TY - JOUR
TI - Mycophenolate mofetil as an alternate immunosuppressor for autoimmune
lymphoproliferative syndrome
AU - Kossiva, Lydia
AU - Theodoridou, Maria
AU - Mostrou, Glykeria and
AU - Vrachnou, Effie
AU - Le Deist, Francoise
AU - Rieux-Laucat, Frederick and
AU - Kanariou, Maria G.
JO - Journal of Pediatric Hematology / Oncology
PY - 2006
VL - 28
TODO - 12
SP - 824-826
PB - Lippincott, Williams & Wilkins
SN - 1077-4114, 1536-3678
TODO - 10.1097/MPH.0b013e31802d7503
TODO - autoimmune lymphoproliferative syndrome; autoimmune cytopenia;
corticosteroid; mycophenolate mofetil
TODO - 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.
ER -