Chest
Volume 124, Issue 4, October 2003, Pages 1242-1249
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Clinical Investigations
Posttransplant Lymphoproliferative Disorder: Incidence, Presentation, and Response to Treatment in Lung Transplant Recipients

https://doi.org/10.1378/chest.124.4.1242Get rights and content

Introduction

Posttransplant lymphoproliferative disorder (PTLD) is a relatively infrequent but devastating complication that occurs after solid-organ transplantation. Although the optimal treatment for this condition is unknown, rituximab, a murine/human chimeric monoclonal antibody, has shown promise in the treatment of PTLD. In this report, we define the incidence, clinical features at presentation, and response to treatment of all cases of PTLD observed at our institution over a 10-year period, including four patients who received treatment with rituximab.

Methods

A review of all patients who underwent lung or heart-lung transplant at Duke University from 1992 to 2002 was performed (n = 400), and demographic and clinical outcome data were extracted.

Results

PTLD was observed in 10 of 400 patients (2.5%). Patients who acquired PTLD were predominately > 55 years old (8 of 10 patients) and with a native disease of COPD (7 of 10 patients). Diagnosis of PTLD was made a median of 343 days after transplant. The type of transplant and Epstein-Barr virus (EBV) status prior to transplant did not appear to influence the risk for PTLD. Patients presented with thoracic organ involvement (7 of 10 patients), extrapulmonary disease (2 of 10 patients), or both (1 of 10 patients). Histologic subtypes included polymorphic B cell (n = 4), monomorphic B cell (n = 3), B cell without further classification (n = 2), and anaplastic T cell (n = 1). Only one patient responded to reduced immunosuppression alone. Patients treated with surgery or radiation (n = 2) or rituximab (n = 4) had favorable responses to therapy. Both patients treated with chemotherapy died related to complications of treatment and PTLD.

Conclusions

Presentation and histologic appearance of PTLD varies considerably among lung transplant recipients. PTLD was more frequent among older patients with COPD, regardless of pretransplant EBV serology. Rituximab appears effective as a first-line therapy for PTLD, but additional studies are needed in order to define its efficacy and side effect profile in this population of patients.

Section snippets

Materials and Methods

We performed a retrospective review of all lung and heart-lung transplant recipients with a diagnosis of PTLD since the inception of our lung transplant program in 1992. Of a total of 400 lung or heart-lung transplant recipients who underwent transplantation from 1992 until July 1, 2002, 10 patients received a diagnosis of PTLD. The demographic information for these patients is shown in Table 1; the data were obtained by medical record review.

Patients who acquired PTLD received postoperative

Results

The most common indications for transplant in our population were as follows: COPD (50%), cystic fibrosis (25%), pulmonary fibrosis (14%), and sarcoidosis (5%). Demographic information, treatment regimens and outcomes are shown in Table 2. The mean (± SD) age of our patients with PTLD was 53.8 ± 15.4 years, and 80% were > 55 years old. In contrast, only 39% (152 of 390 transplant recipients) without PTLD were > 55 years old (p = 0.02, Fisher exact test). There was a trend toward more

Discussion

Our results demonstrate a low incidence of PTLD in a large cohort of lung transplant recipients. Patients with PTLD tended to be > 55 years old and with a native disease of COPD; PTLD in our population appeared unrelated to pretransplant EBV status or immunosuppressive regimen. Presentation with single or multiple nodules or masses in the thorax was most common, although extrapulmonary disease was seen in a few patients. Most patients had histologic evidence of monomorphic or polymorphic B-cell

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