left panel) shows that the hyperintensity lesions further expand and divide into the upper part of C7 to T4 and the lower part of T4 to T9. The gadolinium enhancement T1WI (the right panel) demonstrates the faint enhancement effect on the height of T4 to T5.

Figure 2.

The endoscopic findings and gastric mucosa biopsy findings. a: Esophagogastroduodenoscopy 67 days after admission reveals the growth of the edematous mucosal lesion (arrow). b: The tumor cells with increasing size and cellularity with a high nuclear-to-cytoplasmic ratio [Hematoxylin and Eosin (H&E) staining, ×10]. c: Evident nuclear atypia (H&E staining, ×40). d: The tumor cells with CD20-immunoreactivity (CD20, ×20). e: Ki-67 labeling index over 50% (Ki-67, ×20). Scale bar: 100 μm in b.

Figure 3.

A summary of the patient’s clinical course. The patient died 77 days after admission, approximately 3 months after onset. AMPC: amoxicillin, BMA: bone marrow aspiration, CM: clarithromycin, CPA: cardiopulmonary arrest, EGD: esophagogastroduodenoscopy, LDH: lactate dehydrogenase, mPSL: methylprednisolone, P-CAB: vonoprazan, RBC: red blood cell, sIL-2R: soluble interleukin-2 receptor, u: unit, β2MG: β2-microglobulin

Figure 4.

Histopathologic features of the spinal cord in the studied patient. a: Fixed sections showing extensive and multifocal lesions with hemorrhage from T3 to S1 segments. Note severely affected lesions of the thoracic cord. Macroscopically, no hemorrhagic lesion appears above the level of T2. b: Semi macroscopic features subjected to Klüver-Barrera (KB) staining. Focal lesion of the left lateral column and Wallerian degeneration of the bilateral gracile fasciculus (arrows) in T3. Multifocal lesions in the gray and white matter in T6. i: Focal necrosis, ii: Gray matter hemorrhage, iii: Peripheral vacuolation, iv: Diffuse necrosis [KB, ×1.25, i-iv, Hematoxylin and Eosin (H&E) staining, ×10]. c, d: Some CD20-positive atypical B-lymphocytes within the lumen of the spinal intramedullary vein (c: H&E staining, ×20, d: CD20, ×20). e: A vein in the subarachnoid space with fibrous thickening and obstruction (H&E staining, ×10). Scale bar: 4 mm in a, 1.5 mm in b, 100 μm in i–iv, 25 μm in c and d; 50 μm in e.