Management of EBV+PTLD

Monitoring and clinical presentation

Vigilance for signs of EBV⁺PTLD is important

Although there are no widely adopted guidelines, routine monitoring of EBV reactivation post-transplant is widely available.^1,2

Protocols and the distribution of EBV polymerase-chain-reaction (PCR) values for viral load vary between patient cases and centers, but a high or rapidly increasing viral load is associated with an increased risk of PTLD.^1,2

Transplant recipients with PTLD have a significantly higher viral load than recipients without PTLD, and a higher or rapidly increasing viral load is associated with an increased risk of PTLD.¹

An early determination of risk, close observation of EBV DNA levels, and prompt initiation of therapy are essential to improving patients’ overall prognosis.³

Clinical presentation of EBV⁺PTLD:

Classic B symptoms such as pyrexia, sweats, and weight loss can occur. Clinical features of PTLD are often nonspecific, while extranodal involvement is common, including the gastrointestinal tract, lungs, skin, bone marrow, and central nervous system.⁴

Unmet needs in EBV⁺PTLD

There are no EMA- and FDA-approved treatments for EBV⁺PTLD. Reduction in Immunosuppression (RIS) is the cornerstone of initial management of EBV⁺PTLD.¹

High mortality rates have been reported in HCT patients with EBV⁺PTLD. In patients with EBV⁺PTLD following SOT, reduction of immunosuppression may lead to transplant rejection and organ loss.^1,2

1-year overall survival rates in patients treated with anti-CD20 therapy

28%

of HCT patients who developed EBV⁺PTLD were alive at one year in a retrospective study analyzing possible risk factors associated with the development of PTLD⁵

36%

of high risk* SOT patients who developed PTLD were alive at one year in a prospective study evaluating single-agent, first line anti-CD20 therapy⁶

*Prognostic Risk Factors: Patient age, Performance status, Serum LDH

Management of EBV⁺PTLD is particularly challenging due to variability in patient response to treatment, lack of a global standard treatment protocol, and limited therapy options. Additional treatment options may provide a future benefit.¹

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References

1. Dierickx D, Habermann TM. Post-transplantation lymphoproliferative disorders in adults. N Engl J Med. 2018;378:549-562. 2. Fox CP, Burns D, Parker AN, et al. EBV-associated post-transplant lymphoproliferative disorder following in vivo T-cell-depleted allogeneic transplantation: clinical features, viral load correlates and prognostic factors in the rituximab era. Bone Marrow Transplant. 2014;49:280-286. 3. Jiménez S. Epstein-Barr virus-associated post-transplantation lymphoproliferative disorder: potential treatments and implications for nursing practice. Clin J Oncol Nurs. 2015;19:94-98. 4. Al-Mansour Z, Nelson BP, Evens AM. Post-transplant lymphoproliferative disease (PTLD): risk factors, diagnosis, and current treatment strategies. Curr Hematol Malig Rep. 2013;8:173-183. 5. Uhlin M, Wikell H, Sundin M, et al. Risk factors for Epstein-Barr virus-related post-transplant lymphoproliferative disease after allogeneic hematopoietic stem cell transplantation. Haematologica. 2014;99:346-352. 6. Choquet S, Oertel S, LeBlond V, et al. Rituximab in the management of post-transplantation lymphoproliferative disorder after solid organ transplantation: proceed with caution. Ann Hematol. 2007;86:599-607.

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Monitoring and clinical presentation

Unmet needs in EBV+PTLD

Unmet needs in EBV⁺PTLD