T-cell posttransplant lymphoproliferative disorder occurring in a pediatric solid-organ transplant patient.
Posttransplantation lymphoproliferative disorder (PTLD) is a serious complication seen in transplant patients as a consequence of immunosuppressant therapy. Most cases are of B-cell origin and are commonly associated with Epstein-Barr virus (EBV) infection. T-cell PTLDs are rare and only 13 pediatric T-cell PTLDs with clinicopathologic correlation have been reported previously. We present the histologic, immunophenotypic, and molecular features of a monomorphic PTLD (T-cell lymphoma) identified in a pediatric patient following orthotopic liver transplantation. The lymphoma was identified in the ileum, rectum, and mesenteric lymph nodes. In situ hybridization revealed numerous EBER-1-positive tumor cells. A current review of the literature is also discussed. Of the 14 cases of pediatric T-cell PTLD reported in the literature, only 3 (21.4%) are described as being EBV positive. Most of the reported PTLDs are monoclonal, with 9 of 11 cases (82%) showing a clonal T-cell population by gene rearrangement studies. T-cell PTLD cases appear to have a poor prognosis (11 of 14 patients died of the disease), although patients with involvement of specific anatomic sites may have a better outcome.
Lundell
Ryan
R
Department of Pathology, University of Utah, Salt Lake City, UT, USA.
Elenitoba-Johnson
Kojo S J
KS
Lim
Megan S
MS
D000293:Adolescent; D005260:Female; D005434:Flow Cytometry; D006801:Humans; D017403:In Situ Hybridization; D015458:Leukemia, T-Cell / Q000209:etiology* / Q000473:pathology; D016031:Liver Transplantation; D008232:Lymphoproliferative Disorders / Q000209:etiology*; D011183:Postoperative Complications