Posterior reversible encephalopathy syndrome in a patient with Richter's syndrome on combination DA-R-EPOCH chemotherapy regimen: a case report

Abstract

Background: Posterior reversible encephalopathy syndrome (PRES) is a clinical-radiologic entity characterized by headaches, altered mental status, seizures, visual loss, and a characteristic imaging pattern in brain magnetic resonance images. The exact etiology and pathogenesis of this condition are not yet fully elucidated.

Case presentation: A 72-year-old White man presented with 2 weeks of low-grade fever and chills, night sweats, fatigue, dysphagia, and new-onset rapidly increasing cervical lymphadenopathy. He had a history of chronic lymphocytic leukemia with transformation to diffuse large B-cell lymphoma for which he was started on dose-adjusted rituximab, etoposide, prednisone vincristine, cyclophosphamide, and doxorubicin (DA-R-EPOCH). Shortly after treatment initiation, the patient developed severe airway obstruction due to cervical lymphadenopathy that required emergency intubation. A few days later, the cervical lymphadenopathy and the status of the airway improved, and sedation was consequently weaned off to plan for extubation. However, the patient did not recover consciousness and developed generalized refractory seizures. Brain magnetic resonance imaging revealed edema in the cortical gray and subcortical white matter of the bilateral occipital and inferior temporal lobes, consistent with PRES.

Conclusions: Posterior reversible encephalopathy syndrome refers to a neurological disorder and imaging entity characterized by subcortical vasogenic edema in patients who develop acute neurological signs and symptoms of a usually reversible nature in different settings, including chemotherapy. Despite its name, PRES is not always fully reversible, and permanent sequelae can persist in some patients. Clinicians should be aware of the possible association between chemotherapy and PRES to ensure early recognition and timely treatment.

Keywords: Chronic lymphocytic leukemia; DA-R-EPOCH; Diffuse large B-cell lymphoma; Posterior reversible encephalopathy syndrome; R-EPOCH; Richter syndrome.

Fig. 1

Lymph node biopsy showing Richter syndrome. The lymph node architecture was effaced by a diffuse proliferation of large lymphoid cells with features of immunoblasts. A starry sky pattern and frequent mitoses were also noted. The large cells were intermixed with a numerically smaller population of small, mature-appearing lymphocytes. The large cells were positive for CD20, CD79a, BCL6, and MUM1 and negative for CD10 and CD3 by immunohistochemistry. The Ki-67 proliferation index was approximately 80%, and in situ hybridization for Epstein-Barr virus was negative. According to Hans' algorithm, these findings are consistent with diffuse large B-cell lymphoma, activated B-cell subtype, arising in a background of chronic lymphocytic leukemia/small lymphocytic lymphoma

Fig. 2

Brain magnetic resonance imaging (MRI). a T2 fluid-attenuated inversion recovery (FLAIR) sequence showed mild hyperintensity of the occipital and inferior temporal lobes bilaterally (red arrows). b Diffusion-weighted imaging demonstrated restricted diffusion in the cortical gray and subcortical white matter of the same regions (red arrows) consistent with edema in the cortical gray and subcortical white matter