Epstein-Barr virus-positive post-transplant lymphoproliferative disease: results of a Delphi study in Spain
- 2024, Volume 43 - Number 1
- Published: June 28, 2024
Epstein-Barr virus-positive post-transplant lymphoproliferative disease: results of a Delphi study in Spain
Anna Sureda 1, Pere Barba 2, Marta González 3, Cristina Hesse 4, Mi Kwon 5, Júlia Marsal 6, Alejandro Martín 7, Ma. Jesús Pascual 8, Raquel de Paz 9, Jaime Sanz 10, María Suárez 11, Lucrecia Yañez 12, José A. Pérez 13
1 Hospital Duran i Reynals, Barcelona, Spain; 2 Hospital Universitari Vall d’Hebron, Barcelona, España; 3 University Hospital Niño Jesús, Madrid, Spain; 4 Pierre Fabre, Barcelona, Spain; 5 5Hospital Gregorio Marañon, Madrid, España; 6 Hospital Sant Joan de Déu, Barcelona, Spain; 7 University Hospital of Salamanca, Salamanca, Spain; 8 Hospital Regional of Málaga, Málaga, Spain; 9 University Hospital la Paz, Madrid, Spain; 10 La Fe University and Polytechnic Hospital, Valencia, Spain; 11 Hospital Clínic of Barcelona, Barcelona, Spain; 12 University Hospital Marqués de Valdecilla, Santander, Spain; 13 Unidad de Gestión Clínica de Hematología y Hemoterapia, Hospital Universitario Virgen del Rocío, Sevilla, España
Anna Sureda, Pere Barba, Marta González, Cristina Hesse, Mi Kwon, Júlia Marsal, Alejandro Martín, Ma. Jesús Pascual, Raquel de Paz, Jaime Sanz, María Suárez, Lucrecia Yañez, José A. Pérez
La información completa de afiliaciones y autor de correspondencia está disponible en la versión original en PDF.
*Correspondence: Alejandro Martín, Email not available
Summary
Background: Epstein-Barr virus-positive (EBV+) post-transplant lymphoproliferative disease (PTLD) is a rare, life-threatening condition that affects immunocompromised patients who received hematopoietic stem cell (HSCT) or solid organ (SOT) transplant. However, information regarding EBV+ PTLD patients in Spain is limited. Objective: Determine the burden, patient journey and unmet needs of EBV+ PTLD patients in Spain. Methods: Twelve hematologists with experience managing EBV+ PTLD patients participated in a Delphi study that included a literature review of evidence for EBV+ PTLD in Spain and two consultation rounds. Results: As per the survey results, the estimated incidence of EBV+ PTLD was 1.8% after HSCT and 0.85% after SOT. Among EBV+ patients, 50% after HSCT and 33% after SOT failed first-line treatment, with 8-22 patients being diagnosed with relapsed/refractory EBV+ PTLD after HSCT and 1-25 after SOT each year. The experts agreed that hematologists are primarily responsible for diagnosis, management, treatment, and follow-up. Common treatments include immunosuppression reduction, rituximab, and chemotherapy. After first-line failure, treatment options are limited. The main unmet need is the availability of effective treatment with minimal short- and long-term adverse events. Conclusion: This study provides an accurate representation of the current situation of patients with EBV+ PTLD in Spain.
Keywords: Allogeneic hematopoietic cell transplantation. Delphi methodology. Epstein-Barr virus. Post-transplant lymphoproliferative disease. Solid organ transplantation.
