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Related Concept Videos

Primary Lymphoid Organs01:16

Primary Lymphoid Organs

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Primary lymphoid organs are pivotal in the formation, development, and maturation of lymphocytes, the white blood cells that serve as the backbone of our immune system. This crucial function underscores their fundamental role in maintaining our overall health and immunity. The two primary lymphoid organs of prime importance are the red bone marrow and the thymus.
The red bone marrow is a soft, spongy tissue nestled in the interior of long bones such as the humerus and femur. It is the site...
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Secondary Lymphoid Organs01:15

Secondary Lymphoid Organs

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Secondary organs, including lymph nodes, the spleen, and mucosa-associated lymphoid tissue (MALT), work harmoniously to protect us from disease and infection.
The spleen is a vital organ in the lymphatic system, nestled in the upper left side of the abdomen. It is composed of two primary regions: the red pulp and the white pulp, each having distinct functions. The red pulp performs a significant role in blood filtration. It efficiently purges the blood of old or damaged red blood cells and...
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Lymphoid Cells and Tissues01:18

Lymphoid Cells and Tissues

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Lymphoid cells and tissues are integral to the immune system, which is crucial in maintaining our body's defense against harmful pathogens. They form the building blocks of lymphoid organs, which include the spleen, thymus, and lymph nodes.
Lymphoid cells consist of various types of immune system cells. These include B and T lymphocytes, which are responsible for producing antibodies and killing infected cells, respectively. Dendritic cells act as messengers between the innate and adaptive...
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Detailed Structure and Function of Lymph Nodes01:23

Detailed Structure and Function of Lymph Nodes

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Lymph nodes are bean-shaped structures that cluster along the lymphatic vessels in the inguinal, axillary, and cervical regions. Each node is divided into compartments by a capsule that extends trabeculae inward.
From a histological perspective, lymph nodes can be split into two main areas: the superficial cortex and the deep medulla. The outer cortex is populated by dendritic cells, macrophages, and B lymphocytes, which are densely packed into follicles. When these B-lymphocytes are presented...
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Lymphatic Vessels and Lymph Transport01:16

Lymphatic Vessels and Lymph Transport

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Lymphatic vessels, known as lymphatics, are crucial in transporting lymph from peripheral tissues to our venous system. This process begins with lymph entering through tiny capillaries that branch through tissues. These capillaries have unique features such as larger diameters, thinner walls, and a distinctive one-way valve system formed by overlapping endothelial cells.
This one-way system allows fluids, solutes, and even pathogens to enter but prevents their return to the intercellular...
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Development of the Lymphatic System01:15

Development of the Lymphatic System

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The development of lymphatic tissues and vessels in embryonic life begins around the fifth week. These structures originate from the mesoderm layer, with lymph sacs emerging from developing veins.
The first lymph sacs to form are the paired jugular lymph sacs located at the junction of the internal jugular and subclavian veins. From these sacs, lymphatic capillary plexuses extend to the thorax, upper limbs, neck, and head, eventually forming lymphatic vessels. Each jugular lymph sac maintains a...
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Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
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Intravascular Lymphoma: A Unique Pattern Underlying a Protean Disease.

Mario Della Mura1, Joana Sorino1, Filippo Emanuele Angiuli1

  • 1Section of Molecular Pathology, Department of Precision and Regenerative Medicine and Ionian Area (DiMePRe-J), University of Bari "Aldo Moro", 70124 Bari, Italy.

Cancers
|July 29, 2025
PubMed
Summary

Intravascular lymphoma (IVL) is a rare, aggressive cancer where lymphoma cells grow in blood vessels. Early diagnosis and tailored treatments, including chemotherapy and novel therapies, are crucial for improving outcomes in this challenging non-Hodgkin lymphoma subtype.

Keywords:
B-cell lymphomaNK/T-cell lymphomaintravascular lymphomaintravascular lymphomatosisnon-Hodgkin lymphoma

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Area of Science:

  • Hematology
  • Oncology
  • Pathology

Background:

  • Intravascular lymphoma (IVL) is a rare, aggressive non-Hodgkin lymphoma (NHL) subtype.
  • Characterized by neoplastic lymphoid cells proliferating within blood vessels, IVLBCL often presents with diverse symptoms, leading to diagnostic delays and poor prognosis.
  • IVLBCL variants include classic, hemophagocytic syndrome-associated (HPS), and cutaneous forms, affecting organs like the CNS, skin, lungs, and endocrine system.

Purpose of the Study:

  • To provide a comprehensive review of the clinicopathological, molecular, and therapeutic landscape of intravascular lymphoma (IVL).
  • To emphasize the need for increased clinical awareness, standardized diagnostic protocols, and individualized treatment strategies for IVL.

Main Methods:

  • Histopathologic identification of neoplastic intravascular lymphoid cells via tissue biopsies.
  • Analysis of tumor cell immunophenotype (CD20, MUM1, BCL2, MYC) and molecular mutations (MYD88, CD79B).
  • Review of current and emerging therapeutic approaches, including chemotherapy, CNS-directed therapy, ASCT, and immunotherapy.

Main Results:

  • IVLBCL cells exhibit a non-GCB B-cell phenotype with frequent MYD88 and CD79B mutations, similar to ABC-type DLBCL.
  • Treatment typically involves rituximab-based chemotherapy (R-CHOP) and CNS-directed therapy due to neurotropism.
  • Emerging strategies include ASCT and immunotherapies targeting PD-L1.

Conclusions:

  • Intravascular lymphoma (IVL) requires heightened clinical suspicion due to its elusive presentation and aggressive nature.
  • Standardized diagnostic approaches and personalized treatment strategies are essential for managing IVL.
  • Further research into novel therapies is warranted for this challenging malignancy.