Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly.

Full Abstract

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.

Author information

Author/s: Glerup, Mia (M); Thelle, Thomas (T); Grønbaek, Kirsten (K); Hasle, Henrik (H);

Affiliation: Department of Pediatrics, Aarhus University Hospital Skejby, Aarhus, Denmark.

Journal and publication information

Publication Type: Case Reports; Journal Article

Journal: Pediatric blood & cancer (Pediatr Blood Cancer), published in United States. (Language: eng)

Reference: 2009-Dec; vol 53 (issue 6) : pp 1124-6

Dates: Created 2009/09/16; Completed 2009/10/13;

PMID: 19499578, status: MEDLINE (last retrieval date: 10/13/2009, IMS Date: )

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