Autoimmune Lymphoproliferative Syndrome

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Autoimmune Lymphoproliferative Syndrome

The autoimmune lymphoproliferative syndrome (ALPS) is a rare disease. ALPS is an inherited condition that affects both sexes. ALPS is not cancer, it is not infectious, and its incidence has not yet been estimated. ALPS generally does not lead to death and most individuals with ALPS are able to live normal lives. ALPS is a disorder associated with abnormal lymphocyte apoptosis, lymphoproliferation, and autoimmunity. Serologic testing is critical in the evaluation of these individuals. Lymphoproliferation in ALPS patients is generally benign, but they are at increased risk for the development of Hodgkin's and non-Hodgkin's lymphoma. It is characterized by massive lymphoadenopathy, splenomegaly, autoimmunity including episodes of immune hemolityc anemia, thrombocytopenia, and neutropenia. ALPS patients have lymphocytosis and a number of lymphocyte abnormalities, including the marked expansion of T lymphocytes that express alpha/beta T-cell receptors, but neither CD4 nor CD8 surface markers (TCR alpha/beta+; CD4-; CD8- cells).

Tron F. Physiology of the autoimmune diseases in hematology. Hématologie 2005; 11: 181-8.

Van der Burg M, De Groot R, Commans-Bitter WM, et al. Autoimmune lymphoproliferative syndrome in a child from consanguineous patients: a dominant or recesive disease? Pediatr Res 2000; 47: 336-43.

Deist LF. Autoimmune lymphoproliferative syndrome. 2006 from the Orphanet encyclopedia website:

Fleisher TA, Oliveira JB. Autoimmune lymphoproliferative syndrome. Immunology and Allergies 2005; 7: 758-61.

Oliveira JB, Fleisher TA. Autoimmune lymphoproliferative syndrome. Curr Opin Allergy Clin Immunol 2004; 6: 497-503.

Bleesing JJ. Autoimmune lymphoproliferative syndrome (ALPS). Curr Pharm Des 2003; 9: 265-78.

Sneller MC, Dale JK, Straus SE. Autoimmune lymphoproliferative syndrome. Curr Opin Immunol 2003; 15: 417-21.

Kanegane H, Vilela Mm, Wang Y, Futatani T, Matsukura H, Miyawaki T. Autoimmune lymphoproliferative syndrome presenting with glomerulonephritis. Pediatr Nephrol 2003; 18: 454-6.

Rao VK, Straus SE. Causes and consequences of the autoimmune lymphoproliferative syndrome. Hematology 2006; 11: 15-23.

Feisher TA. The autoimmune lymphoproliferative syndrome: an experiment of nature involving lymphocyte apoptosis. Immunol Res 2008; 40 (1): 87-92.

Rieux-Laucat F, Deist FL, Fischer A. Autoimmune lymphoproliferative syndromes: genetic defects of apoptosis pathways. Cell Death Diff 2003; 10: 124-33.

Hundt M, Posovszky C, Schmidt RE. A new disorder of lymphocyte apoptosis: a combination of autoimmunity, infectious lymphadenopathy, double negative T cells, and impaired activation-induced cell death. Immunobiology. 2002; 206: 514-8.

Carter LB, Procter JL, Dale JK, Straus SE, Cantilena CC. Description of serologic features in autoimmune lymphoproliferative syndrome. Transfusion 2000; 40: 943-8.

Lopatin U, Yao X, Williams RK, et al. Increases in circulating and lymphoid tissue interleukin-10 in autoimmune lymphoproliferative syndrome are associated with disease expression. Blood 2001; 97: 3161-70.

Mijušković Z, Rackov Lj, Pejović J, Živanović S, Stojanović J, Kovačević Z. Immune complexes and complement in serum and synovial fluid of rheumatoid arthritis patients. Journal of Medical Biochemistry 2009; 28: 166-171.

Journal of Medical Biochemistry

The Journal of Society of Medical Biochemists of Serbia

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