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Rapid regression of lymphadenopathy upon rapamycin treatment in a child with autoimmune lymphoproliferative syndrome
Author(s) -
Dragana Janić Mihailo,
Dimitrije Brašanac Čedomir,
Srđa Janković Jovan,
Lidija Dokmanović Bogdan,
Nada Krstovski Radmio,
Nada Kraguljac Kurtović Janko
Publication year - 2009
Publication title -
pediatric blood and cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.116
H-Index - 105
eISSN - 1545-5017
pISSN - 1545-5009
DOI - 10.1002/pbc.22151
Subject(s) - autoimmune lymphoproliferative syndrome , medicine , lymphoma , lymphoproliferative disorders , immunology , immune system , apoptosis , fas receptor , programmed cell death , biochemistry , chemistry
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. Although the clinical course of ALPS is highly variable, without treatment long‐term prognosis is unsatisfactory for most patients. ALPS has been treated with most of the existing immunosuppressive agents, with variable success. We hereby present a case of a child with ALPS whose greatly enlarged lymph nodes rapidly regressed upon initiation of rapamycin, a novel potential therapeutic agent in the treatment of ALPS. Pediatr Blood Cancer 2009;53:1117–1119. © 2009 Wiley‐Liss, Inc.