Recurrent Cytogenetic Abnormalities in Intravascular Large B-Cell Lymphoma

Klairmont, Matthew M; Cheng, Jinjun; Martin, Mike G.; Gradowski, Joel F.

doi:10.1093/ajcp/aqy023

Cited by 10 publications

(8 citation statements)

References 54 publications

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“…These findings were similar to the study of Klairmont et al. which showed most of IVLBCL patients were associated with complex karyotypes with recurrent cytogenetic abnormalities commonly involving chromosomes 1, 6q, and 18 [ 39 ]. Early mortality was found in two of these patients in our study, but the remaining one was still alive to the date of last follow-up.…”

Section: Discussionsupporting

confidence: 91%

Intravascular Large B-cell lymphoma: A case series and review of literatures

et al. 2021

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Background Intravascular large B-cell lymphoma (IVLBCL) is a rare subtype of non-Hodgkin lymphoma with uncommon clinical presentations and poor prognosis. The purpose of this study is to report the clinical features and outcome of IVLBCL in a single institution of Taiwan. Methods Ten patients with IVLBCL diagnosed from June 2006 to January 2018 were retrospectively reviewed. Results The median age was 61 (range 39–88) years. The most common presentation was fever (90%), cytopenia (90%), and confusion (50%). For all patients, the median progression free survival (PFS) and overall survival (OS) were 12.6 (95% confidence interval [CI] 0.0–76.1) and 18.8 (95% CI 0–59.3) months, respectively. Six patients received rituximab combined chemotherapy, and the other one patient was treated with chemotherapy alone. Six of seven (85.7%) patients achieved complete response after chemotherapy. The median PFS and OS for six patients who completed treatment were not reached. Three-year PFS and OS rates were 80% and 75%, respectively. Conclusion Our study showed that patients might achieve durable remission after rituximab-based chemotherapy. The outcome of IVLBCL patients may further improve if early diagnosis and prompt treatment were made.

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Section: Discussionsupporting

confidence: 91%

Intravascular Large B-cell lymphoma: A case series and review of literatures

et al. 2021

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“…Most cases have a complex karyotype. Klairmont et al 42 reported that chromosome 1 is commonly involved in 72% of cases, followed by chromosomes 3, 6q, 14 and 18. Gene mutation analysis has shown mutations in PIM1 (67%), MYD88 (44-59%), CD79b (26-67%), PRDM1 (44%), BTG2 (22%) and EZH2 (4%).…”

Section: Immunophenotypementioning

confidence: 99%

“…Gene mutation analysis has shown mutations in PIM1 (67%), MYD88 (44-59%), CD79b (26-67%), PRDM1 (44%), BTG2 (22%) and EZH2 (4%). [38][39][40][41][42][43] The immunoglobulin genes are clonally rearranged.…”

Section: Immunophenotypementioning

confidence: 99%

Diffuse large B-cell lymphoma variants: an update

et al. 2020

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“…Immunophenotype, however, has not been shown to affect outcomes [16]. IVLBCL has been associated with complex karyotype and structural aberrations in chromosomes 1, 6q, and 18 in over 50% of IVLBCL cases [19].…”

Section: Discussionmentioning

confidence: 99%

CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature

Gupta

Carreon

Şen

et al. 2018

Case Reports in Pathology

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Intravascular large B-cell lymphoma (IVLBCL) is a rare extra-nodal B-cell lymphoma that proliferates within small/intermediate blood vessels and capillaries while sparing large blood vessels and organ parenchyma. Clinical presentation is highly variable and may include B symptoms, neurological deficits, and/or cutaneous findings. The diagnosis of IVLBCL is difficult due to multiorgan involvement and nonspecific symptoms. We describe the case of a 68-year-old male who presented with progressive weakness, confusion, and falls. He had a past medical history of liver cirrhosis secondary to Wilson's disease. Physical exam and laboratory results revealed a lethargic man with jaundice, hepatic encephalopathy, and abnormal liver/kidney function tests. He expired after a short hospital course in the setting of hepatic and renal failure. Postmortem examination revealed large neoplastic lymphoid cells involving multiple organ blood vessels; however skin and neurologic involvement was absent. The neoplastic cells demonstrated B-cells positive for CD5, rendering a diagnosis of IVLBCL. Our case represents the occurrence of IVLBCL with CD5-positivity in a patient with Wilson's disease, diagnosed at autopsy demonstrating the challenging nature of diagnosing IVLBCL.

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Recurrent Cytogenetic Abnormalities in Intravascular Large B-Cell Lymphoma

Abstract: Recurrent cytogenetic abnormalities involving chromosomes 1, 6q, and 18 are present in greater than 50% of ILBCL.

Cited by 10 publications

References 54 publications

Intravascular Large B-cell lymphoma: A case series and review of literatures

Intravascular Large B-cell lymphoma: A case series and review of literatures

Diffuse large B-cell lymphoma variants: an update

CD5-Positive Intravascular Large B-Cell Lymphoma in a Patient with Wilson’s Disease: Case Report and Review of the Literature

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