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Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly
Author(s) -
Glerup Mia,
Thelle Thomas,
Grønbæk Kirsten,
Hasle Henrik
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.22113
Subject(s) - medicine , splenectomy , cytopenia , autoimmune lymphoproliferative syndrome , blood cancer , spleen , pediatrics , anemia , surgery , cancer , bone marrow , apoptosis , biochemistry , chemistry , fas receptor , programmed cell death
Treatment of patients with ALPS has varied but presently there is no consensus about the optimal therapy. Splenectomy is an option but data regarding the postsplenectomy outcome in pediatric ALPS patients remain very limited. We present two children who suffered from anemia and physical discomfort from the large spleen. Both patients underwent uneventful splenectomy and experienced significant improvement in cytopenia, daily activity and well‐being. Furthermore the youngest patient showed a significant catch‐up growth. We conclude that in selected patients with marked splenomegaly and ALPS, splenectomy may be considered a treatment option. Pediatr Blood Cancer 2009;53:1124–1126. © 2009 Wiley‐Liss, Inc.