CNS intravascular lymphoma: an underappreciated cause of rapidly progressive dementia

Panda, Akhila Kumar; Malik, Seema

doi:10.1136/bcr-2014-203772

Cited by 6 publications

(2 citation statements)

References 9 publications

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“…IVL can involve any organ system, particularly the central nervous system (CNS), and its protean neurological manifestations o en hinder diagnosis. Patients can present with subacute encephalopathy or dementia, paralysis, seizures, or multifocal cerebrovascular events, by order of frequency [1,4,5]. Systemic symptoms vary, but fever and skin lesions are the most common, whereas lymphadenopathy is usually absent [2].…”

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confidence: 99%

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections

Chen

Singh

McNally

et al. 2020

Case Reports in Neurological Medicine

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Introduction. Intravascular lymphoma (IVL) is an uncommon and often fatal disease characterized by intraluminal proliferation of lymphomatous cells within blood vessels. Because of a heterogeneous clinical presentation and lack of sensitive diagnostic protocols, diagnosis of IVL is most often made at autopsy. However, with early diagnosis and appropriate chemotherapy, the prognosis is greatly improved and complete remission is possible. In order to broaden the possible presentations of IVL, we present a patient with an atypical manifestation of biopsy-proven intravascular large B-cell lymphoma who suffered dissections of both intracranial and extracranial arteries in addition to progressive intracranial hemorrhages. Case Report. A 47-year-old woman presented with unilateral paresthesias. She developed progressive multifocal infarcts and hemorrhage with dissections of both intracranial and extracranial arteries, resulting in coma. Brain biopsy revealed IVL. She received aggressive chemotherapy and remains in complete remission with good neurologic recovery. Conclusion. IVL is known to exert its pathology on small arteries and capillaries, but is not known to cause dissections of large vessels. The diagnosis should be considered in cases with unexplained arterial dissections and progressive strokes. Early diagnosis with appropriate laboratory screening and tissue confirmation by biopsy can lead to greatly improved outcomes.

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Section: Introductionmentioning

confidence: 99%

Intravascular Lymphoma with Progressive CNS Hemorrhage and Multiple Dissections

Chen

Singh

McNally

et al. 2020

Case Reports in Neurological Medicine

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“…Cases of IVL almost always occur in extranodal sites, including the skin, cen tral nervous system (CNS), bone marrow, lung and liver [1,2]. B-cell lineage lymphoma is the major variant in IVL, accounting for approximately 85-90% of cases of IVL [3]. Intravascular natural killer (NK)/T cell lymphoma (IVNKTL) is regarded as a rare variant, involving mostly the skin [2], To the best of our knowledge, to date no cases of IVNKTL primarily involving the central nervous system (CNS) have been reported.…”

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confidence: 99%

Primary intravascular natural killer/T cell lymphoma of the central nervous system

Xie

Zhou

Zhang

et al. 2014

Leukemia & Lymphoma

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Intravascular lymphoma (IVL) is a rare neoplasm charac terized by proliferation of malignant cells within the small vessel lumina, particularly capillaries. Cases of IVL almost always occur in extranodal sites, including the skin, cen tral nervous system (CNS), bone marrow, lung and liver [1,2]. B-cell lineage lymphoma is the major variant in IVL, accounting for approximately 85-90% of cases of IVL [3]. Intravascular natural killer (NK)/T cell lymphoma (IVNKTL) is regarded as a rare variant, involving mostly the skin [2], To the best of our knowledge, to date no cases of IVNKTL primarily involving the central nervous system (CNS) have been reported. Here, we report a case of primary IVNKTL of the brain with neurological symptoms as the initial presentation.A 46-year-old man developed a left frontotemporal headache and progressive decline of language ability for more than 1 month. Magnetic resonance imaging (MRI) of the brain showed multiple intracranial lesions within the left frontotemporal and right occipital lobes, and punctate ischemic changes in both the frontal and subcortical lobes. Cerebrospinal fluid (CSF) analysis revealed a normal protein level. Afterthepatientwastreatedwithmannitol,theheadache symptoms were slightly alleviated. No other organ alteration was found by computed tomography (CT) scan of the chest and abdomen. A whole body positron emission computed tomography (PECT) scan revealed only a lesion in the brain, and no lesions were detected in the skin. He received a left frontotemporal craniotomy tumor resection on 12 November 2012. A brain biopsy was taken from the left frontotemporal and right occipital lobes. Gross examination showed a grayred tissue that was 5 cm X 4 cm X 4 cm in size. Histologically, medium-to large-sized pleomorphic lymphoid cells with an abundant eosinophilic cytoplasm and irregular nuclei were confined within the small vasculature and capillaries, but not in the brain tissue [ Figure 1(a)]. The majority of the lumen was completely obstructed by neoplastic cells, and some capillaries contained neoplastic cells. Fibrin thrombi mixed with neoplastic cells were occasionally found in large lumina. Several vessels were surrounded by a small number of reac tive lymphocytes. Intraparenchymal necrosis was observed in localized areas. Based on the morphology, we suspected the case to be intravascular large B-cell lymphoma. However, immunohistochemical analysis did not support the diagnosis of B-cell lymphoma. The neoplastic cells were CD3e+, granzyme B + , CD8+, C D 4-, C D 5-, CD30-, CD20-, CD56-, (3F1 -and T cell receptor (TCR)y/8-. Approximately 100% of cells expressed Ki-67. Epstein-Barr virus (EBV) infection was detected in the tumor cells by in situ hybridization for EBV-encoded small RNA (EBER) [ Figure 1(b)]. Based on the morphology, immunophenotype and EBV infection, the case was diagnosed as IVNKTL of the brain. In addition, we took bone marrow from the patient for chromosome analysis and found no abnormalities. The bone marrow lacked CD3e and EBER positive neoplastic ...

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