Autoimmune lymphoproliferative syndrome. Update and review.
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| Title: | Autoimmune lymphoproliferative syndrome. Update and review. |
|---|---|
| Alternate Title: | Síndrome linfoproliferativo autoinmune. Actualización y revisión. |
| Authors: | Suárez-Gutiérrez, Marcos1 (AUTHOR), Yamazaki-Nakashimada, Marco Antonio2 (AUTHOR), Espinosa, Sara3 (AUTHOR), Blancas-Galicia, Lizbeth3 (AUTHOR), Lugo-Reyes, Saúl Oswaldo3 (AUTHOR), Álvarez-Cardona, Aristóteles1 (AUTHOR) dr.aristoteles@yahoo.com.mx |
| Source: | Revista Alergia de Mexico. Oct-Dec2019, Vol. 66 Issue 4, p456-473. 18p. 3 Diagrams, 3 Charts. |
| Abstract (English): | The autoimmune lymphoproliferative syndrome (ALPS) is an inborn immunity error, which is the result of a heterogeneous group of mutations in the genes that regulate the apoptosis phenomenon. It typically appears in the first years of life. The most common clinical signs are lymphoid expansion with lymphadenopathy, splenomegaly, and hepatomegaly; immune disease with different types of cytopenia, including thrombocytopenia, hemolytic anemia, and lymphoma. The lab abnormalities that facilitate the diagnosis of ALPS include the presence of double negative alpha/beta T cells, high interleukin levels, vitamin B12 in the blood, and FAS-mediated defective apoptosis in the in vitro assay. The treatment of ALPS is focused on three aspects: The treatment of the manifestations of the disease, the prevention/treatment of complications, and the curative treatment (hematopoietic progenitor cell transplantation [HPCT]). The use of immunosuppressive therapy is suggested only for severe complications of lymphoproliferation or concomitant autoimmune manifestations. Splenectomy is not recommended for autoimmune manifestations in patients with ALPS. HPCT is reserved for selected patients. The survival rate to 50 years is estimated at 85% for patients with FAS deficiency. [ABSTRACT FROM AUTHOR] |
| Abstract (Spanish): | El síndrome linfoproliferativo autoinmune (ALPS, autoimmune lymphoproliferative syndrome) es un error innato de la inmunidad, resultado de un grupo heterogéneo de alteraciones en los genes que regulan el fenómeno de apoptosis. Se manifiesta típicamente en los primeros años de vida. Las manifestaciones clínicas más comunes son la expansión linfoide con linfadenopatía, esplenomegalia y hepatomegalia, enfermedad autoinmune con citopenias, incluyendo trombocitopenia y anemia hemolítica, así como linfoma. Las anomalías de laboratorio que facilitan el diagnóstico de ALPS incluyen presencia de células alfa-beta T doble negativas, niveles elevados de interleucina 10, vitamina B12 en sangre y apoptosis defectuosa mediada por FAS en ensayo in vitro. El tratamiento de ALPS se centra en tres aspectos: el tratamiento de las manifestaciones de la enfermedad, la prevención y tratamiento de las complicaciones y el tratamiento curativo (trasplante de células progenitoras hematopoyéticas [TCPH]). Se sugiere el uso de tratamiento inmunosupresor solo para las complicaciones graves de la linfoproliferación o manifestaciones autoinmunes concomitantes. La esplenectomía no se recomienda para las manifestaciones autoinmunes en pacientes con ALPS. El TCPH se reserva para pacientes seleccionados. La tasa de supervivencia a 50 años se estima en 85 % para los pacientes con deficiencia de FAS. [ABSTRACT FROM AUTHOR] |
| Copyright of Revista Alergia de Mexico is the property of Coleg. Mexicano de Inmunologia Clinica y Alergia A.C.; Soc. Lat. de Alergia, Asma e Inmunologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.) | |
| Database: | MedicLatina |
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| Header | DbId: lth DbLabel: MedicLatina An: 142035842 AccessLevel: 6 PubType: Academic Journal PubTypeId: academicJournal PreciseRelevancyScore: 0 |
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| Items | – Name: Title Label: Title Group: Ti Data: Autoimmune lymphoproliferative syndrome. Update and review. – Name: TitleAlt Label: Alternate Title Group: TiAlt Data: Síndrome linfoproliferativo autoinmune. Actualización y revisión. – Name: Author Label: Authors Group: Au Data: <searchLink fieldCode="AR" term="%22Suárez-Gutiérrez%2C+Marcos%22">Suárez-Gutiérrez, Marcos</searchLink><relatesTo>1</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Yamazaki-Nakashimada%2C+Marco+Antonio%22">Yamazaki-Nakashimada, Marco Antonio</searchLink><relatesTo>2</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Espinosa%2C+Sara%22">Espinosa, Sara</searchLink><relatesTo>3</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Blancas-Galicia%2C+Lizbeth%22">Blancas-Galicia, Lizbeth</searchLink><relatesTo>3</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Lugo-Reyes%2C+Saúl+Oswaldo%22">Lugo-Reyes, Saúl Oswaldo</searchLink><relatesTo>3</relatesTo> (AUTHOR)<br /><searchLink fieldCode="AR" term="%22Álvarez-Cardona%2C+Aristóteles%22">Álvarez-Cardona, Aristóteles</searchLink><relatesTo>1</relatesTo> (AUTHOR)<i> dr.aristoteles@yahoo.com.mx</i> – Name: TitleSource Label: Source Group: Src Data: <searchLink fieldCode="JN" term="%22Revista+Alergia+de+Mexico%22">Revista Alergia de Mexico</searchLink>. Oct-Dec2019, Vol. 66 Issue 4, p456-473. 18p. 3 Diagrams, 3 Charts. – Name: Abstract Label: Abstract (English) Group: Ab Data: The autoimmune lymphoproliferative syndrome (ALPS) is an inborn immunity error, which is the result of a heterogeneous group of mutations in the genes that regulate the apoptosis phenomenon. It typically appears in the first years of life. The most common clinical signs are lymphoid expansion with lymphadenopathy, splenomegaly, and hepatomegaly; immune disease with different types of cytopenia, including thrombocytopenia, hemolytic anemia, and lymphoma. The lab abnormalities that facilitate the diagnosis of ALPS include the presence of double negative alpha/beta T cells, high interleukin levels, vitamin B12 in the blood, and FAS-mediated defective apoptosis in the in vitro assay. The treatment of ALPS is focused on three aspects: The treatment of the manifestations of the disease, the prevention/treatment of complications, and the curative treatment (hematopoietic progenitor cell transplantation [HPCT]). The use of immunosuppressive therapy is suggested only for severe complications of lymphoproliferation or concomitant autoimmune manifestations. Splenectomy is not recommended for autoimmune manifestations in patients with ALPS. HPCT is reserved for selected patients. The survival rate to 50 years is estimated at 85% for patients with FAS deficiency. [ABSTRACT FROM AUTHOR] – Name: Abstract Label: Abstract (Spanish) Group: Ab Data: El síndrome linfoproliferativo autoinmune (ALPS, autoimmune lymphoproliferative syndrome) es un error innato de la inmunidad, resultado de un grupo heterogéneo de alteraciones en los genes que regulan el fenómeno de apoptosis. Se manifiesta típicamente en los primeros años de vida. Las manifestaciones clínicas más comunes son la expansión linfoide con linfadenopatía, esplenomegalia y hepatomegalia, enfermedad autoinmune con citopenias, incluyendo trombocitopenia y anemia hemolítica, así como linfoma. Las anomalías de laboratorio que facilitan el diagnóstico de ALPS incluyen presencia de células alfa-beta T doble negativas, niveles elevados de interleucina 10, vitamina B12 en sangre y apoptosis defectuosa mediada por FAS en ensayo in vitro. El tratamiento de ALPS se centra en tres aspectos: el tratamiento de las manifestaciones de la enfermedad, la prevención y tratamiento de las complicaciones y el tratamiento curativo (trasplante de células progenitoras hematopoyéticas [TCPH]). Se sugiere el uso de tratamiento inmunosupresor solo para las complicaciones graves de la linfoproliferación o manifestaciones autoinmunes concomitantes. La esplenectomía no se recomienda para las manifestaciones autoinmunes en pacientes con ALPS. El TCPH se reserva para pacientes seleccionados. La tasa de supervivencia a 50 años se estima en 85 % para los pacientes con deficiencia de FAS. [ABSTRACT FROM AUTHOR] – Name: AbstractSuppliedCopyright Label: Group: Ab Data: <i>Copyright of Revista Alergia de Mexico is the property of Coleg. Mexicano de Inmunologia Clinica y Alergia A.C.; Soc. Lat. de Alergia, Asma e Inmunologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract.</i> (Copyright applies to all Abstracts.) |
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| RecordInfo | BibRecord: BibEntity: Identifiers: – Type: doi Value: 10.29262/ram.v66i4.646 Languages: – Code: spa Text: Spanish PhysicalDescription: Pagination: PageCount: 18 StartPage: 456 Titles: – TitleFull: Autoimmune lymphoproliferative syndrome. Update and review. Type: main BibRelationships: HasContributorRelationships: – PersonEntity: Name: NameFull: Suárez-Gutiérrez, Marcos – PersonEntity: Name: NameFull: Yamazaki-Nakashimada, Marco Antonio – PersonEntity: Name: NameFull: Espinosa, Sara – PersonEntity: Name: NameFull: Blancas-Galicia, Lizbeth – PersonEntity: Name: NameFull: Lugo-Reyes, Saúl Oswaldo – PersonEntity: Name: NameFull: Álvarez-Cardona, Aristóteles IsPartOfRelationships: – BibEntity: Dates: – D: 01 M: 10 Text: Oct-Dec2019 Type: published Y: 2019 Identifiers: – Type: issn-print Value: 00025151 Numbering: – Type: volume Value: 66 – Type: issue Value: 4 Titles: – TitleFull: Revista Alergia de Mexico Type: main |
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