Autoimmune lymphoproliferative syndrome

A rare, inherited disorder characterized by non-malignant lymphoproliferation, multilineage cytopenias, and a lifelong increased risk of Hodgkin's and non-Hodgkin's lymphoma.

Generalized lymphadenopathy

A generalized form of lymphadenopathy.


Total: 3

                       


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PMID (PMCID)
19588524
 FEMALE Infant
Rapid regression of lymphadenopathy upon rapamycin treatment in a child with autoimmune lymphoproliferative syndrome.
Janic MD, Brasanac CD, Jankovic JS, Dokmanovic BL, Krstovski RN, Kraguljac Kurtovic JN.
Pediatr Blood Cancer. 2009;53(6):1117-9.
Autoimmune lymphoproliferative syndrome (ALPS) is a genetic disorder of the immune system caused by inadequate induction of apoptosis via the Fas pathway, mainly characterized by generalized lymphadenopathy, splenomegaly, and autoimmune cytopenias, as well as increased risk of lymphoma.
17301439
 OTHER
Autoimmune lymphoproliferative syndrome; a case report.
Alavi S, Taghi Arzanian M, Chavoshzadeh Z, Esteghamati M.
Iran J Allergy Asthma Immunol. 2005;4(3):149-52.
Autoimmune lymphoproliferative syndrome should be considered in differential diagnosis of any patient with unexplained Coomb's positive cytopenias, hypergammaglobulinemia, generalized lymphadenopathy and splenomegaly.
11233777
 MALE
Interferon-alpha activity in a case of severe autoimmune lymphoproliferative disease.
Ceretelli S, Petrini M, Galimberti S, Testi C, Frizzera G.
Ann Hematol. 2001;80(1):49-52.
Clinical and laboratory features of this disease include a generalized lymphadenopathy, splenomegaly, increased levels of circulating CD3+ with low levels of CD4+, CD8+ T-cells, and autoimmune phenomena, characteristics that the autoimmune lymphoproliferative syndrome (ALPS) have in common.