Autoimmune lymphoproliferative syndrome. Update and review
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 l...
| Main Authors: | , , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Colegio Mexicano de Inmunología Clínica y Alergia, A.C.
2019-11-01
|
| Series: | Revista Alergia México |
| Subjects: | |
| Online Access: | http://revistaalergia.mx/ojs/index.php/ram/article/view/646 |
| _version_ | 1797764708620566528 |
|---|---|
| author | Marcos Suárez-Gutiérrez Marco Antonio Yamazaki-Nakashimada Sara Espinosa Lizbeth Blancas-Galicia Saúl Oswaldo Lugo-Reyes Aristóteles Álvarez-Cardona |
| author_facet | Marcos Suárez-Gutiérrez Marco Antonio Yamazaki-Nakashimada Sara Espinosa Lizbeth Blancas-Galicia Saúl Oswaldo Lugo-Reyes Aristóteles Álvarez-Cardona |
| author_sort | Marcos Suárez-Gutiérrez |
| collection | DOAJ |
| description | 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. |
| first_indexed | 2024-03-12T19:59:39Z |
| format | Article |
| id | doaj.art-329488ddf65a49de9154bd4a6b89afaf |
| institution | Directory Open Access Journal |
| issn | 0002-5151 2448-9190 |
| language | English |
| last_indexed | 2024-03-12T19:59:39Z |
| publishDate | 2019-11-01 |
| publisher | Colegio Mexicano de Inmunología Clínica y Alergia, A.C. |
| record_format | Article |
| series | Revista Alergia México |
| spelling | doaj.art-329488ddf65a49de9154bd4a6b89afaf2023-08-02T02:29:14ZengColegio Mexicano de Inmunología Clínica y Alergia, A.C.Revista Alergia México0002-51512448-91902019-11-0166445647310.29262/ram.v66i4.646449Autoimmune lymphoproliferative syndrome. Update and reviewMarcos Suárez-Gutiérrez0Marco Antonio Yamazaki-Nakashimada1Sara Espinosa2Lizbeth Blancas-Galicia3Saúl Oswaldo Lugo-Reyes4Aristóteles Álvarez-Cardona5Clínica Casa Blanca, Unidad de Investigación en Inmunología Clínica y Alergia, Aguascalientes, AguascalientesInstituto Nacional de Pediatría, Departamento de Inmunología Clínica, Ciudad de México Instituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias, Ciudad de MéxicoInstituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias, Ciudad de México, MéxicoInstituto Nacional de Pediatría, Unidad de Investigación en Inmunodeficiencias, Ciudad de México, MéxicoClínica Casa Blanca, Unidad de Investigación en Inmunología Clínica y Alergia, Aguascalientes, AguascalientesThe 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.http://revistaalergia.mx/ojs/index.php/ram/article/view/646síndrome linfoproliferativo autoinmunefasfaslglinfoproliferaciónautoinmunidad |
| spellingShingle | Marcos Suárez-Gutiérrez Marco Antonio Yamazaki-Nakashimada Sara Espinosa Lizbeth Blancas-Galicia Saúl Oswaldo Lugo-Reyes Aristóteles Álvarez-Cardona Autoimmune lymphoproliferative syndrome. Update and review Revista Alergia México síndrome linfoproliferativo autoinmune fas faslg linfoproliferación autoinmunidad |
| title | Autoimmune lymphoproliferative syndrome. Update and review |
| title_full | Autoimmune lymphoproliferative syndrome. Update and review |
| title_fullStr | Autoimmune lymphoproliferative syndrome. Update and review |
| title_full_unstemmed | Autoimmune lymphoproliferative syndrome. Update and review |
| title_short | Autoimmune lymphoproliferative syndrome. Update and review |
| title_sort | autoimmune lymphoproliferative syndrome update and review |
| topic | síndrome linfoproliferativo autoinmune fas faslg linfoproliferación autoinmunidad |
| url | http://revistaalergia.mx/ojs/index.php/ram/article/view/646 |
| work_keys_str_mv | AT marcossuarezgutierrez autoimmunelymphoproliferativesyndromeupdateandreview AT marcoantonioyamazakinakashimada autoimmunelymphoproliferativesyndromeupdateandreview AT saraespinosa autoimmunelymphoproliferativesyndromeupdateandreview AT lizbethblancasgalicia autoimmunelymphoproliferativesyndromeupdateandreview AT sauloswaldolugoreyes autoimmunelymphoproliferativesyndromeupdateandreview AT aristotelesalvarezcardona autoimmunelymphoproliferativesyndromeupdateandreview |