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Primary Lymphoid Organs01:16

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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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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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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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Aggressive CD5-Positive Primary Bone Marrow Diffuse Large B-Cell Lymphoma with Leukemic Presentation.

Mark G Evans1, Sherif A Rezk2, Lauren C Pinter-Brown3

  • 1Department of Hematopathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Case Reports in Hematology
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Primary bone marrow diffuse large B-cell lymphoma (DLBCL) is rare and can present with leukemic features and CD5 expression. This case highlights the aggressive clinical course and poor prognosis associated with this rare lymphoma subtype.

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Area of Science:

  • Hematology
  • Oncology
  • Immunology

Background:

  • Primary bone marrow diffuse large B-cell lymphoma (DLBCL) is a rare non-Hodgkin lymphoma.
  • A subset of DLBCL cases exhibit CD5 expression, but its prognostic significance is unclear.

Observation:

  • A 78-year-old male presented with circulating lymphoma cells in peripheral blood and bone marrow involvement.
  • The lymphoma was identified as diffuse large B-cell lymphoma, germinal center B-cell subtype, with CD5 expression.

Findings:

  • The patient achieved a favorable initial response to etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (EPOCH-R) chemotherapy and intrathecal methotrexate.
  • Despite initial treatment, the patient relapsed with lymphadenopathy and ultimately succumbed to the disease approximately one year post-diagnosis.

Implications:

  • This case underscores the aggressive nature of CD5-positive primary bone marrow DLBCL.
  • Further research is warranted to elucidate the prognostic impact of CD5 expression in this rare lymphoma entity.