Isolated Richter’s syndrome of the brain: diagnosis in the eye of the beholder
Letter to the Editor
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A 67-year-old woman presented with headache, diplopia, reduced bilateral visual acuity, and motor impairment on her right side. She had a diagnosis of high-count monoclonal B-lymphocytosis of chronic lymphocytic leukemia (CLL) for 3 years. Neurological examination revealed a complete left oculomotor palsy and right hemiparesis, and ophtalmological examination showed bilateral vitreous haze. Lymphadenopathy and hepatosplenomegaly were absent. Brain magnetic resonance imaging (MRI) showed a diffuse, gadolinium-enhancing lesion in the upper brainstem, thalamus, and internal capsule, predominantly on the left side (Fig. 1a, b). Cerebrospinal fluid analysis was negative for tumor cells. Brain biopsy revealed a focal infiltrate of a lymphoid neoplasia composed by medium-sized lymphocytes that expressed CD20/Bcl-2/Bcl-6 and high Ki-67 index. Reduced sample size hampered a definitive histopathological diagnosis. Given the risk of a second biopsy of the brainstem, a vitrectomy was...
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Conflict of interest
The authors declare that they have no conflict of interest.
Informed consent was obtained from the patient for being included in the study.
- 1.Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (2008) WHO Classification of tumours of haematopoietic and lymphoid tissues, 4th edn. IRAC, LyonGoogle Scholar
- 8.Dunbar M, Dilli E, Slack GW, Villa D (2016) Chronic lymphocytic leukemia presenting with concurrent isolated CNS parenchymal and leptomeningeal large B-cell lymphoma. Ann Hematol Oncol 3(6):1098Google Scholar
- 10.Woroniecka R, Rymkiewicz G, Grygalewicz B, Błachnio K, Rygier J, Jarmuz-Szymczak M, Ratajczak B, Pienkowska-Grela B (2015) Cytogenetic and flow cytometry evaluation of Richter syndrome reveals MYC, CDKN2A, IGH alterations with loss of CD52, CD62L and increase of CD71 antigen expression as the most frequent recurrent abnormalities. Am J Clin Pathol 143:25–35CrossRefPubMedGoogle Scholar
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