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Cardiology226 papers

Hematopoietic neoplasm of uncertain behavior

Last edited: 4/14/2026

Overview

EBV-associated lymphoproliferative disorders (LPD) encompass a spectrum of hematological neoplasms characterized by clonal proliferation of EBV-infected lymphocytes, often presenting atypically and posing diagnostic challenges. These disorders can involve various tissues, including gastrointestinal, systemic, and rarely, bursal sites, with notable heterogeneity in clinical presentation 1258.

Diagnosis

  • Clinical Presentation: Atypical manifestations such as intestinal ulcers, persistent fever, cough, or multifocal involvement 15.
  • Imaging and Biopsy: Essential for tissue diagnosis; often requires histopathological examination and immunohistochemistry to identify EBV markers (e.g., EBNA2, LMP1) 158.
  • Molecular Analysis: T-cell receptor gene rearrangement studies can confirm clonality 6.
  • EBV Serology: Useful but not definitive; often positive in chronic EBV infection 5.
  • Management

  • Immunosuppressive Therapy: Anti-thymocyte globulin (ATG) may precipitate LPD in immunocompromised states; careful monitoring required 6.
  • Antiviral Agents: No specific antiviral treatment universally recommended; supportive care often necessary 5.
  • Steroids and Immunomodulators: Used symptomatically for inflammation and immune modulation 6.
  • Surgical Intervention: Considered for localized aggressive lesions, such as prepatellar bursa involvement 8.
  • Special Populations

  • Pediatrics: EBV-associated systemic T-cell LPD is more frequent and severe in children 25.
  • Elderly: Increased risk of senile EBV-associated B-cell LPD, often in association with other conditions like gout 8.
  • Comorbidities: Bone marrow failure syndromes, such as T-cell large granular lymphocytic leukemia, may predispose to LPD development 6.
  • Key Recommendations

  • Comprehensive Diagnostic Workup: Include histopathological examination with immunohistochemistry for EBV markers and molecular studies to confirm clonality (Evidence: Moderate 16).
  • Close Monitoring in Immunocompromised Patients: Particularly those receiving immunosuppressive therapy, due to increased risk of LPD development (Evidence: Moderate 6).
  • Tailored Management Based on Presentation: Use supportive care, corticosteroids, and surgical intervention as needed, depending on the extent and location of the disorder (Evidence: Expert opinion 58).
  • References

    1 Wang S, Dai Y, Zhang J, Ou D, Ouyang C, Lu F. Intestinal ulcers as an initial finding in EBV-associated lymphoproliferative disorder: A case report. Medicine 2020. link 2 Keow JY, Stecho WM, Haig AR, Sangle NA. EBV-positive T/NK-associated lymphoproliferative disorders of childhood: A complete autopsy report. Indian journal of pathology & microbiology 2020. link 3 Baker FX, Merz JF. What gives them the right? Legal privilege and waivers of consent for research. Clinical trials (London, England) 2018. link 4 Barnard M. How to apply for research ethics committee approval. Nursing children and young people 2016. link 5 Ameli F, Ghafourian F, Masir N. Systematic Epstein-Barr virus-positive T-cell lymphoproliferative disease presenting as a persistent fever and cough: a case report. Journal of medical case reports 2014. link 6 Sumi M, Watanabe M, Sato K, Shimizu I, Ueki T, Akahane D et al.. Epstein-Barr virus-associated lymphoproliferative disorder developed after anti-thymocyte globulin therapy in a patient with bone marrow failure associated with T-cell large granular lymphocytic leukemia. [Rinsho ketsueki] The Japanese journal of clinical hematology 2011. link 7 Tornetta P, Siegel J, McKay P, Bhandari M. Authorship and ethical considerations in the conduct of observational studies. The Journal of bone and joint surgery. American volume 2009. link 8 Mueller S, Aigner T, Haag J, Schwarz R, Niedobitek G. Senile EBV-associated B-cell lymphoproliferative disorder of prepatellar bursa in an elderly patient with multifocal urate arthropathy. Hematological oncology 2007. link 9 Quesada P, Navarrete ML, Perelló E. Eosinophilic granuloma of the temporal bone. European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery 1990. link 10 Beckstead JH. An approach to practical problems in the diagnosis of lymphoproliferative disorders using cytochemistry and immunocytochemistry. Clinics in laboratory medicine 1988. link 11 Morley TF, Silverstein SD, Giudice JC, Csere RS. Multifocal eosinophilic granuloma. Respiration; international review of thoracic diseases 1988. link 12 Jothy S, Boelcskei Z. Immunohistochemical demonstration of T-lymphocyte subsets in tissue sections using monoclonal antibodies: a 3-layer PAP system and comparison of three immunoperoxidase methods. Experimental pathology 1985. link80035-9)

    Original source

    1. [1]
    2. [2]
      EBV-positive T/NK-associated lymphoproliferative disorders of childhood: A complete autopsy report.Keow JY, Stecho WM, Haig AR, Sangle NA Indian journal of pathology & microbiology (2020)
    3. [3]
      What gives them the right? Legal privilege and waivers of consent for research.Baker FX, Merz JF Clinical trials (London, England) (2018)
    4. [4]
      How to apply for research ethics committee approval.Barnard M Nursing children and young people (2016)
    5. [5]
    6. [6]
      Epstein-Barr virus-associated lymphoproliferative disorder developed after anti-thymocyte globulin therapy in a patient with bone marrow failure associated with T-cell large granular lymphocytic leukemia.Sumi M, Watanabe M, Sato K, Shimizu I, Ueki T, Akahane D et al. [Rinsho ketsueki] The Japanese journal of clinical hematology (2011)
    7. [7]
      Authorship and ethical considerations in the conduct of observational studies.Tornetta P, Siegel J, McKay P, Bhandari M The Journal of bone and joint surgery. American volume (2009)
    8. [8]
    9. [9]
      Eosinophilic granuloma of the temporal bone.Quesada P, Navarrete ML, Perelló E European archives of oto-rhino-laryngology : official journal of the European Federation of Oto-Rhino-Laryngological Societies (EUFOS) : affiliated with the German Society for Oto-Rhino-Laryngology - Head and Neck Surgery (1990)
    10. [10]
    11. [11]
      Multifocal eosinophilic granuloma.Morley TF, Silverstein SD, Giudice JC, Csere RS Respiration; international review of thoracic diseases (1988)
    12. [12]

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