Lymphoproliferative Disease After Lung and Heart-Lung Transplantation: First Description in Spain P. Morales, J. Torres, D. Pérez-Enguix, A. Solé, A. Pastor, A. Segura, and F. Zurbano ABSTRACT Lymphoproliferative syndromes are the most common tumors in transplant recipients. More than 90% of posttransplantation lymphoproliferative syndromes (PTLS) are con- sidered to be associated with Epstein-Barr virus, and 86% are of the B-cell line. Histopathology ranges from polymorphic-reactive to monomorphic forms. Clonality should be studied using molecular biology techniques. Clinically, a differentiation is usually made between early PTLS (occurring within 1 year after transplantation) and late PTLS, which occur as localized or disseminated nodal lymphomas. In localized forms, immuno- suppression should be discontinued or decreased, and the involved area should be subsequently resected or irradiated. In disseminated cases, immunosuppression should be decreased and administration of acyclovir/ganciclovir should be considered. If this is not effective, treatment should be started with anti-CD20 monoclonal antibodies (rituximab). If no response occurs, use of chemotherapy, possibly with interferon, should be considered. Our aim was to report the incidence, clinical signs, and treatment in a series of patients undergoing lung transplantation (LTx). P ATIENTS with congenital or acquired immunodefi- ciencies of any type have an increased risk of suffering a neoplasm. The gradual increase in the number of trans- plants and, therefore, the number of patients with an iatrogenic acquired immunodeficiency, has resulted in an increased number of tumors. Posttransplantation lympho- proliferative syndromes (PTLS) are the most common neoplasms (21%), followed by epithelial tumors in the skin (8%), and Kaposi sarcoma (6%). 1 Lymphoid neoplasms are difficult to manage in these patients from both the diagnos- tic and therapeutic viewpoints. They represent a serious complication, and their overall mortality rate approaches 80% in some series. 2 The incidence of PTLS in lung transplantation (LTx) in Spain has not been reported to date. We report here our experience with a series of 270 LTx performed since the start of the program—from Feb- ruary 1990 –December 2004. CLINICAL OBSERVATION The total 270 LTx included 5 cases (1.85%) of PTLS (Table 1) in 3 men and 2 women of ages 15–54 years, all of whom received a bilateral lung transplant. The reason for trans- plantation was cystic fibrosis (CF) in 3 cases, and emphy- sema and bronchiectasia in 1 patient each. The immuno- suppressive scheme consisted of cyclosporine (CyA), azathioprine (Aza), and corticosteroids in 4 cases, and tacrolimus, Aza, and corticosteroids in the remaining pa- tient. Aza had to be replaced by mycophenolate because of neutropenia in only 1 case. Prior serologic testing for cytomegalovirus (CMV) and Epstein-Barr virus (EBV) was immunoglobulin (Ig)G–positive for CMV in all cases, and for EBV in 4 cases. All of them received ganciclovir prophylaxis after the surgical procedure. The time from transplantation to the occurrence of PTLS ranged from 3–172 months (mean, 39.8). Clinical presentation forms included the following: painless cervical adenopathy, abdominal pain from an ovarian mass, dysphagia with an esophageal mass, costal mass (Fig 1), and multiple pulmonary images (Fig 2). Histopathological study showed a type B lymphoproliferative syndrome with From the Unidad de Trasplante Pulmonar (P.M., A.S., A.P.), Hospital Universitario La Fe, Valencia; Sección de Neumología (J.T.), Hospital de Xátiva, Valencia; Servicio de Radiodiagnóstico (D.P.-E.), Hospital Universitario La Fe, Valencia; Servicio de Oncologia (A.S.), Hospital Universitario La Fe, Valencia; and Servicio de Neumología (F.Z.), Hospital Universitario Marqués de Valdecilla, Santander, Spain. Address reprint requests to Pilar Morales, Servicio de Neu- mología, Hospital Universitario La Fe, Avda. Campanar 21, 46009-Valencia, Spain. E-mail: pila1460@separ.es © 2005 by Elsevier Inc. All rights reserved. 0041-1345/05/$–see front matter 360 Park Avenue South, New York, NY 10010-1710 doi:10.1016/j.transproceed.2005.09.143 Transplantation Proceedings, 37, 4059 – 4063 (2005) 4059