T-cell Posttransplant Lymphoproliferative Disorder Occurring in a Pediatric Solid-organ Transplant Patient

Abstract

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.

Angioimmunoblastic T Cell Lymphoma: An Unusual Presentation of Posttransplant Lymphoproliferative Disorder in a Pediatric Patient

“…In a 19-center study of 1,184 pediatric heart-transplant patients, Webber et al [9] reported 1 case of T cell PTLD out of a total of 56 PTLD cases. In literature reviews published in 2004 and 2006, Lundell et al [5 ]and Yang et al [7 ]identified 14 and 17 reported cases of pediatric T cell PTLD, respectively, including peripheral T cell lymphoma, NOS, hepatosplenic gamma-delta T cell lymphoma, anaplastic large cell lymphoma and T lymphoblastic leukemia/lymphoma.…”

“…Monomorphic T cell PTLD appears to be particularly uncommon in the pediatric population, with few cases reported in the literature [5,7,8,9]. In this report, we present an unusual case of angioimmunoblastic T cell lymphoma (AITL) in an 8-year-old boy after a heart transplant.…”

“…Most cases of monomorphic PTLD are B cell lesions, the majority of which are EBV-positive [4,5]. Monomorphic T cell PTLD is less common, comprising no more than 15% of PTLD cases in Western countries.…”

“…Monomorphic T cell PTLD is less common, comprising no more than 15% of PTLD cases in Western countries. T cell PTLD tends to occur later after transplant, and is EBV-negative in 60-80% of cases [4,5,6]. Reported subtypes of monomorphic T cell PTLD include peripheral T cell lymphoma NOS (not-otherwise-specified), hepatosplenic T cell lymphoma, T lymphoblastic leukemia/lymphoma, anaplastic large cell lymphoma, adult T cell leukemia/lymphoma and large granular lymphocytic leukemia [4,5,6,7].…”

Hematopoietic stem-cell transplantation following solid-organ transplantation in children

“…20,21,22 Only one instance of successful treatment of pediatric T-cell PTLD/lymphoma has been described in the literature. In that case due to the refractory nature of the lymphoma, consolidation therapy with autologous HSCT was performed.…”