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Am J Transplant. 2019 Oct;19(10):2764-2774. doi: 10.1111/ajt.15358. Epub 2019 Apr 22.

Posttransplant lymphoproliferative disorder in pediatric patients: Survival rates according to primary sites of occurrence and a proposed clinical categorization.

L'Huillier AG1,2, Dipchand AI2,3,4, Ng VL2,3,5, Hebert D2,3,6, Avitzur Y2,3,5, Solomon M2,3,7, Ngan BY2,8, Stephens D2,9, Punnett AS2,10, Barton M1,3,11, Allen UD1,2,3,9.

Author information

1
Division of Infectious Diseases, Hospital for Sick Children, Toronto, Ontario, Canada.
2
University of Toronto, Toronto, Ontario, Canada.
3
Transplant and Regenerative Medicine Centre, Hospital for Sick Children, Toronto, Ontario, Canada.
4
Labatt Family Heart Centre, Hospital for Sick Children, Toronto, Ontario, Canada.
5
Division of Pediatric Gastroenterology, Hepatology and Nutrition, Hospital for Sick Children, Toronto, Ontario, Canada.
6
Division of Nephrology, Hospital for Sick Children, Toronto, Ontario, Canada.
7
Division of Respiratory Medicine, Hospital for Sick Children, Toronto, Ontario, Canada.
8
Division of Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
9
The Research Institute, Hospital for Sick Children, Toronto, Ontario.
10
Division of Haematology/Oncology, Hospital for Sick Children, Toronto, Ontario, Canada.
11
Division of Infectious Diseases, London Health Sciences Centre, University of Western Ontario, London, Ontario, Canada.

Abstract

Posttransplant lymphoproliferative disorder (PTLD) is a devastating complication of organ transplant. In a hospital-based registry, we identified biopsy-proven cases of PTLD among children during a 15-year period and reviewed trends in PTLD rates, the sites of involvement, and the associated survival rates. Cases that were included had at least 1 year of follow-up after the diagnosis of PTLD. We studied 82 patients with first-episode PTLD. Median age at diagnosis was 6.4 years (IQR 3.2-12.3 years). The most frequent PTLD sites were tonsillar/adenoidal (T/A [34%]) and gastrointestinal (32%), followed by miscellaneous (defined as less common sites including central nervous system, kidney, lung, and soft tissue [12%]), lymph node (11%), and multisite (11%). Kaplan-Meier survival curves showed that T/A PTLD was associated with decreased all-cause mortality compared with PTLD at other sites (log-rank 0.004), even after adjustment for histological subtype (P = .047). PTLD-related mortality was also decreased among T/A PTLD (log-rank 0.012) but showed a trend toward significance only after adjustment for histological subtype (P = .09). Among first episodes of PTLD, T/A PTLD was associated with a survival advantage compared with PTLD at other sites, even after adjustment for potential confounders. Based on our observations, we propose a clinical categorization of PTLD according to anatomical site of occurrence.

KEYWORDS:

Epstein-Barr Virus (EBV); cancer/malignancy/neoplasia; classification systems; clinical research/practice; hematogenous/leukemia/lymphoma; hematology/oncology; infection and infectious agents - viral; infectious disease; patient survival; pediatrics; posttransplant lymphoproliferative disorder (PTLD)

PMID:
30884098
DOI:
10.1111/ajt.15358

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