Management of Spherocytosis a Review of the Literature

2.3 Contact Hours Management of Spherocytosis A Review of the Literature

Anna Gould Schuettge, MSN, CPNP-PC

in these cases is often recommended. Splenomegaly is Abstract: This literature review seeks to provide an overview of the symptoms, surgical management, infection risk, and long-term care of found in most HS patients; however, without symp- children with hereditary spherocytosis (HS). HS is a hemolytic anemia, toms of anemia and hyperbilirubinemia, this finding ranging in severity from carriers who are asymptomatic to anemic and is of little clinical significance (Bolton-Maggs et al., hyperbilirubinemic patients requiring splenectomy. The gold standard 2012). In individuals with mild HS, the diagnosis of of surgical management for HS is total splenectomy, which potentiates HS is often not made unless a family history is identi- infection risk, particularly that of overwhelming post-splenectomy infection. Another surgical option is partial splenectomy, which may con- fied, the patient develops anemia during or after a viral serve some immune function but threatens return of disease symptoms. infection, or the patient develops gallstones, a com- Infection risk is managed with a vaccine regimen and prophylactic mon comorbidity. In one study, 79 of the 468 pediatric antibiotics for total or partial splenectomy. Long-term care and educa- study participants had gallstones; consequently, regu- tion remain essential for the HS patient’s well-being post-splenectomy. larly scheduled ultrasounds after the age of 5 for HS pa- KEY WORDS: hereditary spherocytosis, hemolytic anemia, splenectomy, pediatrics tients are recommended in order to detect gallstones (Bolton-Maggs et al., 2012). In more symptomatic in- HEREDITARY SPHEROCYTOSIS dividuals, a diagnosis is often made in the neonatal pe- Hereditary spherocytosis (HS) is a hemolytic anemia riod due to jaundice or in young adulthood due to in which affected individuals’ red blood cells (RBCs) symptoms of severe anemia (Perrotta et al., 2008). are spherical instead of round (Perrotta, Gallagher, & Mohandas, 2008). In healthy individuals, the membranes of SURGICAL MANAGEMENT RBCs contain an outer bilayer comprised of cholesterol and If symptoms of anemia and hyperbilirubinemia are in- phospholipids and an inner cytoskeleton; these layers dicated, the current recommendation for surgical man- are not attached. In patients with HS, the space be- agement of HS is splenectomy. The 2011 guidelines tween these layers is reduced due to deficiencies or dys- developed by the General Haemotology Task Force functions of one or more membrane proteins. The of the British Committee for Standards in Haemotology reduced space between layers results in spherical-shaped recommend laparoscopic total splenectomy after the RBCs (spherocytes) that struggle to pass through the age of 6 with concomitant removal of the gallbladder spleen and often become trapped (Bolton-Maggs et al., if gallstones are present (Bolton-Maggs et al., 2012). 2012). In a multi-institutional review in 2010, the inci- This surgery is ideally done laparoscopically: ‘‘the splenic dence of HS was estimated at 1 in 5,000 people in the vessels are divided using a scalpel...clips or surgical United States, making HS the most common disease of stapler. The spleen is caught in a specimen bag and the RBC membrane (Buesing et al., 2011). morcellated inside the bag so that the contents can be The clinical manifestations of HS are dependent on removed through the port’’ (Bolton-Maggs et al., 2012, the severity of disease and can range from symptom- p. 44). A cholecystectomy can be done laparoscopically free to severe. Mild HS manifests with compensated at the same time (Bolton-Maggs et al., 2012). normal hemoglobin levels but high reticulocyte counts Given the spleen’s function in the immune system, and normal bilirubin levels; splenectomy is most often it is not surprising that splenectomized patients have not indicated for these patients (Bolton-Maggs et al., an increased risk of infection (Casale & Perrotta, 2011). 2012). In cases of severe HS, hemoglobin levels can In particular, patients post total splenectomy risk over- be as low as 6–8 g/dl, with high reticulocyte counts over whelming post-splenectomy infection (OPSI), which 10% and bilirubin levels over 50 mmol/L; a splenectomy occurs in up to 4.4% of patients (Buesing et al., 2011). Consequently, there is research interest in the long- Anna Gould Schuettge, MSN, CPNP-PC term clinical outcomes of children who have under- The author declares no conflict of interest. Email: [email protected] gone partial splenectomy. Buesing et al. (2011) describes DOI: 10.1097/JPS.0000000000000010 a partial splenectomy:

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The spleen [is] partially devascularized to main- results (Das et al., 2014). Thrombocytosis and other tain flow either from the short gastric vessels or hypothesized mechanisms can put splenectomized segmental branches of the splenic artery to the patients at risk for pulmonary artery hypertension. superior pole or from the inferior branches of Therefore, HS patients post-splenectomy should have the splenic artery to the lower pole. Splenic a complete blood count drawn every 6–12 months transection [is] accomplished by a variety of (Das et al., 2014). techniques including sharp dissection, stapling, and cauterization. (p. 179) PREVENTION OF INFECTION While the obvious advantage of partial splenectomy In general, HS patients should be immunized according is preserving some of the spleen’s immune function, the to the national routine immunization schedule (Davies procedure jeopardizes tissue regrowth and the consequent et al., 2011). The most important diseases to vaccinate return of disease symptoms (Morinis, Dutta, Blanchette, against prior to splenectomy are Haemophilus influ- Butchart, & Langer, 2008). Moreover, a study by Morinis enza type B, meningococcal group C, and pneumococ- et al. (2008) found that laparoscopic partial splenec- cal disease. Vaccines against these diseases should be tomy (in comparison to laparoscopic total splenectomy) administered at least 2 weeks preoperatively or, less resulted in more hospital days and postoperative pain. ideally, 2 weeks postoperatively. See Figure 1 for im- As such, the current gold standard is laparoscopic total munization recommendations. splenectomy with management targeted toward pre- Presently, the use of prophylactic penicillin (or venting infections preoperatively, immediately postop- macrolide in the case of penicillin allergy) is supported eratively, and long term. in asplenic or hyposplenic patients until the age of 16, Long-term complications of pulmonary artery regardless of immunization status (Davies et al., 2011). hypertension and thromboembolism are also possible The recommended penicillin dose is Penicillin V 250 mg (Das et al., 2014). In one study, 94% of 231 adult patients BID (Price et al., 2006). The greatest risk for infection had thrombocytosis post-splenectomy (Troendle, Adix, is in the immediate postoperative state and in the first Crary, & Buchanan, 2007), and in another study of 19 3 years after surgery; the most offensive agent is pneumo- pediatric patients, 73% experienced these hematologic coccal disease (Price et al., 2006). It is generally accepted

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Copyright © 2014 American Pediatric Surgical Nursing Association. Unauthorized reproduction of this article is prohibited. that these patients should have an emergency supply Buesing K. L., Tracy E. T., Kiernan C., Pastor A. C., Cassidy L. D., of antibiotics on hand at all times (Davies et al., 2011). Scott J. P., Oldham K. T. (2011). Partial splenectomy for hereditary spherocytosis: A multi-institutional review. In the long term, the primary care provider (PCP) Journal of Pediatric Surgery, 46, 178–183. doi:10.1016/j must evaluate the HS patient annually at well-child .jpedsurg.2010.09.090 visits to deliver timely vaccinations, monitor growth, Casale M., & Perrotta S. (2011). Splenectomy for hereditary and ensure continued visits to a pediatric hematologist. spherocytosis: Complete, partial or not at all? Expert Review In a study by Grace, Mednick, and Neufeld (2009), the of Hematology, 4(6), 627–635. doi:10.1586/EHM.11.51 authors established that nearly 75% of HS patients at a DasA.,BansalD.,AhluwaliaJ.,DasR.,RohitM.K.,AttriS., ... particular pediatric hematology clinic were not up to date Marwaha R. K. (2014). Risk factors for thromboembolism and pulmonary artery hypertension following splenec- on vaccinations. Therefore, during these annual visits, the tomy in children with hereditary spherocytosis. Pediatric PCP should order a complete blood count and liver panel, Blood & Cancer, 61(1), 29–33. doi:0.1002/pbc.24766 fill antibiotic prophylaxis prescriptions, and reiterate Davies J. M., Lewis M. P. N., Wimperis J., Rafi I., Ladhani S., the importance of compliance (Bryant, 2010). Bolton-Maggs P. H. B., & British Committee for Standards In summary, HS is a hemolytic anemia often pres- in Haematology. (2011). Review of guidelines for the prevention and treatment of infection in patients with enting so severely that splenectomy is required. Post- an absent or dysfunctional spleen: Prepared on behalf of splenectomy, the PCP must develop an appropriate the British Committee for Standards in Haematology by a vaccination schedule for individual patients, manage working party of the Haemato-Oncology Task Force. British Journal of Haematology, 155,308–317. doi:10.1111/ j.1365- prophylaxis regimens, and educate patients and fami- 2141.2011p.08843.x lies about the ongoing risk of infection in order to prevent Grace R. F., Mednick R. E., & Neufeld E. J. (2009). Compli- OPSI. Future research should focus on the benefits of par- ance with immunizations in splenectomized individuals tial versus total splenectomy and should seek to increase with hereditary spherocytosis. Pediatric Blood & Cancer, understanding of the risks surrounding pulmonary ar- 52,865–867. doi:10.1002/pbc.21961 tery hypertension, a thromboembolism, and/or OPSI. Morinis J., Dutta S., Blanchette V., Butchart S., & Langer J. C. (2008). Laparoscopic partial vs. total splenectomy in children with hereditary spherocytosis. Journal of Pediatric Sur- gery, 43, 1649–1652. doi:10.1016/j.jpedsurg.2008.02.012 References Perrett K. P., Nolan T. M., & McVernon J. (2013). A licensed Advisory Committee on Immunization Practices. (2010). combined Haemophilus influenza type b-serogroups C Prevention of pneumococcal disease among infants and and Y meningococcal conjugate vaccine. Infectious Diseases – children—use of 13-valent pneumococcal conjugate vac- and Therapy, 2(1), 1 13. doi:10.1007/s40121-013-0007-5 cine and 23-valent pneumococcal polysaccharide vaccine. Perrotta S., Gallagher P. G., & Mohandas N. (2008). Hereditary CDC’s Morbidity and Mortality Weekly Report, 59(RR-11), spherocytosis. The Lancet, 372,1411–1426. doi: 10.1016/ 1–19. Retrieved from http://www.cdc.gov/mmwr/pdf/rr/ S0140-6736(08)61588-3 rr5911.pdf Price V. E., Dutta S., Blanchette V. S., Butchart S., Kirby M., Bolton-Maggs P. H. B., Langer J. C., Iolascon A., Tittensor P., Langer J. C., & Ford-Jones E. L. (2006). The prevention King M., & General Haematology Task Force of the British and treatment of bacterial infections in children with Committee for Standards in Haematology. (2012). Guide- asplenia or hyposplenia: Practice considerations at the lines for the diagnosis and management of hereditary Hospital for Sick Children, Toronto. Pediatric Blood & spherocytosis—2011 update. British Journal of Haematology, Cancer, 46,597–603. doi:10.1002/pbc.20477 – 156,37 49. doi:10.1111/j.1365-2141.2011.08921.x Troendle S. B., Adix L., Crary S. E., & Buchanan G. R. (2007). Bryant R. (2010). Anemias. In Tomlinson D. & Kline N. E. Laboratory markers of thrombosis risk in children with (Eds.), Pediatric oncology nursing (pp. 141–172). Heidelberg, hereditary spherocytosis. Pediatric Blood & Cancer, 49(6), Germany: Springer. 781–785. doi:10.1002/pbc.21319

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