title: EBV-associated post-transplant lymphoproliferative disorder after umbilical cord blood transplantation in adults with hematological diseases.
abstract: We analyzed the incidence, clinicopathological features, risk factors and prognosis of patients with EBV-associated post-transplant lymphoproliferative disorder (EBV-PTLD) in 288 adults undergoing umbilical cord blood transplantation (UCBT) at a single institution. Twelve patients developed proven EBV-PTLD at a median time of 73 days (range, 36-812). Three-year cumulative incidence (CI) of EBV-PTLD was 4.3% (95% CI: 1.9-6.7). All patients presented with extranodal involvement. Most frequently affected sites were the liver, spleen, central nervous system (CNS), Waldeyer's ring and BM in 7, 6, 4, 3 and 3 patients, respectively. One patient had polymorphic and 11 had monomorphic EBV-PTLD (7 diffuse large B-cell lymphomas not otherwise specified, 4 plasmablastic lymphomas). We confirmed donor origin and EBV infection in all histological samples. EBV-PTLD was the cause of death in 11 patients at a median time of 23 days (range, 1-84). The 3-year CI of EBV-PTLD was 12.9% (95% CI: 3.2-22.5) and 2.6% (95% CI: 0.5-4.7) for patients receiving reduced-intensity conditioning (RIC) and myeloablative conditioning, respectively (P<0.0001). In conclusion, adults with EBV-PTLD after UCBT showed frequent visceral and CNS involvement. The prognosis was poor despite routine viral monitoring and early intervention. An increased risk of EBV-PTLD was noted among recipients of RIC regimens.
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: J
authors.initials: J
authors.lastname: Sanz
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Arango
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: L
authors.initials: L
authors.lastname: Senent
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Jarque
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: P
authors.initials: P
authors.lastname: Montesinos
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: A
authors.initials: A
authors.lastname: Sempere
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: I
authors.initials: I
authors.lastname: Lorenzo
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: G
authors.initials: G
authors.lastname: Martín
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: F
authors.initials: F
authors.lastname: Moscardó
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: E
authors.initials: E
authors.lastname: Mayordomo
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: M
authors.initials: M
authors.lastname: Salavert
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Cañigral
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: B
authors.initials: B
authors.lastname: Boluda
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: C
authors.initials: C
authors.lastname: Salazar
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: J L
authors.initials: JL
authors.lastname: López-Hontangas
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: M A
authors.initials: MA
authors.lastname: Sanz
authors.affiliation: Servicio de Hematología, Hospital Universitari i Politècnic La Fe, Valencia, Spain.
authors.forename: G F
authors.initials: GF
authors.lastname: Sanz
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D002490:Central Nervous System / Q000473:pathology; D036101:Cord Blood Stem Cell Transplantation / Q000009:adverse effects*; D020031:Epstein-Barr Virus Infections / Q000150:complications*; D005260:Female; D006086:Graft vs Host Disease; D006402:Hematologic Diseases / Q000503:physiopathology* / Q000628:therapy*; D004854:Herpesvirus 4, Human; D006801:Humans; D015994:Incidence; D008099:Liver / Q000473:pathology; D008232:Lymphoproliferative Disorders / Q000209:etiology / Q000821:virology*; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012189:Retrospective Studies; D012307:Risk Factors; D013154:Spleen / Q000473:pathology; D013997:Time Factors; D019172:Transplantation Conditioning; D055815:Young Adult