Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome

Sips, Gregorius J.; Amory, Colum F.; Delman, Bradley N.; Kleinman, George; Lipsey, Lewis R.; Tuhrim, Stanley

doi:10.1038/nrneurol.2009.36

Cited by 4 publications

(4 citation statements)

References 27 publications

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“…Fifty-three cases of brain MRI showed either diffusion restriction (39.6%) or abnormal T2-lesions (56.6%), which were mostly located in the subcortical regions (58.5%). The dynamic pattern of MRI diffusion-weighted imaging (DWI)/T2 lesions was noted in multiple studies (n=23) where serial neuroimaging was performed [11,12,15,16,18,[20][21][22][23][24][26][27][28][29]31,32,[34][35][36][38][39][40]44,45]. Cerebral hemorrhages were noted in 11.3% of cases (n=53) [19,25,28,39,43,46].…”

Section: Figure 3: Frequency Of Non-lateralizing Symptoms Of All Casesmentioning

confidence: 99%

“…About 30.2% of cases had enhancing lesions in brain MRI [11,13,15,16,18,30,36,39,43,45,46]. Cerebral vessel imaging was mostly unremarkable in 72.7% of cases (n=33), while predominant abnormalities include multifocal stenosis (21.2%) [20,23,[32][33][34]36,39], while two cases had large/medium vessel occlusion [12,19], and one case had distal M1 aneurysm [19]. In electroencephalogram (EEG) findings, background slowing was commonly noted (64%; n=12), while two cases had epileptiform discharges [31,37].…”

Section: Figure 3: Frequency Of Non-lateralizing Symptoms Of All Casesmentioning

confidence: 99%

“…Richie et al's study showed at least one negative result on flow cytometry in six cases, and three cases had unremarkable results[43]. Also, CSF flow cytometry of a case from Zhong et al and Sips et al showed no evidence of malignancy[20,46]. Kimura et al reported monoclonal B-cell lymphocytosis in flow cytometric analysis from cerebral bleeding sites…”

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confidence: 97%

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Intravascular Lymphoma-Associated Stroke: A Systematic Review of Case Studies

Bhagat,

Shahab,

Karki

et al. 2023

Cureus

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Intravascular lymphoma (IVL) is an aggressive systemic large B-cell lymphoma that is a rare cause of stroke. The clinical characteristics of stroke associated with IVL remain underexplored, contributing to diagnostic complexities and a high mortality rate. This study endeavors to elucidate the salient clinical and investigative features of stroke linked to this condition. A systematic review was performed using the PubMed database from the incident to August 2023 including search categories for IVL and stroke. All studies, excluding review articles, were included in this study. There were 58 cases with a confirmed diagnosis of IVL associated with stroke, with a mean age of 62.9 ± 9.6 years (female 50%). Classical lateralizing stroke symptoms were noted in only 69% of cases. Other clinical syndromes included altered sensorium (31%), rapidly progressive cognitive impairment (23%), seizures (22%), and gait disturbances (19%). Common hematological abnormalities included elevated lactate dehydrogenase (LDH, 97%), erythrocyte sedimentation rate (ESR, 79%), C-reactive protein (CRP, 61%), interleukin-2, microglobulins, and cerebrospinal fluid (CSF) protein. CSF flow cytometry was not diagnostic, and cytology was mostly negative. The dynamic pattern for DWI/T2 lesions was predominant and primarily located in the subcortical regions. Diffuse background slowing (64%) was a major finding in the electroencephalogram. Seventy-one percent of cases died (n=45) mostly due to delayed diagnosis. Only 31% were treated with first-line R-CHOP (rituximab, cyclophosphamide, doxorubicin hydrochloride, vincristine, prednisone) chemotherapy, among whom 25% died. This study suggests that IVL-associated strokes carry a high mortality rate, largely due to challenges in timely diagnosis and therapy. Unlike classical stroke syndrome, key indicators to aid in early diagnosis include a clinical syndrome of multiple non-lateralizing neurological symptoms, dynamic MRI DWI/T2-lesions primarily located in subcortical regions, elevated serum LDH, ESR, CRP, interleukins, microglobulin, CSF protein, and CSF polymerase chain reaction analysis, apart from tissue examination. Larger studies should be performed to establish diagnostic and predictive scores.

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Section: Figure 3: Frequency Of Non-lateralizing Symptoms Of All Casesmentioning

confidence: 99%

Section: Figure 3: Frequency Of Non-lateralizing Symptoms Of All Casesmentioning

confidence: 99%

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Intravascular Lymphoma-Associated Stroke: A Systematic Review of Case Studies

Bhagat,

Shahab,

Karki

et al. 2023

Cureus

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“…Cancers presenting as subacute encephalopathy are usually primary central nervous system (CNS) neoplasms, and involve temporal and forniceal areas with processes such as gliomatosis cerebri or diffusely infiltrating large B cell lymphoma or intravascular lymphoma, a rare form of large cell non-Hodgkin's lymphoma, whose variable clinical picture can include cognitive deficits with a subacute course and superimposed focal deficits and delirious episodes. 19,20 Cerebrospinal fluid and angiographic studies may not be helpful, and as MRI may not show any enhancement of characteristic tumors, brain biopsy is required to establish the diagnosis.…”

Section: Differential Diagnosis Of Immune-mediated Encephalopathy In mentioning

confidence: 99%

Immune-Mediated Encephalopathies with an Emphasis on Paraneoplastic Encephalopathies

Pruitt

2011

Semin Neurol

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Recent identification of syndromes encompassing psychiatric symptoms, seizures, and movement disorders has led to effective treatments for several previously obscure conditions now known to be immune-mediated encephalopathies. In contrast to long-recognized paraneoplastic neurologic syndromes associated with antibodies to intracellular antigens, these more recently described disorders are not always paraneoplastic, are associated with cell surface antibodies, and may respond to immunosuppressive therapies. In this review, the author discusses clinical presentations, differential diagnosis, immunologic evaluation, and therapeutic options for both groups of disorders.

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Intravascular lymphomatosis and intracerebral haemorrhage

Passarin¹,

Wen

Vattemi

et al. 2010

Neurol Sci

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Intravascular lymphomatosis (IVL) is a rare, malignant B- or T-cell lymphoma with remarkable affinity for the endothelial cells of small vessels, particularly within the skin and central nervous system. It is a disease that mimics several neurological disorders, particularly those of cerebrovascular ischemic origin. The prognosis is generally poor, with a rapidly fatal outcome. As a result the diagnosis is often made at post-mortem. We report a rare case of a 73-year-old patient with IVL complicated by intracerebral haemorrhage. In literature two cases of systemic IVL complicated by intracerebral haemorrhage have been reported, but they presented initially with a disseminated intravascular coagulation (DIC). This is the first case of brain IVL complicated by intracerebral haemorrhage not associated to DIC. Increasing awareness of this disease as a differential diagnosis to a common clinical presentation may lead to more opportunities to evaluate new diagnostic and treatment approaches.

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Intravascular lymphomatosis of the brain in a patient with myelodysplastic syndrome

Abstract: Combined chemotherapy with CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) and rituximab.

Cited by 4 publications

References 27 publications

Intravascular Lymphoma-Associated Stroke: A Systematic Review of Case Studies

Intravascular Lymphoma-Associated Stroke: A Systematic Review of Case Studies

Immune-Mediated Encephalopathies with an Emphasis on Paraneoplastic Encephalopathies

Intravascular lymphomatosis and intracerebral haemorrhage

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