Unmet needs of patients with intravascular large B-cell lymphoma: three case reports and a literature review.

in Journal of Zhejiang University. Science. B by Xian Li, Ru Luo, Jiaming Xu, Xueli Jin, Weiqin Wang, Xibin Xiao, Wenbin Qian

TLDR

  • IVLBCL is a rare subtype of non-Hodgkin lymphoma classified as an independent subtype of extranodal DLBCL, characterized by neoplastic lymphocyte proliferation within small blood vessels invading specific organs.
  • Understanding IVLBCL's classification, characteristics, and implications for clinical practice and future research can improve diagnosis and treatment options for patients.

Abstract

Intravascular large B-cell lymphoma (IVLBCL), a rare subtype of non-Hodgkin lymphoma, is classified as an independent subtype of extranodal diffuse large B-cell lymphoma (DLBCL) in the 2008 World Health Organization (WHO) Classification (Turner et al., 2010). The 5th edition of the World Health Organization (WHO 2022) classification of hematolymphoid tumors retains this subtype (Alaggio et al., 2022). IVLBCL, which is characterized by neoplastic lymphocyte proliferation within the lumen of small blood vessels, tends to invade organs, such as the nervous system, skin, bone marrow (BM), and lung (D'Angelo et al., 2019; Satoh et al., 2019; Vásquez et al., 2019; Fukami et al., 2020).

Overview

  • The study focuses on Intravascular large B-cell lymphoma (IVLBCL), a rare subtype of non-Hodgkin lymphoma, and its classification as an independent subtype of extranodal diffuse large B-cell lymphoma (DLBCL) according to the 2008 World Health Organization (WHO) Classification.
  • The study further explores IVLBCL's characteristics, including neoplastic lymphocyte proliferation within the lumen of small blood vessels, which tends to invade organs such as the nervous system, skin, bone marrow, and lung.
  • The primary objective of the study is to understand IVLBCL's classification, characteristics, and implications for clinical practice and future research.

Comparative Analysis & Findings

  • IVLBCL is classified as an independent subtype of extranodal DLBCL in the 2008 WHO Classification and retained in the 5th edition of the WHO classification of hematolymphoid tumors (2022).
  • IVLBCL is characterized by neoplastic lymphocyte proliferation within the lumen of small blood vessels, which tends to invade specific organs.
  • Studies have shown that IVLBCL tends to invade organs such as the nervous system, skin, bone marrow, and lung, which are critical to understanding the disease's progression and treatment.

Implications and Future Directions

  • Understanding IVLBCL's classification and characteristics can improve diagnosis and treatment options for patients with this rare subtype of lymphoma.
  • Future research directions could explore novel therapeutic approaches for IVLBCL, given its tendency to invade specific organs and its poor prognosis.
  • Developing diagnostic biomarkers and conducting largescale studies to improve our understanding of IVLBCL's epidemiology and disease progression could also be crucial.
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