Post-Transplant Lymphoproliferative Disorders in Bone Marrow

Post-Transplant Lymphoproliferative Disorders in Bone Marrow

Mohammad A. Vasef, MD

Wright-stained peripheral blood smear of an adult male with a history of liver transplantation demonstrates a rare large circulating atypical lymphoid cell with fine cytoplasmic vacuoles.

H&E stained bone marrow core biopsy shows large aggregates of monotonous lymphoid cells consistent with a monomorphic post-transplant lymphoproliferative disorder.



  • Post-transplant lymphoproliferative disorders (PTLD)


  • Lymphoid proliferation secondary to immunosuppression in setting of allogeneic hematopoietic stem cell transplantation (HSCT) or solid organ transplantation (SOT)


Risk Factors of PTLD in SOT

  • Epstein-Barr virus (EBV) serology status

    • 20-50x higher risk for PTLD development in seronegative recipient from seropositive donor

    • Significantly higher risk in children due to frequent EBV-naive pretransplant status

    • Primary EBV infection in transplant children is commonly acquired from donor organ

    • Lower risk in adults due to EBV reactivation rather than primary EBV infection

  • Type of organ transplanted

    • Lowest risk in renal allograft recipients

    • Highest risk in intestinal recipients

    • High rate of PTLD in intestinal transplants may be due to large quantity of lymphocytes in intestine allograft

  • Type and intensity of immunosuppressive regimen

    • Higher risk in individuals receiving anti-CD3 or anti-thymocyte globulin (ATG)

    • Significant increased risk in individuals receiving high-intensity immunosuppression

  • Age of patient

    • Significantly higher risk of PTLD in pediatric organ recipients compared to adults

    • Higher risk of PTLD in children is attributable to primary EBV infection after transplantation

Risk Factors of PTLD in Allogeneic HSCT

  • Overall much lower risk in hematopoietic stem cell recipients compared to solid organ recipients

  • Majority of PTLD cases occur < 1 year after transplantation

  • Increased risk of early-onset PTLD with unrelated or HLA mismatched related donors

  • Increased risk of PTLD with ATG therapy for graft-vs.-host disease prophylaxis

  • Increased risk of PTLD with T-cell depleted donor marrow transplantation



  • Incidence

    • Significant variability in incidence ranging from < 1% to > 20%

    • < 1% in renal transplant patients

    • 2-3% in heart or liver transplant patients

    • 5-10% in lung or heart-lung recipients

    • 20% in intestinal transplant recipients

    • 1% in hematopoietic stem cell recipients


  • Involvement of nodal and extranodal sites common in PTLD in all types of allograft transplantation

  • Gastrointestinal tract, lungs, and liver are common extranodal sites of involvement by PTLD

  • Central nervous system involvement by PTLD is rare

  • PTLD frequently involves allograft in solid organ transplantation except in heart allograft

  • Tonsils and adenoids are commonly involved sites in early PTLD lesions

  • Overt bone marrow involvement is uncommon

  • Peripheral blood involvement by PTLD is rare

  • Disseminated disease more common in allogeneic bone marrow transplantation


  • Clinical presentation of PTLD highly variable

    • Patients with PTLD may be asymptomatic

    • Some patients present with fever and weight loss

    • PTLD in children may present with features mimicking infectious mononucleosis

    • Tonsillitis &/or lymphadenopathy may be present

  • PTLD can develop at any time post transplantation

    • Early-onset PTLD

      • Develops within 1 year following transplant

    • Late-onset PTLD

      • Develops > 1 year after transplant

      • Accounts for approximately 40% of all PTLD cases

      • PTLD occurs after 5 years post transplantation in < 10% of transplant patients


Jun 13, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Post-Transplant Lymphoproliferative Disorders in Bone Marrow
Premium Wordpress Themes by UFO Themes
%d bloggers like this: