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Open Access Case report

Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant

Ekkehard M Kasper13*, Fred C Lam1, Markus M Luedi1, Pascal O Zinn1 and German A Pihan2

Author Affiliations

1 Division of Neurosurgery, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

2 Department of Pathology, Beth Israel Deaconess Medical Center, Harvard Medical School, Boston, MA, USA

3 110 Francis Street, Suite 3B, Boston, MA, 02215, USA

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BMC Neurology 2012, 12:64  doi:10.1186/1471-2377-12-64

Published: 3 August 2012

Abstract

Background

Lymphocyte-depleted Hodgkin’s lymphoma is the rarest form of classical Hodgkin’s lymphoma, accounting for < 1% of all cases. Patients often have advanced-stage disease at the time of presentation with an aggressive clinical course. Even more uncommon is primary extranodal disease and rarely it will be presenting with spinal cord compression.

Case presentation

An 88-year-old Caucasian female presented with a history of upper back pain for several months and new onset bilateral leg numbness and weakness. MRI of the spine showed a dorsal epidural lesion with cord compression at T1-T4 with involvement of the paraspinal muscles. The patient received urgent surgical decompression, with final histopathology showing a lymphocyte-depleted Hodgkin’s lymphoma. Systemic work-up did not show evidence of nodal disease. Following surgery, she received a course of radiotherapy with good outcome.

Conclusion

To the best of our knowledge, this is the first reported case of primary lymphocyte-depleted Hodgkin lymphoma presenting as epidural spinal cord compression. Our report, in conjunction with a review of the literature, suggests that surgical intervention is clearly indicated in de novo disease followed by radiotherapy.

Keywords:
Spinal cord compression; Primary; Lymphocyte-depleted Hodgkin’s lymphoma