World Lymphoma Awareness Day – Understanding Post-Transplant Lymphoproliferative Disorders (PTLD)

Join us in recognizing World Lymphoma Awareness Day, 15th September, a day dedicated to raising awareness of lymphomas, which are cancers of the lymphocytes or white blood cells. 

This day sheds light on a type of cancer with more than 80 subtypes and that affects up to 864,000 people world wide^1,2. One of these subtypes is Post-Transplant Lymphoproliferative disorder (PTLD).

PLTD is a rare disease characterized by the development of lymphoid neoplasms after solid organ or hematopoietic stem cell transplantation^3,4. 

As we mark World Lymphoma Awareness Day this September, Pierre Fabre Laboratories is dedicated to shine a light on this rare condition. PTLD is a potentially life-threatening complication that can occur after an organ transplant. The occurrence of PTLD varies from 2% to 20%, depending on the type of transplant⁵.

Our commitment is to educate, support, and inspire action among patients, healthcare professionals, and the broader community to improve understanding and management of PTLD.

Understanding PTLD 

Post-Transplant Lymphoproliferative Disorder (PTLD) is a spectrum of lymphoid proliferations that can occur following hematopoietic (bone marrow) or solid organ transplantation^3,4. Patients who receive a transplant are subjected to a strong immunosuppressive regimen to prevent rejection, which makes individuals susceptible to the disease, resulting in very few cases of PTLD⁵.
PTLD is characterized by proliferation of white blood cells (B lymphocytes) which often are infected by the Epstein Barr virus.⁶

What causes PTLD?

•    Although children who are infected with Epstein Barr Virus (EBV), also called infectious mononucleosis, may not display any symptoms, the virus can lead to the development of mononucleosis in adolescents and adults.⁷
•    It is estimated that 90% of the world-wide adult population has life-long EBV infection.⁷
•    Immunosuppressive therapy can produce the reactivation of EBV-infected B lymphocytes.^8,9
•    In an immunosuppressed patient, T-cells are low in number, and they cannot properly eliminate the virus.¹⁰ This can lead to the rapid proliferation of EBV-infected B-cells, causing PTLD.⁶

Prevalence and Risk of PTLD

PTLD occurs in solid organ transplants and in hematopoietic stem cell transplantation^3,4.

 

 

The risk of developing PTLD depends on the degree of immunosuppression, the EBV serostatus and age.^8,9,13,14

•    The more immunosuppressed the patient is, the greater the risk of developing PTLD.^12,14 The degree of compatibility between recipient and donor is very important, especially in the case of stem cell transplants.^12,14
•    PTLD can occur when the transplanted patient’s own EBV is reactivated or because the EBV from the donor is reactivated in the patient.^8,9
•    Children under 10 years and adults over 50 years (haematopoietic transplant) or 60 years (solid organ transplant) are at higher risk of developing PTLD.^12,14

Symptomatology and diagnosis

Some of the common PTLD symptoms are: ^8,9,14

These symptoms are not unique to PTLD and are similar to the symptomatology associated with organ rejection that can occur after undergoing a transplant.^8,14

Different methods are used to establish a diagnosis of PTLD: ^9,15 

Other complementary test:^9,15

Le Comité Scientifique est composé d'experts de renommée internationale. Il se réunit périodiquement pour évaluer notre stratégie de recherche et de développement de nouveaux actifs thérapeutiques et de partenariats en innovation médicale.

 

 

 

Blood tests

 

 

Imaging tests

(CT Sca, PET scan or MRI)

 

 

 

 Lambar puncture or bone marrow biopsy

 

 

Treatment options

There are different treatments options:

 

 

 

Reduction of the immunosuppression

 

 

Antibody therapy

 

 

 

 Chemotherapy

 

 

 

 

 

Surgery

 

 

Radiotherapy

 

 

 

T-cell therapy

 

 

Know more about PTLD information

Our A Bright Journey campaign

Throughout 2024 and during this World Lymphoma Awareness Month, the Pierre Fabre Group wants to raise awareness about this rare condition and share more information about this disease, to offer better support to those affected by it and trying to improve their quality of life.

Show your support to the #ABrightJourney movement this day by liking, sharing and commenting on our posts.

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REFERENCES

1.    [Internet]. [cited 2024 Aug 26]. Available from: https://www.lymphoma.org.au/lymphoma-types/
2.    [Internet]. [cited 2024 Aug 26]. Available from: https://lymphomacoalition.org/wp-content/uploads/WLAD-2023-Infographic-Signs-and-Symptoms.pdf
3.    Engels EA, et al. Spectrum of cancer risk among US solid organ transplant recipients. JAMA [Internet]. 2011;306(17):1891–901. 
4.    Kinch A, et al. A population-based study of 135 lymphomas after solid organ transplantation: The role of Epstein-Barr virus, hepatitis C and diffuse large B-cell lymphoma subtype in clinical presentation and survival. Acta Oncol [Internet]. 2014;53(5):669–79. A
5.    Samant H, Vaitla P, Kothadia JP. Post-Transplant Lymphoproliferative Disorders. [Updated 2023 Feb 12]. In: Stat Pearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2023 Jan-. 
6.    Martinez OM, Krams SM. The immune response to Epstein Barr virus and implications for posttransplant lymphoproliferative disorder. Transplantation [Internet]. 2017;101(9):2009–16.
7.    Cohen JI. Epstein-Barr virus infection. N Engl J Med [Internet]. 2000;343(7):481–92. 
8.    Loren AW, Porter DL, Stadtmauer EA, Tsai DE. Post-transplant lymphoproliferative disorder: a review. Bone Marrow Transplant [Internet]. 2003;31(3):145–55. 
9.    DeStefano CB, et al. Management of post-transplant lymphoproliferative disorders. Br J Haematol [Internet]. 2018;182(3):330–43. 
10.    Ligeti K, Müller LP, Müller-Tidow C, Weber T. Risk factors, diagnosis, and management of posttransplant lymphoproliferative disorder: improving patient outcomes with a multidisciplinary treatment approach. Transpl Res Risk Manag [Internet]. 2017;9:1–14..
11.    Curtis RE, Travis LB, Rowlings PA, Socié G, Kingma DW, Banks PM, et al. Risk of lymphoproliferative disorders after bone marrow transplantation: a multi-institutional study. Blood. 1999;94(7):2208–16. 
12.    Styczynski J, Gil L, Tridello G, Ljungman P, Donnelly JP, van der Velden W, et al. Response to rituximab-based therapy and risk factor analysis in Epstein Barr Virus-related lymphoproliferative disorder after hematopoietic stem cell transplant in children and adults: a study from the Infectious Diseases Working Party of the European Group for Blood and Marrow Transplantation. Clin Infect Dis [Internet]. 2013;57(6):794–802. 
13.    Al-Mansour Z, Nelson BP, Evens AM. Post-transplant lymphoproliferative disease (PTLD): risk factors, diagnosis, and current treatment strategies. Curr Hematol Malig Rep [Internet]. 2013;8(3):173–83. 
14.    Gulley ML, Tang W. Using Epstein-Barr viral load assays to diagnose, monitor, and prevent posttransplant lymphoproliferative disorder. Clin Microbiol Rev [Internet]. 2010;23(2):350–66. Available from: http://dx.doi.org/10.1128/CMR.00006-09. 
15.    Styczynski J, van der Velden W, Fox CP, Engelhard D, de la Camara R, Cordonnier C, et al. Management of Epstein-Barr Virus infections and post-transplant lymphoproliferative disorders in patients after allogeneic hematopoietic stem cell transplantation: Sixth European Conference on Infections in Leukemia (ECIL-6) guidelines. Haematologica [Internet]. 2016;101(7):803–11. 
16.    Starzl TE, Nalesnik MA, Porter KA, Ho M, Iwatsuki S, Griffith BP, et al. Reversibility of lymphomas and lymphoproliferative lesions developing under cyclosporin-steroid therapy. Lancet [Internet]. 1984;1(8377):583–7. 
17.    Roschewski M, Wilson WH. EBV-associated lymphomas in adults. Best Pract Res Clin Haematol [Internet]. 2012;25(1):75–89. 
18.    Oertel SHK, Verschuuren E, Reinke P, Zeidler K, Papp-Váry M, Babel N, et al. Effect of anti-cd 20 antibody rituximab in patients with post-transplant lymphoproliferative disorder (PTLD). American Journal of Transplantation. 2005;5(12):2901–6. 
19.    Choquet S. Efficacy and safety of Rituximab in B-cell post-transplantation lymphoproliferative disorders: Results of a prospective Multicenter Phase 2 study. Blood.
2006;107(8):3053–7. 
20.    Choquet S, Oertel S, LeBlond V, Riess H, Varoqueaux N, Dörken B, et al. Rituximab in the management of post-transplantation lymphoproliferative disorder after solid organ
transplantation: Proceed with caution. Annals of Hematology. 2007;86(8):599–607.