Partial Splenectomy for Hereditary Spherocytosis—What Do We Know and What Do We Need to Know?

Partial Splenectomy for Hereditary Spherocytosis—What Do We Know and What Do We Need to Know?

Rice Henry E, Tracy Elisabeth T
US Hematology Volume 2
Published: January 2010
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Abstract
Partial splenectomy is an alternative to total splenctomy for the treatment of children with hereditary spherocytosis (HS) and other congenital hemolytic anemias. Total splenectomy effectively controls the symptoms of the disease, but places children at risk for overwhelming postsplenectomy sepsis (OPSI), as well as other severe complications. A growing body of data suggests that partial splenectomy provides acceptable hematological outcomes compared with total splenectomy while preserving splenic function. In this article we briefly review the rationale for partial splenectomy, parameters to evaluate splenic function, and recent clinical data on outcomes after partial splenectomy. Although many questions remain regarding the role of partial splenectomy, it may be a promising alternative to total splenectomy in children with HS and other congential hemolytic anemias.

Keywords
Hereditary spherocytosis, partial splenectomy, splenomegaly, congenital hemolytic anemia
Disclosure: The authors have no conflicts of interest to declare.
Received: March 16, 2009 Accepted: April 27, 2009
Correspondence: Henry Rice, MD, Division of Pediatric Surgery, Box 3815, Duke University Medical Center, Durham, NC, 27710. E: rice0017@mc.duke.edu

Partial splenectomy has attracted increasing attention as an alternative to total splenectomy for the treatment of children with hereditary spherocytosis (HS) and other congenital hemolytic anemias. Total splenectomy, although quite effective for the treatment of HS, has been associated with a variety of significant complications, notably overwhelming post-splenectomy sepsis (OPSI).1,2 Other less recognized but potentially severe complications of splenectomy include increased risk for vascular thrombosis, vascular derangements such as hypertension and vascular disease, and various immunological impairments.3–7 Given these concerns, the use of partial splenectomy as an alternative surgical option for the treatment of HS is attractive, with the goal of providing acceptable long-term hematological outcomes as well as preserving splenic function. In this article, we discuss what is known about the efficacy and safety of partial splenectomy in the treatment of HS. We also critically examine both what is known and what is not known about the success of partial splenectomy in the preservation of splenic function in these children.

Hereditary Spherocytosis
HS is the most common inherited red blood cell (RBC) membrane disorder in Northern Europe and North America, with an incidence of one in 5,000 births. HS results from a deficiency or dysfunction of one of the constituents of the RBC cytoskeleton (most commonly ankyrin and spectrin), which leads to shortened RBC lifespan.8–11 Most children with HS have a benign clinical phenotype and will not require surgical intervention; however, a subset of patients develop severe clinical sequelae of their disease, including accelerated hemolysis, hemolytic crises, splenic sequestration, sepsis, and gallstones. These severely affected children with HS have traditionally been treated with total splenectomy, resulting in improvement of anemia, reduced rates of hemolysis, elimination of splenic sequestration, and alleviation of symptoms associated with splenomegaly.9


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Keywords:
Hereditary spherocytosis, partial splenectomy, splenomegaly, congenital hemolytic anemia, Hereditary spherocytic hemolytic anemia, Hereditary spherocytosis osmotic fragility, cell membrance proteins, Hereditary spherocytosis symptoms, laparoscopic surgery, splenic artery, mild splenomegaly, differential diagnosis splenomegaly, signs of splenomegaly,

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