Intravascular large B-cell lymphoma presenting with hearing loss and dizziness: A case report

Abstract

Rationale: Intravascular large B-cell lymphoma (IVLBCL) is a type of malignant lymphoma in which neoplastic B cells proliferate selectively within the lumina of small- and medium-sized vessels. Patients with IVLBCL frequently develop neurological manifestations during their disease course. Patients are known to often develop various neurological manifestations, but there are only a few reports of IVLBCL whose initial symptoms are deafness and/or disequilibrium.

Patient concerns: A 66-year-old Japanese man was provisionally diagnosed with sudden sensorineural hearing loss. Administration of prednisolone did not improve his symptoms, and then he experienced amaurosis fugax. Magnetic resonance imaging (MRI) showed multiple brain infarcts, so he was administered antithrombotic drugs. Nevertheless, he experienced recurrent strokes, became irritable, and had visual hallucinations. He was emergently admitted to our hospital with disturbance of consciousness.

Diagnosis: Blood tests showed elevation of lactose dehydrogenase and soluble interleukin-2 receptor. Cranial MR diffusion-weighted imaging showed multiple lesions bilaterally in the cerebral white matter and cortex, posterior limbs of the internal capsule, and cerebellar hemispheres, which were hypointense on apparent diffusion coefficient maps. Hyperintense lesions were detected bilaterally in the cerebral white matter and basal ganglia on both T2-weighted imaging and fluid-attenuated inversion recovery imaging. Contrast-enhanced brain MRI demonstrated contrast-enhancing high-signal lesions along the cerebral cortex. Brain biopsy revealed a diagnosis of IVLBCL.

Interventions: The patient could not receive chemotherapy because of his poor general condition. Therefore, we administered high-dose methylprednisolone (mPSL) pulse therapy.

Outcomes: There was little improvement in consciousness levels after the high-dose mPSL pulse therapy. On the forty-ninth day of hospitalization, he was transferred to another hospital to receive supportive care.

Lessons: IVLBCL should be regarded as an important differential diagnosis of hearing loss and dizziness. Most importantly, if the symptoms are fluctuant and steroid therapy is not effective, biopsy should be considered as early as possible.

Figure 1

Diffusion-weighted magnetic resonance (MR) images on the admission day (A–C) and on the thirteenth day (D–E) of hospitalization. (A) Diffusion-weighted images, showing multiple high-intensity lesions in bilateral cerebral white matter and cortex, posterior limbs of the internal capsule, and cerebellar hemispheres. (B) Apparent diffusion coefficient maps. (C) T2-weighted MR images demonstrating multiple high-intensity areas in bilateral cerebral white matter and basal ganglia. (D and E) Gadolinium-enhanced T1-weighted MR images on the thirteenth day of hospitalization showing contrast-enhancing high-signal areas along the cortex in regions of the right frontal lobe (open arrow in D), bilateral parietal lobe, and left parieto-occipital region (arrows in D). The lesion at the right frontal lobe is magnified and demonstrated in (E).

Figure 2

Histopathology of the biopsy brain specimen. (A) Hematoxylin and eosin staining showing intravascular proliferation of neoplastic lymphoid cells (arrows). (B) CD20 and (C) CD79a staining. Original magnifications: A, ×200; B and C, ×100.