Primary low-grade diffuse small lymphocytic lymphoma of the central nervous system

There have been few reports on central nervous system (CNS) involvement in chronic lymphocytic leukemia (CLL). This is an extremely rare disease with poor prognosis, owing to resistance to various treatments. We describe a 33-year-old man with intractable CLL with CNS involvement. He was diagnosed with CLL, with diplopia as the first manifestation. Magnetic resonance imaging revealed a contrast-enhancing tumor in the right temporal lobe, which was diagnosed as CNS involvement in CLL on brain biopsy. High-dose methotrexate therapy was ineffective for this lesion, which was also resistant to subsequent whole-brain irradiation, treatment with fludarabine–cyclophosphamide–rituximab chemoimmunotherapy, and ibrutinib administration. Because no standard protocol exists for CLL with CNS involvement, it is important to accumulate case data to verify the choice of new drugs for administration at an early stage. Therefore, we also conducted a literature review of 50 case reports of CNS lesions in the last 10 years to consider the pathophysiology, diagnosis, and treatment of CNS involvement in CLL. The possibility of new therapeutic agents, eg, ibrutinib and venetoclax, or a combination of these agents and methotrexate, can be envisioned as a treatment strategy for CLL with CNS involvement.

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“… Bcr-2013-202,051. 37 45 F Seizures, headaches, and vomiting NA NA NA NA Biopsy Surgical excision RT Relapse Clin Lymphoma Myeloma Leuk. 2013.13.…”

Section: Discussionmentioning

confidence: 99%

Refractory Chronic Lymphocytic Leukemia with Central Nervous System Involvement: A Case Report with Literature Review

Nakanishi

Ito

Fujita

et al. 2020

JBM

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“…Primary CNS SLL is extremely rare. By review of the literature, only two cases of primary SLL involving the brain parenchyma and dura have been reported so far [5, 6]. In this study, for the first time, we reported two cases of primary SLL located in the bilateral ventricles with identical clinical presentations, neuroimaging features, pathological results and prognoses.…”

Section: Discussionmentioning

confidence: 60%

Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports

et al. 2019

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Background Primary central nervous system (CNS) small lymphocytic lymphoma (SLL), as a type of low-grade lymphoma, is extremely rare. The diagnosis of CNS SLL is challenging due to its variable clinical and radiological features, which may overlap with those of diffuse large B-cell lymphoma (DLBCL). Primary CNS SLL differs from DLBCL in that it has an indolent clinical course and a good prognosis. Thus, it is important to distinguish SLL from DLBCL. By reviewing the literature, only two cases of low-grade SLL, primarily located in the CNS and involving the brain parenchyma and dura, have been reported. To our knowledge, primary CNS SLL in the bilateral ventricles has never been reported. Interestingly, the two cases in our report are identical in terms of the clinical presentations, magnetic resonance imaging (MRI) features, pathological results and prognoses. Case presentation Both patients presented with headaches. MRI suggested solid lesions located in the bilateral ventricles that were isointense on T1-weighted images and hypointense on T2-weighted images. After the injection of contrast agent (gadolinium, Gd), the intraventricular lesions were homogeneously enhanced and hyperperfused. CSF cytology revealed malignant cells. Brain biopsy revealed diffuse proliferation of small lymphocytes with positive labelling of B-cell immunomarkers. The primary origin in the CNS was confirmed with no evidence of systemic lymphoma. Two patients were given high doses of methotrexate-based chemotherapy and were free from symptoms and progression for more than 1-year of follow-up. Conclusions The presence of homogeneously enhanced intraventricular MRI lesions should raise the suspicion of primary CNS SLL.

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“…To gain further insight into the occurrence of low grade B-cell lymphomas and their association with the formation of CNS amyloidomas, we performed a literature search of the PubMed database for primary CNS small low grade B-cell lymphoma. Our search identified 12 cases of primary CNS lymphoplasmacytic lymphoma [ 2 , 8 – 10 ], one large case series with 69 cases of primary CNS marginal zone lymphoma (MZL) [ 11 ], one case of primary CNS follicular lymphoma [ 12 ], and one case of primary CNS small lymphocytic lymphoma (SLL) [ 13 ]. Among these cases, we found 5 cases of primary CNS low grade small B-cell lymphomas that presented in association with intracerebral AL amyloid deposition [ Table 1 ].…”

Section: Discussionmentioning

confidence: 99%

Cerebral Amyloidoma Resulting from Central Nervous System Lymphoplasmacytic Lymphoma: A Case Report and Literature Review

Jagannathan

Uppal

Judy

et al. 2018

Case Reports in Pathology

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Cerebral amyloidomas are rare cerebral mass lesions often associated with significant morbidity. Cerebral amyloid accumulation can be the result of a number of disease states and it is crucial for proper patient care to identify the pathogenic process leading to amyloidoma formation. Low grade clonal B-cell processes are one cause of cerebral amyloidomas. We report a case of an 87-year-old woman who presented with a lymphoplasmacytic lymphoma associated cerebral amyloidoma complicated by cerebral hemorrhage, discuss the proper workup of this disease entity, and present a review of the literature on this topic.

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Primary low-grade diffuse small lymphocytic lymphoma of the central nervous system

Cited by 7 publications

References 16 publications

Refractory Chronic Lymphocytic Leukemia with Central Nervous System Involvement: A Case Report with Literature Review

Refractory Chronic Lymphocytic Leukemia with Central Nervous System Involvement: A Case Report with Literature Review

Primary central nervous system small lymphocytic lymphoma in the bilateral ventricles: two case reports

Cerebral Amyloidoma Resulting from Central Nervous System Lymphoplasmacytic Lymphoma: A Case Report and Literature Review

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