Pleura
Other tumors
Posttransplant lymphoproliferative disorder

Author: Vaidehi Avadhani, M.D. (see Authors page)

Revised: 27 March 2018, last major update June 2014

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Posttransplant lymphoproliferative disorder pleural

Cite this page: Avadhani, V. Posttransplant lymphoproliferative disorder. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/pleuraptlpd.html. Accessed August 16th, 2018.
Definition / general
  • Lymphoid or plasmacytic lesions that develop due to immunosuppression after solid organ, hematopoietic or stem cell transplant are called PTLD (posttransplant lymphoproliferative disorders)
Epidemiology
  • PTLD involving pleura is very rare (more common in lymph node, lung, liver)
  • Case series of 34 cases (MD Anderson Cancer Center) of lymphomas involving the pleura had 1 case of PTLD (Arch Pathol Lab Med 2006;130:1497)
Etiology
    1. EBV seronegativity before transplantation is considered to be an important risk factor
      • Most PTLD are associated with EBV infection
    2. The stronger the immunosuppressive regimen, the higher the risk
      • Solid organ: heart / lung transplant > cardiac and hepatic > renal
    3. PTLD in solid organ transplants are of host origin but in BM transplants are of donor origin
    4. Patients less than 18 years old at time of transplantation have a 2 - 4 times higher risk of developing PTLD than adult patients
Clinical features
  • Nonspecific; very variable depending on type of allograft
Diagnosis
  • Based on WHO classification:
    • Early lesions are plasmacytic hyperplasia or Infectious mononucleosis (IM)-like PTLD
    • Polymorphic PTLD
    • Monomorphic PTLD
      • B cell neoplasms: diffuse large B cell lymphoma (DLBCL), Burkitt lymphoma, plasma cell myeloma, plasmacytoma-like lesions, other
      • T cell neoplasms: peripheral T cell lymphoma, NOS; hepatosplenic T cell lymphoma; other
    • Classical Hodgkin lymphoma type PTLD
Radiology images

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Right pleural effusion

Case reports
Treatment
  • Reduction of immunosuppression
  • Immunotherapy with CD20 monoclonal antibody rituximab
  • Chemotherapy
  • Radiation therapy
  • Combinations of above
  • Goal of therapy is to eradicate the PTLD and preserve graft function
Gross images

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Tan-white nodule in lobectomy specimen

Microscopic (histologic) description
  • Early lesions - plasmacytic hyperplasia
    • Looks like infectious mononucleosis
    • Architecture of tissue is preserved
  • Polymorphic PTLD:
    • Architecture is effaced
    • Mixed population of plasma cells, immunoblasts, lymphoid cells
  • Monomorphic PTLD:
    • Architecture is effaced
  • Non-Hodgkin lymphoma (other than low grade lymphoma) and myeloma
  • Classical Hodgkin type lymphoma
Microscopic (histologic) images

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Various stains

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CD3, CD20

Positive stains
  • EBV positive in many cases
  • CD20, CD79a, light chain restriction (if lymphoma)
  • Immunostains specific for the particular lymphoma
Negative stains
Differential diagnosis
  • Rejection in allografts (resembles polymorphic PTLD): EBER positive in polymorphic PTLD
Additional references