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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.
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The T and B lymphocytes of the adaptive immune system develop from common lymphoid progenitor cells in the bone marrow. These progenitors give rise to precursors that eventually develop into both T and B lymphocytes. As these precursors mature, they gain the ability to detect and respond to foreign antigens in the body, a process known as immunocompetence. Additionally, these precursors acquire self-tolerance, a process that ensures they do not react to self-antigens. This intricate system...
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Bone marrow transplant is a potential cure for several diseases, including cancer and specific genetic disorders. Notably, this procedure is applicable for patients suffering from aplastic anemia, certain types of leukemia, severe combined immunodeficiency disease (SCID), Hodgkin's disease, non-Hodgkin's lymphoma, multiple myeloma, thalassemia, sickle-cell disease, and certain cancers.
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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.
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Leukocyte disorders can lead to either leukopenia, characterized by an abnormally low leukocyte count, or leukocytosis, marked by a very high leukocyte number.
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Tumor Engraftment in a Xenograft Mouse Model of Human Mantle Cell Lymphoma
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Aggressive B-cell lymphomas with a primary bone marrow presentation.

Alberto Zamò1,2, Peter Johnston3, Ayoma D Attygalle4

  • 1Department of Oncology, University of Turin, Turin, Italy.

Histopathology
|April 24, 2020
PubMed
Summary
This summary is machine-generated.

Aggressive B-cell lymphomas primarily in the bone marrow (BM) are challenging to diagnose. This review clarifies diagnostic issues and suggests work-up strategies for these challenging hematologic malignancies.

Keywords:
EAHP/SH bone marrow workshopaggressive B-cell lymphomabone marrow biopsybone marrow-spleen-liver large B-cell lymphomadiffuse large B-cell lymphomahigh-grade B-cell lymphomaintravascular large B-cell lymphomaprimary bone marrow presentation

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Area of Science:

  • Hematopathology
  • Oncology
  • Molecular diagnostics

Background:

  • Aggressive B-cell lymphomas are a diverse group of cancers.
  • Primary diagnosis in the bone marrow (BM) presents diagnostic and clinical challenges due to limited architectural data.
  • Understanding entities primarily presenting in the BM is crucial for accurate classification and management.

Purpose of the Study:

  • To review aggressive B-cell lymphomas with a primary bone marrow presentation.
  • To highlight diagnostic challenges, grey zones, and unusual manifestations.
  • To provide recommendations for immunophenotypic and genetic work-up.

Main Methods:

  • Review of cases presented at the 2018 European Association of Haematopathology/Society of Hematopathology BM workshop.
  • Analysis of diagnostic difficulties and clinicopathological overlaps.
  • Synthesis of current understanding and proposed diagnostic strategies.

Main Results:

  • Identified challenges in classifying Burkitt lymphoma (BL), high-grade B-cell lymphomas (HG-BCLs), and diffuse large B-cell lymphomas (DLBCLs) with primary BM presentation.
  • Highlighted diagnostic grey zones, including cases with MYC/bcl-2/bcl-6 translocations and TdT expression.
  • Discussed clinicopathological overlaps between various DLBCL subtypes and intravascular large B-cell lymphoma.

Conclusions:

  • Accurate diagnosis of aggressive B-cell lymphomas in the bone marrow requires careful consideration of morphological, immunophenotypic, and genetic features.
  • Further refinement of diagnostic criteria is needed for challenging cases.
  • Standardized work-up protocols are essential for consistent classification and improved patient care.