Case Report: Intravascular Large B-Cell Lymphoma: A Clinicopathologic Study of Four Cases With Review of Additional 331 Cases in the Literature

Abstract

Intravascular large B-cell lymphoma (IVLBCL) is a rare and highly malignant non-Hodgkin B-cell lymphoma with uncommon clinical presentation and poor prognosis. The diagnostic pitfall of IVLBCL is mainly due to the fact that subtle histological changes could be easily overlooked, in addition to its rare occurrence, non-specific and variable clinical presentations, and the absence of significant mass lesions. The purpose of this study is to further explore the clinicopathologic and molecular features of IVLBCL to ensure an accurate diagnosis of this entity. Here, we retrospectively present the data of the four new cases and the literature cases. The age ranged from 23 to 92, with a medium age of 67 and a male-to-female ratio of 1:1. The clinical manifestations are extremely variable, including fever, night sweats, weight loss, anemia, thrombocytopenia, unexplained hypoxemia, impaired consciousness, and skin lesions, as well as the extremely low levels of serum albumin, high levels of serum lactate dehydrogenase (LDH), soluble interleukin-2 receptor (sIL2R), and ferritin. Morphologically, 99.9% of cases showed a selective growth pattern with large, atypical lymphocytes within the lumen of small blood vessels. In addition, vast majority of cases were positive for CD20, CD79a, PAX5, MUM1, and BCL6, and a subset of cases expressed BCL2 and CD5, whereas CD3 and CD10 were typically negative. Ki-67 proliferative index ranged from 20% to 100%. To sum up, we have conducted comprehensive case reports, to the best of our knowledge, this is the largest reported cohort of IVLBCL cases. Comprehensive assessments and more IVLBCL cases are required for early diagnosis and prompt treatment.

Rapidly progressive dementia due to intravascular lymphoma: A prion disease reference center experience

“…with a more frequent expression of MUM1 and Bcl2, variable Bcl6, and high proliferation index [23,24]. Our results were consistent with this immunophenotype.…”

“…With regard to immunohistochemistry, CD20 is positive in most cases; when negative, another pan B-cell marker is required for the diagnosis. Most cases of IVLBCL have a non-germinal center B-cell immunophenotype [23,24], according to the Hans algorithm [11],…”

Intravascular Large B-Cell Lymphoma Identified by PET/CT

“…6 This is mainly because it selectively encapsulated in the lumen of small blood vessels of various organs including the liver, spleen, lungs, skin, brain, and kidney rather than infiltrating into parenchymal tissue. [7][8][9] The usefulness of random skin or senile hemangioma biopsy for the diagnosis of intravascular large B-cell lymphoma has been proposed. 3-5 18 F-FDG PET/CT, as a noninvasive anatomical and functional imaging modality, is widely used in the diagnosis, staging, and response assessment of lymphoma.…”

“…The clinical manifestations are very heterogeneous and noncharacteristic; therefore, the accurate diagnosis is challenging 6 . This is mainly because it selectively encapsulated in the lumen of small blood vessels of various organs including the liver, spleen, lungs, skin, brain, and kidney rather than infiltrating into parenchymal tissue 7–9 . The usefulness of random skin or senile hemangioma biopsy for the diagnosis of intravascular large B-cell lymphoma has been proposed 3–5 .…”

Intravascular large B-cell lymphoma (IVLBCL): Subtle Presentation and Challenging Diagnosis

“…IVLBCL can present with a wide range of clinical symptoms, such as unexplained fever, night sweats, anemia, weight loss, decreased cognition, and skin lesions. The diagnostic challenge of IVLBCL is mainly due to the fact that subtle histological changes could be easily overlooked, in addition to its rarity, non-speci c and diverse clinical presentations, and lack of large mass lesions (5). The de ning feature of IVLBCL diagnosis is the remarkable degree of sparing of the surrounding tissue along with the absence of tumor cells in the lymph nodes and reticuloendothelial system (6).…”