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 57 free, full-text research articles on human participants. First 3 results:

Gray platelet syndrome can mimic autoimmune lymphoproliferative syndrome.
 

Author(s): Anne Rensing-Ehl, Ulrich Pannicke, Stefanie-Yvonne Zimmermann, Myriam Ricarda Lorenz, Benedicte Neven, Ilka Fuchs, Ulrich Salzer, Carsten Speckmann, Anne Strauss, Eberhard Maaβ, Benedicte Collet, Anselm Enders, Remi Favier, Marie Christine Alessi, Frederic Rieux-Laucat, Barbara Zieger, Klaus Schwarz, Stephan Ehl

Journal: Blood. 2015 Oct;126(16):1967-9.

 

Last Updated: 16 Oct 2015

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Natural history of autoimmune lymphoproliferative syndrome associated with FAS gene mutations.
 

Author(s): Susan Price, Pamela A Shaw, Amy Seitz, Gyan Joshi, Joie Davis, Julie E Niemela, Katie Perkins, Ronald L Hornung, Les Folio, Philip S Rosenberg, Jennifer M Puck, Amy P Hsu, Bernice Lo, Stefania Pittaluga, Elaine S Jaffe, Thomas A Fleisher, V Koneti Rao, Michael J Lenardo

Journal: Blood. 2014 Mar;123(13):1989-99.

 

Autoimmune lymphoproliferative syndrome (ALPS) presents in childhood with nonmalignant lymphadenopathy and splenomegaly associated with a characteristic expansion of mature CD4 and CD8 negative or double negative T-cell receptor αβ(+) T lymphocytes. Patients often present with chronic ...

Last Updated: 28 Mar 2014

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Autoimmune lymphoproliferative syndrome misdiagnosed as hemophagocytic lymphohistiocytosis.
 

Author(s): Amanda Rudman Spergel, Kelly Walkovich, Susan Price, Julie E Niemela, Dowain Wright, Thomas A Fleisher, V Koneti Rao

Journal: Pediatrics. 2013 Nov;132(5):e1440-4.

 

Autoimmune lymphoproliferative syndrome (ALPS) is a rare inherited disorder of apoptosis, most commonly due to mutations in the FAS (TNFRSF6) gene. It presents with chronic lymphadenopathy, splenomegaly, and symptomatic multilineage cytopenias in an otherwise healthy child. Unfortunately, ...

Last Updated: 4 Nov 2013

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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 Disease; Lymphatic Disease; Lymphoproliferative Disorder; Canale-Smith Syndrome

 

Last Updated: 14 Mar 2014

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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 Disorder:; Hemophagocytic Lymphohistiocytosis; IPEX; Autoimmune Lymphoproliferative Syndrome; X-linked Lymphoproliferative Syndrome

 

Last Updated: 9 Jan 2016

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Sirolimus for Autoimmune Disease of Blood Cells
 

Status: Recruiting

Condition Summary: Autoimmune Pancytopenia; Autoimmune Lymphoproliferative Syndrome (ALPS); Evans Syndrome; Idiopathic Thrombocytopenic Purpura; Anemia, Hemolytic, Autoimmune; Autoimmune Neutropenia; Lupus Erythematosus, Systemic; Inflammatory Bowel Disease; Rheumatoid Arthritis

 

Last Updated: 20 Oct 2015

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