Table of Contents
Lymphoma
Volume 2015, Article ID 123548, 10 pages
http://dx.doi.org/10.1155/2015/123548
Research Article

Non-Hodgkin Lymphoma in Children with Primary Immunodeficiencies: Clinical Manifestations, Diagnosis, and Management, Belarusian Experience

1Clinical Research Department, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
2Laboratory of Immunology, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
3Department of Pathology, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
4Consultative and Outpatient Department, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
5Oncohematological Department N2, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
6Laboratory of Genetic Biotechnology, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
7Research Department, Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus
8Belarusian Research Center for Pediatric Oncology, Hematology and Immunology, Borovlyany, 223053 Minsk, Belarus

Received 29 September 2014; Accepted 11 January 2015

Academic Editor: Wolfram Klapper

Copyright © 2015 Alina Fedorova et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Introduction. Non-Hodgkin lymphoma (NHL) is the most frequent malignancy associated with primary immune deficiency disease (PID). We aimed to present the clinical characteristics and outcomes of Belarusian children with PID who developed NHL. Procedure. We reviewed 16 patients with PID and NHL. Eight patients had combined PID: 5—Nijmegen breakage syndrome, 1—Bloom syndrome, 1—Wiskott-Aldrich syndrome, and 1—Х-linked lymphoproliferative syndrome. Results. In 75% cases PID was diagnosed simultaneously or after the NHL was confirmed. PID-associated NHL accounted for 5.7% of all NHL and was characterized by younger median age (6.3 versus 10.0 years, ) and by prevalence of large-cell types (68.8% versus 24.5%, ). Children with combined PID had median age of 1.3 years; 5 of them developed EBV-associated diffuse large B-cell lymphoma with lung involvement. Five of 6 patients with chromosomal breakage syndrome developed T-NHL. Six patients died of infections; two died after tumor progression; one child had early relapse; two died of second NHL and one of secondary hemophagocytic syndrome. Overall, 4 children are alive and disease-free after a follow-up from 1.4 to 5.7 years. Conclusions. PID needs to be diagnosed early. Individualized chemotherapy, comprehensive supportive treatment, and hematopoietic stem cell transplantation may improve survival of children with PID and NHL.