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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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Germinotropic lymphoproliferative disorder: a systematic review.

Magda Zanelli1, Maurizio Zizzo2,3, Alessandra Bisagni4

  • 1Pathology Unit, Azienda USL-IRCCS di Reggio Emilia, Reggio Emilia, Italy. magda.zanelli@ausl.re.it.

Annals of Hematology
|April 21, 2020
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Summary

Germinotropic lymphoproliferative disorder is a rare condition often co-infected with Human herpesvirus 8 and Epstein-Barr virus. This review clarifies its features, diagnosis, and outcomes, distinguishing it from similar diseases.

Keywords:
DisorderEpstein-Barr virusGerminotropicHuman herpesvirus 8Lymphoproliferative

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Area of Science:

  • Hematology
  • Oncology
  • Virology

Background:

  • Germinotropic lymphoproliferative disorder (GLPD) is a rare entity.
  • It is characterized by co-infection with Human herpesvirus 8 (HHV-8) and Epstein-Barr virus (EBV).
  • GLPD shares HHV-8 co-infection with primary effusion lymphoma (PEL), but differs in clinical presentation and prognosis.

Purpose of the Study:

  • To enhance understanding of GLPD.
  • Focus on epidemiology, clinical presentation, pathology, treatment, and outcomes.
  • Clarify differential diagnosis with other HHV-8-related lymphoproliferative disorders.

Main Methods:

  • Systematic literature search using PRISMA guidelines.
  • Databases searched: PubMed/MEDLINE, Web of Science, Scopus, EMBASE, Cochrane Library.
  • Search terms: "germinotropic" and "lymphoproliferative disorder".

Main Results:

  • Current literature reports only 19 cases of GLPD.
  • GLPD typically presents as lymphadenopathy in immunocompetent individuals.
  • GLPD generally follows an indolent course, unlike the aggressive PEL.

Conclusions:

  • GLPD is a distinct lymphoproliferative disorder with unique clinicopathological features.
  • Further research is needed to fully elucidate GLPD's characteristics and management.
  • Distinguishing GLPD from PEL and other HHV-8-associated conditions is crucial.