Intravascular large B-cell lymphoma as a cause of terminal acute respiratory distress syndrome: atypical presentation of a rare disease

Lazzari, Ilaria; Galetti, C.; Corvalli, Giulia; Bernardi, R; Gianotti, Giordano Cabral; Sagramoso, Carlo; Calogero, Pietro

doi:10.1007/s40520-017-0754-3

Cited by 1 publication

(2 citation statements)

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“…IVIBCL, together with primary effusion lymphoma and large B cell lymphoma of the mediastinum, was classified as a subtype of DLBCL (2,3). According to the new classification standard (2), IVLBCL belongs to extranodal lymphoma, which is characterized by diffuse and obliterative proliferation of lymphoma cells in tissues, organs and lumens of small and medium vessels (terminal arteries, veins, capillaries and blood sinusoid), and involvement of different extranodal organs and tissues, including the central nervous system, skin, lung, kidney, adrenal gland and bone marrow (2)(3)(4)(5). It does not involve lymph nodes and peripheral blood, and has several clinical manifestations, including fever, large liver and spleen, hemocytopenia, disseminated intravascular coagulation and organ damage (2)(3)(4)(5).…”

Section: Introductionmentioning

confidence: 99%

“…The reported incidence rate of men and women worldwide is ~1:1, the median age of onset is ~60 years and the average survival time is ~6-9 months (1-5). Most patients die within 1 year, and it is a malignant tumor with poor prognosis (1)(2)(3)(4)(5). IVL is a highly invasive and extremely rare malignant hematological disease, with poor prognosis and a lack of specificity in the clinical setting (1,2,4).…”

Section: Introductionmentioning

confidence: 99%

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Analysis of clinicopathological features and prognostic factors of non‑Hodgkin's intravascular large B‑cell lymphoma

Liu

Zhou

et al. 2020

Oncol Lett

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Non-Hodgkin's intravascular large B-cell lymphoma is a highly invasive extranodal lymphoma. The proliferating tumor cells invade the small vessels and capillaries of different organs. The clinical symptoms are atypical, there is lack of specificity, and the molecular and biological behaviors are not clear, thus, the present study aimed to improve the current understanding of non-Hodgkin's intravascular large B-cell lymphoma (IVL) and provide an accurate basis for clinical treatment and prognosis, by retrospectively analyzing and summarizing the clinicopathological features, immunohistochemical findings and molecular characteristics of 17 patients with IVL. The Kaplan-Meier method and log rank test were implemented to determine survival outcomes. Fisher's exact test was used to determine the association between clinicopathological features and the expression levels of Ki-67, c-Myc, B-cell lymphoma 6 (Bcl-6) and B-cell lymphoma 2 (Bcl-2), while multivariate Cox regression analysis was performed to identify the independent risk factors that affect the survival rates of patients with IVL. P<0.05 was considered to indicate a statistically significant difference. Among the 17 patients with IVL, 13 cases (76.47%) occurred in the adrenal gland and four cases (23.53%) occurred on the skin demonstrated positive IgH gene rearrangement. FISH analysis indicated that cleavage of the c-Myc gene was closely associated with sex, hypertension status and tumor size, while cleavage of the Bcl-6 gene was closely associated with tumor size parameters. Overall, the results suggest that the Ki-67 proliferation index is an independent risk factor for the prognosis (survival time) of patients with IVL.

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