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PKP Metadata Items |
Metadata for this Document |
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| 1. |
Title |
Title of document |
Autoimmune lymphoproliferative syndrome. |
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| 2. |
Creator |
Author's name, affiliation, country |
Vera Rodrigues; Serviço de Pediatria, Hospital Fernando Fonseca, Amadora-Sintra, Portugal.; Portugal |
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Creator |
Author's name, affiliation, country |
Marta Conde; Portugal |
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Creator |
Author's name, affiliation, country |
António Figueiredo; Portugal |
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Creator |
Author's name, affiliation, country |
Júlia Vasconcelos; Portugal |
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Creator |
Author's name, affiliation, country |
Alexandra Dias; Portugal |
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| 3. |
Subject |
Discipline(s) |
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Subject |
Keyword(s) |
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| 4. |
Description |
Abstract |
The Autoimmune Lymphoproliferative Syndrome (ALPS) is an impairment of lymphocyte apoptosis expressed by generalized non-malignant lymphoproliferation, lymphadenopathy and/or splenomegaly. This article describes a seven and 14 year old males. The first one was admitted at 3 years of age with fever, bicytopenia and generalized lymphadenopathy. Hystopathological analysis of lymph nodes showed reactive follicular hyperplasia and marked paracortical expansion. He was readmitted three years later presenting herpes zoster and similar clinical features. High levels of IL-10 and increasing tendency of Fas-L in plasma and serum. The second child was admitted at 13 years of age presenting thigh and gluteus cellulitis, anemia and neutropenia. T lymphocytes aß+CD4-CD8- 3,1%. Hystopathological analysis of lymph nodes showed marked paracortical hyperplasia. Both children are treated with mycophenolate mofetil with good response. ALPS is an underestimated entity that must be considered in non malign lymphoproliferation, autoimmunity and expansion of an unusual population of a/ßCD3+CD4-CD8-(double-negative T cells>1%). |
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Publisher |
Organizing agency, location |
Ordem dos Médicos |
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Contributor |
Sponsor(s) |
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Date |
(YYYY-MM-DD) |
2011-12-29
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Type |
Status & genre |
article |
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| 8. |
Type |
Type |
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| 9. |
Format |
File format |
PDF (Português) |
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| 10. |
Identifier |
Uniform Resource Identifier |
https://actamedicaportuguesa.com:443/revista/index.php/amp/article/view/501 |
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| 10. |
Identifier |
Digital Object Identifier (DOI) |
http://dx.doi.org/10.20344/amp.501 |
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| 11. |
Source |
Title; vol., no. (year) |
Acta Médica Portuguesa; Vol 24, No 5 (2011): Setembro-Outubro |
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Language |
English=en |
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Coverage |
Geo-spatial location, chronological period, research sample (gender, age, etc.) |
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| 15. |
Rights |
Copyright and permissions |
Copyright (c)
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