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

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...

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Autores principales: Kasper, Ekkehard M, Lam, Fred C, Luedi, Markus M, Zinn, Pascal O, Pihan, German A
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2012
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3482592/
https://www.ncbi.nlm.nih.gov/pubmed/22862748
http://dx.doi.org/10.1186/1471-2377-12-64
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author Kasper, Ekkehard M
Lam, Fred C
Luedi, Markus M
Zinn, Pascal O
Pihan, German A
author_facet Kasper, Ekkehard M
Lam, Fred C
Luedi, Markus M
Zinn, Pascal O
Pihan, German A
author_sort Kasper, Ekkehard M
collection PubMed
description 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.
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spelling pubmed-34825922012-10-29 Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant Kasper, Ekkehard M Lam, Fred C Luedi, Markus M Zinn, Pascal O Pihan, German A BMC Neurol Case Report 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. BioMed Central 2012-08-03 /pmc/articles/PMC3482592/ /pubmed/22862748 http://dx.doi.org/10.1186/1471-2377-12-64 Text en Copyright ©2012 Kasper et al.; licensee BioMed Central Ltd. http://creativecommons.org/licenses/by/2.0 This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Kasper, Ekkehard M
Lam, Fred C
Luedi, Markus M
Zinn, Pascal O
Pihan, German A
Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
title Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
title_full Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
title_fullStr Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
title_full_unstemmed Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
title_short Primary epidural lymphocyte-depleted Hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
title_sort primary epidural lymphocyte-depleted hodgkin’s lymphoma of the thoracic spine – presentation of a rare disease variant
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3482592/
https://www.ncbi.nlm.nih.gov/pubmed/22862748
http://dx.doi.org/10.1186/1471-2377-12-64
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