Intravascular large B-cell lymphoma presenting as multiple stroke

Yünce, Muharrem; Muganlinskaya, Nargiz; Selinger, Stephen

doi:10.1097/md.0000000000012793

Cited by 8 publications

(6 citation statements)

References 14 publications

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“…IVLBCL is rare; therefore, the majority of the data are case reports and expert panel opinions [1]. Although the true incidence of IVLBCL is unknown, some studies have reported that the incidence is less than one person per million [2][3]. The average age at the time of diagnosis is in the sixth and seventh decades of their lives, and men and women are affected equally [2,[4][5].…”

Section: Discussionmentioning

confidence: 99%

“…Traditionally, such cases have been diagnosed during an autopsy [5,12]. To date, the diagnosis of IVLBCL relies on a combination of clinical features (multiple admissions and progressive symptoms) and pathological findings on biopsy [3]. The consensus on biopsy strategy is poor.…”

Section: Discussionmentioning

confidence: 99%

“…One of the largest retrospective studies conducted on 106 patients were treated with either chemotherapy alone (57%) or chemotherapy plus rituximab (49%) [17]. Response to treatment may vary depending on the organs involved; however, studies have reported generally favorable responses, with some patients showing complete remission [2][3].…”

Section: Discussionmentioning

confidence: 99%

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A Rare Case of Intravascular Large B-cell Lymphoma Presenting With Bilateral Ophthalmoplegia, Along With a Literature Review

Allen¹,

Shaikh²,

Laurent-Ariot³

et al. 2022

Cureus

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Intravascular large B-cell lymphoma (IVLBCL) is a subtype of extranodal lymphoma that characteristically contains malignant lymphocytes within blood vessels. The clinical presentation of IVLBCL has high variability. In our case, the patient's initial presentation involved bilateral ptosis, restricted extraocular movements, periorbital pain, and bitemporal headache. The patient denied the classic "B symptoms" such as fever, night sweats, or weight loss. The patient also denied a family history of malignancy. Initial imaging studies were unremarkable, making diagnosis particularly challenging. Ultimately, functional endoscopic sinus surgery was performed. Pathological examination of the intraoperative specimen revealed a CD5+ large B-cell lymphoma within the vessels involving the left ethmoid sinus, respiratory mucosa, and nasal septum. The patient underwent steroid therapy prior to diagnosis, which led to rapid improvement in headache and mild improvement in extraocular function and ptosis. Following diagnosis, the patient underwent chemotherapy with supportive medications. Our case report may be considered a reference for cases presenting with extensive bilateral extraocular muscle deficits and levator palpebrae dysfunction in the absence of notable initial imaging findings, "B symptoms," or positive family history. The teaching point from this case is to demonstrate the difficulty of diagnosis and our train of thought in investigating an abnormal presentation with no clearly identifiable etiology following initial diagnostic workup and treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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A Rare Case of Intravascular Large B-cell Lymphoma Presenting With Bilateral Ophthalmoplegia, Along With a Literature Review

Allen¹,

Shaikh²,

Laurent-Ariot³

et al. 2022

Cureus

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“…Immunophenotyping of peripheral or CSF leucocytes does not appear to be a helpful technique in the diagnosis of this neoplasm, as it turns out negative in the majority of cases 3 (as disclosed in this case), due to the lack of circulating lymphoma cells. 9 There are no pathognomonic imagiological features in IVLBCL. Previous studies reported no brain lesions on MRI in half of patients with neurological manifestations, 5 however a recent study reported otherwise: the presence of brain lesions in MRI in patients without neurological symptoms.…”

Section: Discussionmentioning

confidence: 99%

Intravascular Lymphoma: the great imitator – a case report

Raimundo¹

2020

Sinapse

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Introduction: Intravascular large B-cell lymphoma (IVLBCL) is an uncommon and aggressive lymphoma, presented typically with diverse and progressive neurological manifestations associated with constitutional symptoms. However, the lack of specific features makes its diagnosis challenging.Case Report: A 58-year-old man presented with progressive focal deficits, behavioral alterations, consciousness fluctuation and fever of unknown cause. Neuroimaging showed multiple ischemic infarctions, despite the absence of embolic source, and CSF analysis showed pleocytosis with elevated proteins, with no evidence of neoplastic cells. Brain biopsy revealed intravascular proliferation of neoplastic lymphoid cells, CD20 positive, confirming the diagnosis of IVLBCL.Discussion: Although the IVLBCL demonstrates a central nervous system predilection, it is uncommon to exclusively involve it. This case highlights the difficulty of achieving this diagnosis, suggesting that lesion biopsy should be planned as soon as possible. ResumoIntrodução: O linfoma intravascular de grandes células B (IVLBCL) é um linfoma raro e agressivo, cuja apresentação consiste tipicamente em manifestações neurológicas progressivas associadas a sintomas constitucionais. Contudo, a ausência de achados específicos torna o seu diagnóstico desafiante.Caso Clínico: Senhor de 58 anos apresentou uma clínica de défices neurológicos focais progressivos, alteração de comportamento, flutuação da consciência e febre de etiologia indeterminada. O estudo imagiológico cerebral revelou múltiplas lesões isquémicas recorrentes, na ausência de foco embólico, e o estudo de líquor revelou uma pleocitose com proteinorráquia, com estudo anatomopatológico negativo. A biópsia lesional revelou proliferação intravascular de células linfoides neoplásicas, CD20 positivas, confirmando o diagnóstico de IVBCL.Discussão: Apesar do IVLBCL apresentar uma predileção pelo sistema nervoso central, o seu envolvimento exclusivo é raro e acarreta dificuldades diagnósticas. Este caso exemplifica a necessidade de realização precoce de biópsia lesional.

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“…subaguda (27 %) y neuropatías periféricas o craneales (21 %) (5). Además, pueden observarse síntomas inespecíficos como fiebre, malestar general, pérdida de peso y astenia en 50 % de los casos (5,6).…”

Section: Discussionunclassified

Linfoma intravascular de células B grandes con afección cerebral

Mayner-Tresol

2020

CES Med

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El linfoma intravascular de células B grandes es una rara variante del linfoma no Hodgkin que se caracteriza por proliferación clonal de células linfoides neoplásicas que crecen dentro de la luz de la microvasculatura. La diversidad de su presentación clínica dada su posibilidad de aparición en cualquier órgano, a menudo lo convierte en un diagnóstico difícil. El espectro clínico es heterogéneo dominado por manifestaciones neurológicas y puede pasarse por alto o diagnosticarse erróneamente. El diagnóstico sigue siendo un reto, ya que no existen hallazgos patognomónicos de neuroimágenes y aún no se ha establecido un tratamiento óptimo. Presentamos el caso de una mujer de 38 años con linfoma intravascular de células B grandes con afección cerebral.

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Intravascular large B-cell lymphoma presenting as multiple stroke

Cited by 8 publications

References 14 publications

A Rare Case of Intravascular Large B-cell Lymphoma Presenting With Bilateral Ophthalmoplegia, Along With a Literature Review

A Rare Case of Intravascular Large B-cell Lymphoma Presenting With Bilateral Ophthalmoplegia, Along With a Literature Review

Intravascular Lymphoma: the great imitator – a case report

Linfoma intravascular de células B grandes con afección cerebral

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