Conflict of interest: Nothing to report
Diagnostic Imaging in Hematology
Hodgkin lymphoma of the central nervous system†
Article first published online: 8 JUL 2010
Copyright © 2010 Wiley-Liss, Inc.
American Journal of Hematology
Volume 85, Issue 12, page 953, December 2010
How to Cite
Simons, L., Neerudu, M., Lissack, M. and McNamara, C. (2010), Hodgkin lymphoma of the central nervous system. Am. J. Hematol., 85: 953. doi: 10.1002/ajh.21817
- Issue published online: 23 NOV 2010
- Article first published online: 8 JUL 2010
- Manuscript Accepted: 30 JUN 2010
- Manuscript Revised: 29 JUN 2010
- Manuscript Received: 23 JUN 2010
A 72-year-old Cypriot man presented with collapse and confusion. He had commenced chemotherapy for stage IVB Hodgkin lymphoma (HL) 6 months previously with good clinical response, but was noted to be poorly compliant with supportive medication, including anti-infective therapy. Computerized tomography scanning showed no evidence of residual lymphadenopathy or occult malignancy, and magnetic resonance imaging of the brain demonstrated multiple ring-enhancing lesions (left). Human immunodeficiency virus (HIV) serology was negative. Cerebrospinal fluid examination revealed raised protein levels, no bacterial growth or abnormal cells on cytospin, but Epstein-Barr virus (EBV) DNA was detected at 6,122 copies/ml. The patient was commenced on Dexamethasone, Co-trimoxazole, and Caspofungin and, following a positive result for Toxoplasma serology, empirical anti-protozoal therapy was added.
Despite anti-infective therapy, the patient continued to deteriorate clinically, and a stereotactic biopsy of one of the brain lesions was performed. This demonstrated an inflammatory cell infiltrate composed of lymphocytes, histiocytes, eosinophils, and occasional plasma cells. Admixed were many large atypical cells aggregated around the blood vessels containing pleomorphic nuclei and prominent eosinophilic nucleoli (middle). These cells stained positive for CD30 (right), CD15, and EBV-encoded RNA in situ. No microorganisms or viral inclusions were seen on immunostaining. A diagnosis of intracerebral HL was made; unfortunately the patient died before further treatment could be implemented.
Central nervous system (CNS) involvement by HL is rare, with a reported incidence of less than 0.02% . In most cases, it occurs in the setting of relapsed systemic disease, but has been known to present in isolation or with systemic disease remission. The role of EBV infection in the disease pathogenesis of systemic and CNS HL requires further characterization. Intracerebral involvement should always be considered as a cause of unexplained confusion or neurological abnormality in patients with HL. 1