Autoimmune Lymphoproliferative Syndrome

Common Name(s)

Autoimmune Lymphoproliferative Syndrome

Autoimmune lymphoproliferative syndrome (ALPS) is a disorder in which the body cannot properly regulate the number of immune system cells (lymphocytes). This results in the overproduction of lymphocytes, which build up and cause enlargement of the lymph nodes, liver and spleen. Affected individuals can have a variety of autoimmune disorders, most of which damage the blood cells; some of the autoimmune disorders associated with ALPS can also damage the kidneys, liver, eyes, nerves, or connective tissues. Other signs and symptoms may include skin rashes or panniculitis; arthritis; inflammation of blood vessels (vasculitis); mouth sores; premature ovarian failure; and the development of neurological damage. ALPS is caused by mutations in the FAS gene in about 75% of cases. It is usually inherited in an autosomal dominant manner, although some severe cases are inherited in an autosomal recessive manner. Management may include steroids or other medications, blood transfusions, and/or splenectomy depending on the severity of the disorder. ALPS is categorized into several types based mainly on the genetic cause.

 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Autoimmune Lymphoproliferative Syndrome" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Autoimmune Lymphoproliferative Syndrome" returned 66 free, full-text research articles on human participants. First 3 results:

Unexplained lymphadenopathies: autoimmune lymphoproliferative syndrome in an adult patient.
 

Author(s): Fatima Leal-Seabra, Gonçalo Sarmento Costa, Henrique Pereira Coelho, Agripino Oliveira

Journal:

 

Autoimmune lymphoproliferative syndrome (ALPS) is characterised by massive enlargement of the lymphoid organs, autoimmune cytopenias and a predisposition to develop lymphoid malignancies. The basic defect is a disturbance of the lymphocyte apoptosis, and a high number of circulating ...

Last Updated: 16 Dec 2016

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Bone marrow findings in autoimmune lymphoproliferative syndrome with germline FAS mutation.
 

Author(s): Yi Xie, Stefania Pittaluga, Susan Price, Mark Raffeld, Jamie Hahn, Elaine S Jaffe, V Koneti Rao, Irina Maric

Journal: Haematologica. 2017 Feb;102(2):364-372.

 

Autoimmune lymphoproliferative syndrome is a rare genetic disorder characterized by defective FAS-mediated apoptosis, autoimmune disease, accumulation of mature T-cell receptor alpha/beta positive, CD4 and CD8 double-negative T cells and increased risk of lymphoma. Despite frequent ...

Last Updated: 15 Nov 2016

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Hyperactive mTOR pathway promotes lymphoproliferation and abnormal differentiation in autoimmune lymphoproliferative syndrome.
 

Author(s): Simon Völkl, Anne Rensing-Ehl, Andrea Allgäuer, Elisabeth Schreiner, Myriam Ricarda Lorenz, Jan Rohr, Christian Klemann, Ilka Fuchs, Volker Schuster, André O von Bueren, Nora Naumann-Bartsch, Eleonora Gambineri, Kathrin Siepermann, Robin Kobbe, Michaela Nathrath, Peter D Arkwright, Maurizio Miano, Klaus-Daniel Stachel, Markus Metzler, Klaus Schwarz, Anita N Kremer, Carsten Speckmann, Stephan Ehl, Andreas Mackensen

Journal: Blood. 2016 Jul;128(2):227-38.

 

Autoimmune lymphoproliferative syndrome (ALPS) is a human disorder characterized by defective Fas signaling, resulting in chronic benign lymphoproliferation and accumulation of TCRαβ(+) CD4(-) CD8(-) double-negative T (DNT) cells. Although their phenotype resembles that of terminally ...

Last Updated: 15 Jul 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Autoimmune Lymphoproliferative Syndrome" returned 8 free, full-text review articles on human participants. First 3 results:

[Autoimmune lymphoproliferative syndrome: a case report and literature review].
 

Author(s): Jia-peng Sun, Xin-tian Lu, Wei-hong Zhao, Ying Hua

Journal: Beijing Da Xue Xue Bao. 2015 Dec;47(6):1022-7.

 

We described 1 case of autoimmune lymphoproliferative syndrome (ALPS), first diagnosed in our hospital, and reviewed the recent literature. The 11-month old male patient presented with a history of splenomegaly and hepatomegaly since 1 month after birth. He suffered recurrent infectious ...

Last Updated: 18 Dec 2015

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Autoimmune lymphoproliferative syndrome: an update and review of the literature.
 

Author(s): Shaili Shah, Eveline Wu, V Koneti Rao, Teresa K Tarrant

Journal: Curr Allergy Asthma Rep. 2014 Sep;14(9):462.

 

Autoimmune lymphoproliferative syndrome (ALPS) is characterized by immune dysregulation due to a defect in lymphocyte apoptosis. The clinical manifestations may be noted in multiple family members and include lymphadenopathy, splenomegaly, increased risk of lymphoma, and autoimmune ...

Last Updated: 4 Aug 2014

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[Advances in the knowledge and management of autoimmune lymphoproliferative syndrome].
 

Author(s): C Garrido Colino

Journal: An Pediatr (Barc). 2014 Feb;80(2):122.e1-7.

 

Autoimmune lymphoproliferative syndrome (ALPS) represents a failure of apoptotic mechanisms to maintain lymphocyte homeostasis. ALPS often manifest in childhood with cytopenias, chronic non-malignant lymphoproliferation and autoimmune complications. A number of new insights have improved ...

Last Updated: 31 Jan 2014

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Study of Autoimmune Lymphoproliferative Syndrome (ALPS)
 

Status: Recruiting

Condition Summary: Autoimmune Lymphproliferative Syndrome

 

Last Updated: 18 Oct 2017

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Immune Disorder HSCT Protocol
 

Status: Recruiting

Condition Summary: Immune Deficiency Disorders; Severe Combined Immunodeficiency; Chronic Granulomatous Disease; X-linked Agammaglobulinemia; Wiskott-Aldrich Syndrome; Hyper-IgM; DiGeorge Syndrome; Chediak-Higashi Syndrome; Common Variable Immune Deficiency; Immune Dysregulatory Disorders; Hemophagocytic Lymphohistiocytosis; IPEX; Autoimmune Lymphoproliferative Syndrome; X-linked Lymphoproliferative Syndrome

 

Last Updated: 1 Nov 2016

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Reduced Intensity Conditioning for Non-Malignant Disorders Undergoing UCBT, BMT or PBSCT
 

Status: Recruiting

Condition Summary: Primary Immunodeficiency (PID); Congenital Bone Marrow Failure Syndromes; Inherited Metabolic Disorders (IMD); Hereditary Anemias; Inflammatory Conditions

 

Last Updated: 25 Oct 2017

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