Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report
Abstract Background Hematologic malignancies can spread to the central nervous system (CNS), either as focal lesions or as leptomeningeal disease. Marginal zone lymphoma (MZL) is a low-grade non-Hodgkin lymphoma and generally presents as an indolent disease. This case report illustrates an unexpecte...
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| Format: | Article |
| Language: | English |
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BMC
2020-06-01
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| Series: | BMC Geriatrics |
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| Online Access: | http://link.springer.com/article/10.1186/s12877-020-01608-6 |
| _version_ | 1819006990795407360 |
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| author | Lisa Dreessen Nicolas Maréchal Michel Verheyden Ann De Becker Kristin Jochmans Tim Vanderhasselt Bert Bravenboer Ingo Beyer |
| author_facet | Lisa Dreessen Nicolas Maréchal Michel Verheyden Ann De Becker Kristin Jochmans Tim Vanderhasselt Bert Bravenboer Ingo Beyer |
| author_sort | Lisa Dreessen |
| collection | DOAJ |
| description | Abstract Background Hematologic malignancies can spread to the central nervous system (CNS), either as focal lesions or as leptomeningeal disease. Marginal zone lymphoma (MZL) is a low-grade non-Hodgkin lymphoma and generally presents as an indolent disease. This case report illustrates an unexpected diagnosis of leptomeningeal metastasis in an MZL, presenting as a delirium without B symptoms, pronounced hematologic progression or abnormalities on cerebral imaging. Case presentation An 80-year-old patient with a medical history of monoclonal B-cell lymphocytosis (MBL) with a clone indicative for an MZL, presented to the emergency and the geriatric departments with a recent cognitive deterioration and behavioral changes. MMSE score was 18/30. After excluding the most common etiologies through classical work-up including a normal head magnetic resonance imaging, a lumbar puncture was performed. In the cerebrospinal fluid an elevated protein level and increased lymphocyte count were identified, whereas beta-amyloid and tau protein levels were normal. Immunophenotyping of the lymphocytes confirmed CNS invasion by the MZL clone. Staging revealed mild splenomegaly. Prednisolone, intrathecal and systemic chemotherapy were initiated, leading to quick cognitive improvement with a final MMSE score of 28/30. Conclusions To the best of our knowledge a delirium in an older patient due to leptomeningeal disease in MZL has never been described. To date, rare reports of CNS invasion by MZL describe focal intracranial lesions. After exclusion of common etiologies, physicians should remain vigilant when confronted with a patient with history of MBL presenting neurological symptoms. This case illustrates the importance of low threshold for lumbar punctures in this population, also for those patients with normal imaging studies. |
| first_indexed | 2024-12-21T00:17:28Z |
| format | Article |
| id | doaj.art-0488fb25385440678277c3a3fb43b8de |
| institution | Directory Open Access Journal |
| issn | 1471-2318 |
| language | English |
| last_indexed | 2024-12-21T00:17:28Z |
| publishDate | 2020-06-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Geriatrics |
| spelling | doaj.art-0488fb25385440678277c3a3fb43b8de2022-12-21T19:22:11ZengBMCBMC Geriatrics1471-23182020-06-012011510.1186/s12877-020-01608-6Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case reportLisa Dreessen0Nicolas Maréchal1Michel Verheyden2Ann De Becker3Kristin Jochmans4Tim Vanderhasselt5Bert Bravenboer6Ingo Beyer7Department of Geriatric Medicine, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Geriatric Medicine, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Internal Medicine, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Hematology, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Hematology, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Radiology, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Geriatric Medicine, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Department of Geriatric Medicine, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel)Abstract Background Hematologic malignancies can spread to the central nervous system (CNS), either as focal lesions or as leptomeningeal disease. Marginal zone lymphoma (MZL) is a low-grade non-Hodgkin lymphoma and generally presents as an indolent disease. This case report illustrates an unexpected diagnosis of leptomeningeal metastasis in an MZL, presenting as a delirium without B symptoms, pronounced hematologic progression or abnormalities on cerebral imaging. Case presentation An 80-year-old patient with a medical history of monoclonal B-cell lymphocytosis (MBL) with a clone indicative for an MZL, presented to the emergency and the geriatric departments with a recent cognitive deterioration and behavioral changes. MMSE score was 18/30. After excluding the most common etiologies through classical work-up including a normal head magnetic resonance imaging, a lumbar puncture was performed. In the cerebrospinal fluid an elevated protein level and increased lymphocyte count were identified, whereas beta-amyloid and tau protein levels were normal. Immunophenotyping of the lymphocytes confirmed CNS invasion by the MZL clone. Staging revealed mild splenomegaly. Prednisolone, intrathecal and systemic chemotherapy were initiated, leading to quick cognitive improvement with a final MMSE score of 28/30. Conclusions To the best of our knowledge a delirium in an older patient due to leptomeningeal disease in MZL has never been described. To date, rare reports of CNS invasion by MZL describe focal intracranial lesions. After exclusion of common etiologies, physicians should remain vigilant when confronted with a patient with history of MBL presenting neurological symptoms. This case illustrates the importance of low threshold for lumbar punctures in this population, also for those patients with normal imaging studies.http://link.springer.com/article/10.1186/s12877-020-01608-6Case reportDeliriumLeptomeningeal metastasisMarginal zone lymphoma |
| spellingShingle | Lisa Dreessen Nicolas Maréchal Michel Verheyden Ann De Becker Kristin Jochmans Tim Vanderhasselt Bert Bravenboer Ingo Beyer Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report BMC Geriatrics Case report Delirium Leptomeningeal metastasis Marginal zone lymphoma |
| title | Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report |
| title_full | Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report |
| title_fullStr | Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report |
| title_full_unstemmed | Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report |
| title_short | Leptomeningeal metastasis in a marginal zone lymphoma, presenting as a delirium: case report |
| title_sort | leptomeningeal metastasis in a marginal zone lymphoma presenting as a delirium case report |
| topic | Case report Delirium Leptomeningeal metastasis Marginal zone lymphoma |
| url | http://link.springer.com/article/10.1186/s12877-020-01608-6 |
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