Paraneoplastic Neuro-Ophthalmologic Symptoms as Initial Manifestation of Hodgkin Lymphoma

<b>Background and Clinical Significance:</b> Patients with Hodgkin lymphoma (HL) often present with lymphadenopathy, biochemical inflammation, and constitutional symptoms, but may experience symptoms from extra-nodal organs. Symptoms are caused by either lymphoma or a paraneoplastic phenomenon but overt central nervous system (CNS) involvement in HL is very uncommon. However, in rare cases, paraneoplastic neuro-ophthalmologic manifestations occur. <b>Case Presentation:</b> This case report describes a young female diagnosed with HL initially presenting with visual loss, reduced visual field, impaired balance, and sensory disturbances but no evidence of CNS-lymphoma. After treatment with bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, and prednisolone (escalated BEACOPP), she experienced full recovery of all neurological and ophthalmological symptoms. She experienced complete remission without any signs of relapse at follow-up after 2.5 years. Paraneoplastic cerebellar degeneration (PCD) related to HL have been described as a rare neurological syndrome, with varying neurological symptoms preceding the diagnosis of HL. PCD is typically associated with anti-Tr antibodies. Despite being negative for anti-Tr antibodies in both serum and cerebrospinal fluid (CSF), the neuro-ophthalmologic symptoms were interpreted as a paraneoplastic phenomenon in HL resembling PCD. The exact pathophysiology in this case is unknown but might be associated with undetected antigens and T-cell-mediated autoimmunity because of the presence of non-malignant T-cells in the CSF. <b>Conclusions:</b> This manuscript describes a case of an atypical presentation of HL with neuro-ophthalmologic symptoms which fully recovered upon anti-lymphoma treatment. Because of the good prognosis, we aim to emphasize the awareness of rare cases of HL initially presenting such manifestations to avoid diagnostic delays.

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Publication Details

Journal
Hematology Reports
Published
2026-01-05
DOI
https://doi.org/10.3390/hematolrep18010008
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
Field-Weighted Citation Impact
0.00

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article

Paraneoplastic Neuro-Ophthalmologic Symptoms as Initial Manifestation of Hodgkin Lymphoma

Sophie-Charlott Seidenfaden, M. A. Pedersen, Peter Kamper, Thomas Graversgaard Adams et al.
Hematology Reports
Autoimmune Neurological Disorders and Treatments
article

Paraneoplastic Neuro-Ophthalmologic Symptoms as Initial Manifestation of Hodgkin Lymphoma

Sophie-Charlott Seidenfaden, M. A. Pedersen, Peter Kamper, Thomas Graversgaard Adams, Sanne Jespersen
article en

Abstract

<b>Background and Clinical Significance:</b> Patients with Hodgkin lymphoma (HL) often present with lymphadenopathy, biochemical inflammation, and constitutional symptoms, but may experience symptoms from extra-nodal organs. Symptoms are caused by either lymphoma or a paraneoplastic phenomenon but overt central nervous system (CNS) involvement in HL is very uncommon. However, in rare cases, paraneoplastic neuro-ophthalmologic manifestations occur. <b>Case Presentation:</b> This case report describes a young female diagnosed with HL initially presenting with visual loss, reduced visual field, impaired balance, and sensory disturbances but no evidence of CNS-lymphoma. After treatment with bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, procarbazine, and prednisolone (escalated BEACOPP), she experienced full recovery of all neurological and ophthalmological symptoms. She experienced complete remission without any signs of relapse at follow-up after 2.5 years. Paraneoplastic cerebellar degeneration (PCD) related to HL have been described as a rare neurological syndrome, with varying neurological symptoms preceding the diagnosis of HL. PCD is typically associated with anti-Tr antibodies. Despite being negative for anti-Tr antibodies in both serum and cerebrospinal fluid (CSF), the neuro-ophthalmologic symptoms were interpreted as a paraneoplastic phenomenon in HL resembling PCD. The exact pathophysiology in this case is unknown but might be associated with undetected antigens and T-cell-mediated autoimmunity because of the presence of non-malignant T-cells in the CSF. <b>Conclusions:</b> This manuscript describes a case of an atypical presentation of HL with neuro-ophthalmologic symptoms which fully recovered upon anti-lymphoma treatment. Because of the good prognosis, we aim to emphasize the awareness of rare cases of HL initially presenting such manifestations to avoid diagnostic delays.

Hematology ReportsVol. 18(1)
Aarhus University Hospital (DK)
Aarhus Universitet, Aarhus Universitetshospital
Gender equality
Openalex Percentile: Top 96%
Autoimmune Neurological Disorders and Treatments
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