Common Variable Immunodeficiency Associated with Hepatosplenic T-Cell Lymphoma Mimicking Juvenile Systemic Lupus Erythematosus
Carregando...
Citações na Scopus
22
Tipo de produção
article
Data de publicação
2011
Título da Revista
ISSN da Revista
Título do Volume
Editora
HINDAWI PUBLISHING CORPORATION
Citação
CLINICAL & DEVELOPMENTAL IMMUNOLOGY, article ID 428703, 4p, 2011
Resumo
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 40mg/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.
Palavras-chave
Referências
- Hochberg MC, 1997, ARTHRITIS RHEUM, V40, P1725, DOI 10.1002/art.1780400928
- Chapel H, 2009, BRIT J HAEMATOL, V145, P709, DOI 10.1111/j.1365-2141.2009.07669.x
- Tripodo C, 2009, NAT REV CLIN ONCOL, V6, P707, DOI 10.1038/nrclinonc.2009.169
- Carneiro-Sampaio M, 2008, J CLIN IMMUNOL, V28, pS34, DOI 10.1007/s10875-008-9187-2
- Notarangelo LD, 2009, J ALLERGY CLIN IMMUN, V124, P1161, DOI 10.1016/j.jaci.2009.10.013
- Gonzalez EG, 2007, CLIN RHEUMATOL, V26, P244, DOI 10.1007/s10067-005-0125-x
- Carneiro-Sampaio M, 2007, ADV IMMUNOL, V95, P51, DOI 10.1016/S0065-2776(07)95002-6
- Parodi A, 2009, ARTHRITIS RHEUM-US, V60, P3388, DOI 10.1002/art.24883
- Fernandez-Castro M, 2007, SEMIN ARTHRITIS RHEU, V36, P238, DOI 10.1016/j.semarthrit.2006.09.005
- FERRAZ MB, 1994, CLIN EXP RHEUMATOL, V12, P83
- KUMAR R, DIAGNOSTIC IN PRESS
- MARTINAUD C, 2011, REV MED INTERNE, V32, P15
- Seyrek N, 1996, NEPHRON, V72, P489