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.


TERMINOLOGY


Abbreviations



  • Post-transplant lymphoproliferative disorders (PTLD)


Definitions



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


ETIOLOGY/PATHOGENESIS


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


CLINICAL ISSUES


Epidemiology



  • 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


Site



  • 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


Presentation



  • 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


Treatment

Jun 13, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Post-Transplant Lymphoproliferative Disorders in Bone Marrow
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