Rapid regression of lymphadenopathy upon rapamycin treatment in a child with autoimmune lymphoproliferative syndrome

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...

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Bibliographic Details
Published in:Pediatric blood & cancer 2009-12, Vol.53 (6), p.1117-1119
Main Authors: Dragana, Janić Mihailo, Dimitrije, Brašanac Čedomir, Srđa, Janković Jovan, Lidija, Dokmanović Bogdan, Nada, Krstovski Radmio, Nada, Kraguljac Kurtović Janko
Format: Article
Language:English
Subjects:
Apoptosis
Autoimmune Diseases - drug therapy
Blood
Cancer
CD95 antigen
Fas antigen
Female
Humans
immune cytopenias
Immune system
immunology
Immunosuppressive agents
Immunosuppressive Agents - therapeutic use
Infant
Lymph nodes
Lymph Nodes - pathology
Lymphadenopathy
Lymphatic Diseases - drug therapy
Lymphatic Diseases - pathology
Lymphocytes
Lymphoma
Lymphoproliferative Disorders - drug therapy
Lymphoproliferative Disorders - immunology
Lymphoproliferative Disorders - pathology
pharmacology
Prognosis
Rapamycin
Remission Induction - methods
Sirolimus - therapeutic use
Splenomegaly
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Summary: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.
ISSN:1545-5009
1545-5017
1545-5017
DOI:10.1002/pbc.22151