Medical and Quality of Life Indications for Splenectomy in Children with Hereditary Spherocytosis.

Splenectomy has been considered the treatment of choice for patients with “symptomatic” hereditary spherocytosis (HS). However, given the long-term risks associated with asplenia, including sepsis and thrombosis, the risks and benefits of splenectomy in HS require ongoing analysis. To our knowledge, no comprehensive institutional review summarizing the medical and quality of life indications for splenectomy in children with HS has been reported to date.

A retrospective record review was performed of 64 children, age ' 18 yrs, having splenectomy for HS at Children's Medical Center Dallas from January 1973 through March 2009. The following patient characteristics were abstracted: age at splenectomy, family history of HS and/or splenectomy, baseline hemoglobin concentration and reticulocyte count prior to splenectomy, degree of splenomegaly, total number of prior transfusions, hyperhemolytic events, and aplastic crisis. Patients were classified based on mean hemoglobin concentration as having mild (≥11.0 g/dL), moderate (8.0-10.9 g/dL), or severe HS ('7.9 g/dL) (Bolton-Maggs PH, et al. Br J Haematol 2004;126:455-74). The primary indication for splenectomy was characterized in each case as either a “quality of life (QOL) indication” or a “medical indication.” QOL indicators included jaundice, pallor, maxillary hyperplasia, poor growth, fatigue, exercise intolerance, and family history of splenectomy for HS which, having been deemed successful, fostered parental preference for splenectomy in their child. Medical indicators included baseline hemoglobin approaching transfusion-dependent threshold, multiple or severe hyperhemolytic events, severe aplastic crisis, symptomatic gallbladder disease, marked splenomegaly, and the need for numerous transfusions. Some patients had both medical and QOL indications but only one was designated as the primary indication for splenectomy based on documentation in the medical record.

When classifying HS patients by hemoglobin concentration, 16 patients (25%) were mild, 38 (59%) moderate, and 10 (16%) severe. The following table summarizes the patient characteristics.

Despite having just mild HS, 16 patients had splenectomy, 6 for QOL indications. Moreover, 11 of 38 moderate HS patients had splenectomy for a primary QOL indication. Medical indications predominated in all 10 patients with severe HS. Of the 17 patients with QOL indications who had splenectomy, 8 (47%) also had documentation of medical indications. Among 45 patients with primary medical indications for splenectomy, 18 (40%) also had QOL issues contributing to the decision. Two patients' indications for splenectomy were unclear.

Although splenectomy is generally felt to be unnecessary in patients with mild HS and many with moderate HS as well, our experience demonstrates that splenectomy is often performed primarily for QOL indications in both risk categories. A validated assessment tool that incorporates QOL indicators in addition to the traditional medical reasons for splenectomy in patients with HS would be of great value in an evidence-based assessment of the patient's need for splenectomy. We recommend inclusion of QOL indicators when considering splenectomy in patients with mild and moderate HS.

No relevant conflicts of interest to declare.