Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy

Ababou, Mounir; Mahtat, El Mehdi; Jennane, Selim; Elmaaroufi, Hicham; Mikdame, M.; Doghmi, Kamal

doi:10.1016/j.hemonc.2019.05.007

Cited by 6 publications

(6 citation statements)

References 15 publications

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“…In certain non-CNS extranodal tissue sites, the development of MZBL has been described in association with specific chronic infectious and autoimmune processes, such as within gastrointestinal mucosa colonized by H. pylori and within lacrimal glands in Sjögren syndrome [3,5]. A small number of cases of patients with both HBV and MZBL have been reported in the literature, consistent with our second reported patient who presented with multifocal lesions and a long-standing history of untreated HBV [6]. This raises consideration for potential mechanisms behind the pathogenesis of CNS MZBL in our second patient, possibly arising either secondarily to direct chronic antigenic stimulation of the dura or as metastasis from an HBV-associated primary extracranial MZBL that went undetected on PET-CT.…”

Section: Discussion/conclusionsupporting

confidence: 88%

Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

Ariani

Ballas

2021

Case Rep Oncol

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Primary CNS extranodal marginal zone B-cell lymphoma (MZBL) is a rare low-grade non-Hodgkin lymphoma characterized predominantly by small B cells, plasma cells, monocytoid cells, and scattered large immunoblasts. As a slow-growing tumor that remains localized, primary CNS MZBL carries an excellent clinical prognosis. Here, we report two cases of primary CNS MZBL successfully treated using external beam radiation therapy along with a literature review.

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Section: Discussion/conclusionsupporting

confidence: 88%

Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

Ariani

Ballas

2021

Case Rep Oncol

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“…Recently, some studies have shown coexistence between chronic hepatitis B virus infection and splenic lymphomas, particularly SMZL, suggesting a new etiological association for this neoplasm. Similar to that observed in HCV positive cases, treatment for HBV with antiviral agents is capable of inducing neoplasm remission [ 23 – 26 ].…”

Section: Discussionmentioning

confidence: 72%

Risk adapted approach: How to treat splenic marginal zone lymphoma in resource-poor settings? - The real-life experience of a Brazilian cancer treatment center

et al. 2020

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Background Splenic marginal zone lymphoma (SMZL) is a rare lymphoid B-cell malignant neoplasm with primary involvement of the spleen. It is a chronic disease, of indolent behavior and prolonged survival. However, 25% of cases have higher biological aggressiveness, propensity for histological transformation to high grade B-cell non-Hodgkin lymphoma and shortened survival. Recognition of these cases of reserved outcome is important for selecting a risk-adapted therapeutic approach in a resource-poor settings. Methods We described clinical and epidemiological characteristics, survival analysis and prognostic factors in a retrospective cohort of 39 SMZL patients, treated in Latin America. Results We observed a predominance of female (71.8%), median age of 63 years and higher incidence of B symptoms (56.4%) and extra-splenic involvement (87.1%) than in European and North-American series. With a median follow-up of 8.7 years (0.6-20.2 years), estimated 5-year overall survival (OS) and progression-free survival (PFS) were 76.9% and 63.7%, respectively. Factors with adverse prognostic impact on OS and PFS were Hb < 100 g/L, platelet count < 100 x 10 9 /L, albumin < 3.5 g/dL, LDH > 480 U/L and high-risk Arcaini and SMZL/WG scores. Despite a relative low number of patients, no superiority was observed among the therapeutic regimens used including rituximab monotherapy, splenectomy and cytotoxic chemotherapy. Conclusion Therefore, in resource-poor settings, where access to immunotherapy is not universal for all SMZL patients, we suggest that first-line should consist on rituximab therapy for elderly patients or with high surgical risk or with at least 1 risk factor identified in our study. Remainders can be safely managed with splenectomy.

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“…The possible mechanisms behind the pathogenesis of CNS MZBL could be represented by a localization of an undetected primary extracranial MZBL or a chronic in ammation. This last condition could derive from a direct chronic antigenic stimulation of the dura, for example for a long-standing history of untreated HBV [12,13] or HCV [14]. One patient with CNS MZBL had a long history of white matter disease with some features of multiple sclerosis [5] and another patient was suffering from a Chlamydia psittaci infection [15].…”

Section: Case and Literature Discussionmentioning

confidence: 99%

Primary CNS extranodal marginal zone B-cell lymphoma: an unusual localization and clinical presentation

Fiorentino

Pizzimenti

Pierconti

et al. 2023

Preprint

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Background Primary Central Nervous System (CNS) extranodal marginal zone B-cell lymphoma (MZBL) is a rare low-grade non-Hodgkin lymphoma, characterized predominantly by small B cells, plasma cells, monocytoid cells, and scattered large immunoblasts. Primary CNS MZBL is a slow-growing tumour that remains localized and is characterized by an excellent clinical prognosis. Case presentation: A 48-year-old HIV-negative female with a history of a head trauma a year earlier, presented with worsening neurological symptoms and an MRI finding of an extra-axial mass of about 3 cm within the left lateral ventricle. From histopathology and immunohistochemistry, the lesion was diagnosed as a CNS MZBL: since no other primary lesions were found, the base of the chorioid plexuses of the left lateral ventricle was considered the primary site. Conclusions The current case is the first literature reported primary CNS MZBL arising in this anatomical site and paves the way for further studies on the role of chronic inflammation (in our case resulting from a trauma) in the pathogenesis not only of primary CNS MZBLs, but also of lymphomas in general. Additionally, it could be a starting point for studies analyzing the role of meningothelial cells in the pathogenesis of primary CNS MZBL.

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Splenic marginal zone lymphoma associated with hepatitis B virus infection, remission after viral treatment, and splenectomy

Cited by 6 publications

References 15 publications

Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

Primary CNS Extranodal Marginal Zone B-Cell Lymphoma: A Case Series of 2 Patients Treated with External Beam Radiation Therapy

Risk adapted approach: How to treat splenic marginal zone lymphoma in resource-poor settings? - The real-life experience of a Brazilian cancer treatment center

Primary CNS extranodal marginal zone B-cell lymphoma: an unusual localization and clinical presentation

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