Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly

TY - JOUR

T1 - Splenectomy in two children with autoimmune lymphoproliferative syndrome and massive splenomegaly

AU - Glerup, Mia

AU - Thelle, Thomas

AU - Grønbaek, Kirsten

AU - Hasle, Henrik

N1 - Keywords: Adolescent; Anemia, Hemolytic, Autoimmune; Female; Growth; Humans; Infant; Lymphoproliferative Disorders; Male; Splenectomy; Splenomegaly; Treatment Outcome

PY - 2009

Y1 - 2009

N2 - 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.

AB - 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.

U2 - 10.1002/pbc.22113

DO - 10.1002/pbc.22113

M3 - Journal article

C2 - 19499578

SN - 1545-5009

VL - 53

SP - 1124

EP - 1126

JO - Pediatric Blood and Cancer

JF - Pediatric Blood and Cancer

IS - 6

ER -