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Clinical and Developmental Immunology
Volume 2011, Article ID 428703, 4 pages
http://dx.doi.org/10.1155/2011/428703
Case Report

Common Variable Immunodeficiency Associated with Hepatosplenic T-Cell Lymphoma Mimicking Juvenile Systemic Lupus Erythematosus

1Rheumatology Division, Children's Hospital, Avenida Dr. Eneas Carvalho de Aguiar, 647, 05403-000 Sao Paulo, SP, Brazil
2Allergy and Immunology Division, Children's Hospital, Avenida Dr. Eneas Carvalho de Aguiar, 647, 05403-000 Sao Paulo, SP, Brazil
3Division of Rheumatology, Hospital das Clínicas, Faculdade de Medicina, Universidade São Paulo, Av. Dr. Arnaldo 455, sala 3133, 01246-903 São Paulo, SP, Brazil

Received 11 March 2011; Revised 29 April 2011; Accepted 2 May 2011

Academic Editor: Clelia M. Riera

Copyright © 2011 A. A. Jesus 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

Common variable immunodeficiency (CVID) is a heterogeneous disorder with susceptibility to infections, autoimmune manifestations, and cancer. To our knowledge, CIVD with T-cell lymphoma mimicking juvenile systemic lupus erythematosus (JSLE) was not described in the literature, and one case was reported herein. An 8-year-old female was admitted in our Pediatric Immunology Unit with a clinical history of hypogammaglobulinemia, recurrent upper respiratory infections, and pneumonias. She had a marked decrease of three serum immunoglobulin isotypes, and the diagnosis of CVID was established. At the age of 17 years, she presented with oral ulceration, nonerosive arthritis, nephritis, serositis, cytopenia, positive antiphospholipid antibodies, and positive antinuclear antibody fulfilling the American College of Rheumatology (ACR) criteria for SLE. She was treated with intravenous methylprednisolone for three consecutive days, and intravenous immunoglobulin, and maintenance therapy of chloroquine, azathioprine and prednisone 40 mg/day. Two months later, she died of septic shock secondary to acute pneumonia. The necropsy showed hepatosplenic T-cell lymphoma with diffuse involvement of bone marrow, spleen, liver, and lungs. The lymphoma cells were positive for CD3 immunostaining and negative for CD20 and lysozyme. In conclusion, the association of CVID and hepatosplenic T-cell lymphoma may simulate JSLE diagnosis.