Τίτλος:
Mycophenolate mofetil as an alternate immunosuppressor for autoimmune
lymphoproliferative syndrome
Γλώσσες Τεκμηρίου:
Αγγλικά
Περίληψη:
Autoimmune lymphoproliferative syndrome (ALPS) is a disorder due to a
genetic defect concerning programmed cell death (apoptosis). Most
patients are carriers of a heterozygous mutation affecting the TNFRSF6
(Fas). Treatment of autoimmune complications of ALPS includes
corticosteroids, gamma-globulin infusions, and in refractory cases,
splenectomy, cytostatic agents, and bone marrow transplantation. A
10-year-old boy with ALPS manifested by recurrent febrile episodes,
lymphadenopathy, splenomegaly, and cytopenias refractory to
corticosteroid therapy is presented. Treatment with mycophenolate
mofetil, an immunosuppressive agent typically used in organ
transplantation was initiated. This treatment was successful with
resolution of thrombocytopenia, decrease in lymphadenopathy, and
improvement of his general clinical condition for over 2 years of
duration.
Συγγραφείς:
Kossiva, Lydia
Theodoridou, Maria
Mostrou, Glykeria and
Vrachnou, Effie
Le Deist, Francoise
Rieux-Laucat, Frederick and
Kanariou, Maria G.
Περιοδικό:
Journal of Pediatric Hematology / Oncology
Εκδότης:
Lippincott, Williams & Wilkins
Λέξεις-κλειδιά:
autoimmune lymphoproliferative syndrome; autoimmune cytopenia;
corticosteroid; mycophenolate mofetil
DOI:
10.1097/MPH.0b013e31802d7503
