History

Fact Explanation
Family history A normal red cell has a 120 days lifespan. The red cell skeletal network helps in the special deforming ability of the red cell plasma membrane. In elliptocytosis, weakened “horizontal” linkages in membrane skeleton due either to a defective spectrin dimer-dimer interaction or a defective spectrin-actin-protein 4.1R junctional complex causes elliptical shaped red blood cells. Hence, the red cells hemolyze faster than it's normal variant by the reticuloendothelial system. The disorder is inherited in an autosomal dominant manner, as a result, the family history of similar disease should be evident. [1] [2] [3] [4]
Incidental finding Most of the patients are asymptomatic and can be referred from another place due to an incidental finding of red cell morphology. [5] [6] [7]
Low hemoglobin levels despite treated with hematinics several times The patients with milder form of disease present with anemia for long time which has not been correctable with hematinic therapy. [7] [8] [9] [10]
Yellow discolouration of eyes and skin Due to hyperbilirubinemia following hemolysis. [6] [7] [11]
Shortness of breath Due to severe anemia, which is rare in hereditary elliptocytosis. [7] [8] [12]
Previous or current gallstones Pigment stones due to hemolysis. [13] [14]
References
  1. BARCELLINI W, BIANCHI P, FERMO E, IMPERIALI FG, MARCELLO AP, VERCELLATI C, ZANINONI A, ZANELLA A. Hereditary red cell membrane defects: diagnostic and clinical aspects Blood Transfus [online] 2011 Jul, 9(3):274-277 [viewed 09 August 2014] Available from: doi:10.2450/2011.0086-10
  2. DA COSTA LYDIE, GALIMAND JULIE, FENNETEAU ODILE, MOHANDAS NARLA. Hereditary spherocytosis, elliptocytosis, and other red cell membrane disorders. Blood Reviews [online] 2013 July, 27(4):167-178 [viewed 09 August 2014] Available from: doi:10.1016/j.blre.2013.04.003
  3. DELAUNAY JEAN, ALLOISIO NICOLE, MORLé LAURETTE, POTHIER BRIGITTE. The red cell skeleton and its genetic disorders. Molecular Aspects of Medicine [online] 1990 January, 11(3):161-241 [viewed 09 August 2014] Available from: doi:10.1016/0098-2997(90)90001-I
  4. MCGUIRE M, SMITH BL, AGRE P. Distinct variants of erythrocyte protein 4.1 inherited in linkage with elliptocytosis and Rh type in three white families. Blood [online] 1988 Jul, 72(1):287-93 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3134067
  5. COETZER T, LAWLER J, PRCHAL JT, PALEK J. Molecular determinants of clinical expression of hereditary elliptocytosis and pyropoikilocytosis. Blood [online] 1987 Sep, 70(3):766-72 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3620700
  6. GALLAGHER PATRICK G. Hereditary elliptocytosis: spectrin and protein 4.1R. Seminars in Hematology [online] 2004 April, 41(2):142-164 [viewed 09 August 2014] Available from: doi:10.1053/j.seminhematol.2004.01.003
  7. SCHOLNIK A. P., VAN TILBURG C. P., HOFFMAN G. C.. Hereditary elliptocytosis. Cleveland Clinic Journal of Medicine [online] 1974 March, 41(1):23-31 [viewed 09 August 2014] Available from: doi:10.3949/ccjm.41.1.23
  8. MARCHESI SL, KNOWLES WJ, MORROW JS, BOLOGNA M, MARCHESI VT. Abnormal spectrin in hereditary elliptocytosis. Blood [online] 1986 Jan, 67(1):141-51 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3940543
  9. GREENBERG LH, TANAKA KR. Hereditary elliptocytosis with hemolytic anemia--a family study of five affected members. Calif Med [online] 1969 May, 110(5):389-393 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1503499
  10. KAHWASH EIAD, BAUER PETER, YEARSLEY MARTHA. A Case of Hereditary Elliptocytosis. Laboratory Hematology [online] December, 13(3):113-114 [viewed 09 August 2014] Available from: doi:10.1532/LH96.07010
  11. SODERQUIST C., BAGG A.. Hereditary elliptocytosis. Blood [online] December, 121(16):3066-3066 [viewed 09 August 2014] Available from: doi:10.1182/blood-2012-09-457788
  12. PERROTTA SILVERIO, GALLAGHER PATRICK G, MOHANDAS NARLA. Hereditary spherocytosis. The Lancet [online] 2008 October, 372(9647):1411-1426 [viewed 09 August 2014] Available from: doi:10.1016/S0140-6736(08)61588-3
  13. GALLAGHER P. G.. Red Cell Membrane Disorders. Hematology [online] 2005 January, 2005(1):13-18 [viewed 09 August 2014] Available from: doi:10.1182/asheducation-2005.1.13
  14. TROTMAN BW, BERNSTEIN SE, BOVE KE, WIRT GD. Studies on the pathogenesis of pigment gallstones in hemolytic anemia: description and characteristics of a mouse model. J Clin Invest [online] 1980 Jun, 65(6):1301-8 [viewed 09 August 2014] Available from: doi:10.1172/JCI109793

Examination

Fact Explanation
Pallor Due to anemia, which is due to hemolysis of the un-deformable red cells in the reticuloendothelial system. [1] [2] [3] [4]
Icterus Due to hyperbilirubinemia in hemolysis. [5] [6] [7]
Splenomegaly Due to expansion of the reticuloendothelial system for excessive red blood cell destruction. [5] [8] [9]
Leg ulcers Due to clogging of undeformable red blood cells in the peripheral arteries. [10] [11]
References
  1. GALLAGHER P. G.. Red Cell Membrane Disorders. Hematology [online] 2005 January, 2005(1):13-18 [viewed 09 August 2014] Available from: doi:10.1182/asheducation-2005.1.13
  2. SCHOLNIK A. P., VAN TILBURG C. P., HOFFMAN G. C.. Hereditary elliptocytosis. Cleveland Clinic Journal of Medicine [online] 1974 March, 41(1):23-31 [viewed 09 August 2014] Available from: doi:10.3949/ccjm.41.1.23
  3. MARCHESI SL, KNOWLES WJ, MORROW JS, BOLOGNA M, MARCHESI VT. Abnormal spectrin in hereditary elliptocytosis. Blood [online] 1986 Jan, 67(1):141-51 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3940543
  4. PERROTTA SILVERIO, GALLAGHER PATRICK G, MOHANDAS NARLA. Hereditary spherocytosis. The Lancet [online] 2008 October, 372(9647):1411-1426 [viewed 09 August 2014] Available from: doi:10.1016/S0140-6736(08)61588-3
  5. GALLAGHER PATRICK G. Hereditary elliptocytosis: spectrin and protein 4.1R. Seminars in Hematology [online] 2004 April, 41(2):142-164 [viewed 09 August 2014] Available from: doi:10.1053/j.seminhematol.2004.01.003
  6. SCHOLNIK A. P., VAN TILBURG C. P., HOFFMAN G. C.. Hereditary elliptocytosis. Cleveland Clinic Journal of Medicine [online] 1974 March, 41(1):23-31 [viewed 09 August 2014] Available from: doi:10.3949/ccjm.41.1.23
  7. SODERQUIST C., BAGG A.. Hereditary elliptocytosis. Blood [online] December, 121(16):3066-3066 [viewed 09 August 2014] Available from: doi:10.1182/blood-2012-09-457788
  8. BRILL JR and BAUMGARDNER DJ. Normocytic Anemia. Am Fam Physician. [online] 2000 Nov 15;62(10):2255-2263. [viewed 09 August 2014] Available from:http://www.aafp.org/afp/2000/1115/p2255.html
  9. MORINIÈRE M et al. Elliptocytosis in patients with C-terminal domain mutations of protein 4.1 correlates with encoded messenger RNA levels rather than with alterations in primary protein structure. Blood [online] March 1, 2000; Blood: 95 (5) [viewed 09 August 2014] Available from: http://www.bloodjournal.org/content/95/5
  10. GIRALDI SUSANA, ABBAGE KERSTIN T., MARINONI LEIDE P., OLIVEIRA VANIA, PIANOWSKI MARA A., LEHMKUHL ADELINA E., NETO JOSE FILLUS. Leg Ulcer in Hereditary Spherocytosis. Pediatr Dermatol [online] 2003 September, 20(5):427-428 [viewed 09 August 2014] Available from: doi:10.1046/j.1525-1470.2003.20512.x
  11. RABHI SAMIRA, BENJELLOUNE HIND, MEZIANE MARIAM, AMRANI MONCEF, BERRADY RHIZLANE, MIKOU OUAFAE, MERNISSI FATIMA ZOHRA, BONO WAFAA. Hereditary Spherocytosis with Leg Ulcers Healing After Splenectomy. Southern Medical Journal [online] 2011 February, 104(2):150-152 [viewed 09 August 2014] Available from: doi:10.1097/SMJ.0b013e318200c6ba

Differential Diagnoses

Fact Explanation
Hereditary spherocytosis In both conditions, genetics, pathophysiology, clinical features and management are similar. The two entities can be differentiated from peripheral blood smear, in elliptocytosis there are elliptical cells lacking a central paleness, in spherocytosis there are spherocytes lacking a central paleness. But in elliptocytosis, the clinical course is somewhat milder. [1] [2] [3] [4] [5] [6] [7] [8] [9]
Other congenital hemolytic syndromes i.e. Glucose 6-Phosphate deficiency, Pyruvate kinase deficiency, Glutathione deficiency. Mainly due to absence of enzymes of red cell metabolism. These entities also should be evaluated and excluded by radioimmune assays. [10] [11] [12]
References
  1. SALOMAO M., CHEN K., VILLALOBOS J., MOHANDAS N., AN X., CHASIS J. A.. Hereditary spherocytosis and hereditary elliptocytosis: aberrant protein sorting during erythroblast enucleation. Blood [online] December, 116(2):267-269 [viewed 09 August 2014] Available from: doi:10.1182/blood-2010-02-264127
  2. DA COSTA LYDIE, GALIMAND JULIE, FENNETEAU ODILE, MOHANDAS NARLA. Hereditary spherocytosis, elliptocytosis, and other red cell membrane disorders. Blood Reviews [online] 2013 July, 27(4):167-178 [viewed 09 August 2014] Available from: doi:10.1016/j.blre.2013.04.003
  3. MAILLET P, ALLOISIO N, MORLé L, DELAUNAY J. Spectrin mutations in hereditary elliptocytosis and hereditary spherocytosis. Hum Mutat [online] 1996, 8(2):97-107 [viewed 09 August 2014] Available from: doi:10.1002/(SICI)1098-1004(1996)8:2<97::AID-HUMU1>3.0.CO;2-M
  4. DELAUNAY JEAN. Genetic disorders of the red cell membranes. FEBS Letters [online] 1995 August, 369(1):34-37 [viewed 09 August 2014] Available from: doi:10.1016/0014-5793(95)00460-Q
  5. BARCELLINI W, BIANCHI P, FERMO E, IMPERIALI FG, MARCELLO AP, VERCELLATI C, ZANINONI A, ZANELLA A. Hereditary red cell membrane defects: diagnostic and clinical aspects Blood Transfus [online] 2011 Jul, 9(3):274-277 [viewed 09 August 2014] Available from: doi:10.2450/2011.0086-10
  6. COETZER T, LAWLER J, PRCHAL JT, PALEK J. Molecular determinants of clinical expression of hereditary elliptocytosis and pyropoikilocytosis. Blood [online] 1987 Sep, 70(3):766-72 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/3620700
  7. GALLAGHER PATRICK G. Hereditary elliptocytosis: spectrin and protein 4.1R. Seminars in Hematology [online] 2004 April, 41(2):142-164 [viewed 09 August 2014] Available from: doi:10.1053/j.seminhematol.2004.01.003
  8. SCHOLNIK A. P., VAN TILBURG C. P., HOFFMAN G. C.. Hereditary elliptocytosis. Cleveland Clinic Journal of Medicine [online] 1974 March, 41(1):23-31 [viewed 09 August 2014] Available from: doi:10.3949/ccjm.41.1.23
  9. LYNCH EC. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd edition: Chapter 155; Peripheral Blood Smear. [web] [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/books/NBK263/
  10. VALENTINE WN. Hereditary hemolytic anemias associated with specific erythrocyte enzymopathies. Calif Med [online] 1968 Apr, 108(4):280-294 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1503104
  11. VALENTINE WN. Hereditary hemolytic anemias associated with specific erythrocyte enzymopathies. Calif Med [online] 1968 Apr, 108(4):280-94 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/4298122
  12. HIRONO A, IYORI H, SEKINE I, UEYAMA J, CHIBA H, KANNO H, FUJII H, MIWA S. Three cases of hereditary nonspherocytic hemolytic anemia associated with red blood cell glutathione deficiency. Blood [online] 1996 Mar 1, 87(5):2071-4 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pubmed/8634459

Investigations - for Diagnosis

Fact Explanation
Hemoglobin level Low hemoglobin level due to hemolysis. The structurally abnormal red cells are destroyed at the reticuloendothelial system when passing through capillaries, just like in hereditary spherocytosis. [1] [2] [3] [4]
Serum bilirubin level Higher than the normal level due to hemolysis. The pathology is the same as hereditary spherocytosis, but milder in form. [5] [6]
Blood picture Expected findings are elliptocytes, poikilocytes and nucleated red cells. The elliptocytes lack the central pallor. In cases where iron deficiency co-exists, there can be microcytes also. [7] [8] [9]
Reticulocyte count Higher reticulocyte level due to hemolysis. [7] [9]
Serum haptoglobins Done by rapid diagnostic testing. In hemolytic conditions haptoglobins are usually low or undetectable since they bind to free hemoglobin in hemolysis. [10] [11] [12] [13]
Lactic dehydrogenase Elevated in hemolytic anemias. [14] [15] [16]
Osmotic fragility test Osmotic fragility is increased because of red cell membrane instability. [17] Autohemolysis test, the hypertonic cryohemolysis test, and the acidified glycerol test can be used. [18]
Red blood cell skeletal structure In elliptocytosis, weakened “horizontal” linkages in membrane skeleton due either to a defective spectrin dimer-dimer interaction or a defective spectrin-actin-protein 4.1R junctional complex causes elliptical shaped red blood cells.
References
  1. CROOM RD 3RD, MCMILLAN CW, ORRINGER EP, SHELDON GF. Hereditary spherocytosis. Recent experience and current concepts of pathophysiology. Ann Surg [online] 1986 Jan, 203(1):34-39 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1251036
  2. HUQ S, PIETRONI MA, RAHMAN H, ALAM MT. Hereditary Spherocytosis J Health Popul Nutr [online] 2010 Feb, 28(1):107-109 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2975852
  3. WILEY JAMES S.. Red cell survival studies in hereditary spherocytosis. J. Clin. Invest. [online] 1970 April, 49(4):666-672 [viewed 09 August 2014] Available from: doi:10.1172/JCI106278
  4. GARWICZ S.. Atypical spherocytosis, a disease of spleen as well as of red blood-cells. The Lancet [online] 1975 April, 305(7913):956-957 [viewed 09 August 2014] Available from: doi:10.1016/S0140-6736(75)92012-7
  5. CHRISTENSEN R. D., HENRY E.. Hereditary Spherocytosis in Neonates With Hyperbilirubinemia. PEDIATRICS [online] December, 125(1):120-125 [viewed 09 August 2014] Available from: doi:10.1542/peds.2009-0864
  6. WREE A., CANBAY A., MüLLER-BEIßENHIRTZ H., DECHêNE A., GERKEN G., DüHRSEN U., LAMMERT F., NüCKEL H.. Excessive Bilirubin Elevation in a Patient with Hereditary Spherocytosis and Intrahepatic Cholestasis. Z Gastroenterol [online] December, 49(08):977-980 [viewed 09 August 2014] Available from: doi:10.1055/s-0031-1273368
  7. LYNCH EC. Clinical Methods: The History, Physical, and Laboratory Examinations. 3rd edition: Chapter 155; Peripheral Blood Smear. [web] [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/books/NBK263/
  8. CARPENTIERI U., GUSTAVSON L. P., HAGGARD M. E.. Pyknocytosis in a Neonate: An Unusual Presentation of Hereditary Elliptocytosis. Clinical Pediatrics [online] 1977 January, 16(1):76-78 [viewed 09 August 2014] Available from: doi:10.1177/000992287701600114
  9. KAHWASH EIAD, BAUER PETER, YEARSLEY MARTHA. A Case of Hereditary Elliptocytosis. Laboratory Hematology [online] December, 13(3):113-114 [viewed 09 August 2014] Available from: doi:10.1532/LH96.07010
  10. PRUEKSARITANOND SUARTCHA, BARBARYAN ARAM, MIRRAKHIMOV AIBEK E., LIANA PALACCI, ALI ALAA M., GILMAN ALAN D.. A Puzzle of Hemolytic Anemia, Iron and Vitamin B12 Deficiencies in a 52-Year-Old Male. Case Reports in Hematology [online] 2013 December, 2013:1-5 [viewed 09 August 2014] Available from: doi:10.1155/2013/708489
  11. MARCHAND ANTHONY. The Predictive Value of Serum Haptoglobin in Hemolytic Disease. JAMA [online] 1980 May [viewed 09 August 2014] Available from: doi:10.1001/jama.1980.03300450023014
  12. GUPTA SHILPI, AHERN KATHLEEN, NAKHL FADI, FORTE FRANK. Clinical Usefulness of Haptoglobin Levels to Evaluate Hemolysis in Recently Transfused Patients. Advances in Hematology [online] 2011 December, 2011:1-4 [viewed 09 August 2014] Available from: doi:10.1155/2011/389854
  13. CONFER BD. Questionable Diagnosis of Hemolytic Anemia? J Am Osteopath Assoc [online] July 1, 2008 vol. 108 no. 7 351-352. [viewed 09 August 2014] Available from: http://www.jaoa.org/content/108/7/351
  14. ZIMMERMAN HYMAN J.. Serum Enzymes in Disease. AMA Arch Intern Med [online] 1958 July [viewed 09 August 2014] Available from: doi:10.1001/archinte.1958.00260190117013
  15. MYHRE E., RASMUSSEN K., ANDERSEN A.. Serum lactic dehydrogenase activity in patients with prosthetic heart valves: A parameter of intravascular hemolysis. American Heart Journal [online] 1970 October, 80(4):463-468 [viewed 09 August 2014] Available from: doi:10.1016/0002-8703(70)90192-4
  16. STARKWEATHER WH et al. Alterations of Erythrocyte Lactate Dehydrogenase in Man. Blood. [online] July 1, 1965; Blood: 26 (1) [viewed 09 August 2014] Available from: http://www.bloodjournal.org/content/26/1/63.short
  17. BARCELLINI W, BIANCHI P, FERMO E, IMPERIALI FG, MARCELLO AP, VERCELLATI C, ZANINONI A, ZANELLA A. Hereditary red cell membrane defects: diagnostic and clinical aspects Blood Transfus [online] 2011 Jul, 9(3):274-277 [viewed 09 August 2014] Available from: doi:10.2450/2011.0086-10
  18. GALLAGHER P. G.. Red Cell Membrane Disorders. Hematology [online] 2005 January, 2005(1):13-18 [viewed 09 August 2014] Available from: doi:10.1182/asheducation-2005.1.13

Investigations - Fitness for Management

Fact Explanation
Complete blood count To evaluate the presence and development of anemia and hypersplenism. [1] [2]
Ultrasound scan of the abdomen To exclude spenomegaly. [3] [4] [5] [6]
References
  1. DAVIDSON RJ, STRAUSS WT. Hereditary elliptocytic anaemia J Clin Pathol [online] 1961 Nov, 14(6):615-621 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC480304
  2. GREENBERG LH, TANAKA KR. Hereditary elliptocytosis with hemolytic anemia--a family study of five affected members. Calif Med [online] 1969 May, 110(5):389-393 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1503499
  3. GALLAGHER PATRICK G. Hereditary elliptocytosis: spectrin and protein 4.1R. Seminars in Hematology [online] 2004 April, 41(2):142-164 [viewed 09 August 2014] Available from: doi:10.1053/j.seminhematol.2004.01.003
  4. BARCELLINI W, BIANCHI P, FERMO E, IMPERIALI FG, MARCELLO AP, VERCELLATI C, ZANINONI A, ZANELLA A. Hereditary red cell membrane defects: diagnostic and clinical aspects Blood Transfus [online] 2011 Jul, 9(3):274-277 [viewed 09 August 2014] Available from: doi:10.2450/2011.0086-10
  5. GALLAGHER P. G.. Red Cell Membrane Disorders. Hematology [online] 2005 January, 2005(1):13-18 [viewed 09 August 2014] Available from: doi:10.1182/asheducation-2005.1.13
  6. OHMAE H., KAWAMOTO F., ISHII A., LEAFASIA J., KERE N.. Detecting splenomegaly by ultrasound. The Lancet [online] 1991 September, 338(8770):826-827 [viewed 09 August 2014] Available from: doi:10.1016/0140-6736(91)90720-A

Management - General Measures

Fact Explanation
Pedigree analysis Since hereditary elliptocytosis is of autosomal dominant inheritance, it's important to analyze the family tree to find asymptomatic affected individuals. [1] [2]
Patient education Education about the nature of inheritance, clinical course and it's prognosis as well as the importance of family tree screening is necessary in good management. [3]
References
  1. BOLTON-MAGGS PH, STEVENS RF, DODD NJ, LAMONT G, TITTENSOR P, KING MJ, GENERAL HAEMATOLOGY TASK FORCE OF THE BRITISH COMMITTEE FOR STANDARDS IN HAEMATOLOGY. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol [online] 2004 Aug, 126(4):455-74 [viewed 09 August 2014] Available from: doi:10.1111/j.1365-2141.2004.05052.x
  2. HASSOUN H. et al. Characterization of the Underlying Molecular Defect in Hereditary Spherocytosis Associated With Spectrin Deficiency. Blood [online] April 1, 1997; 90 (1). [viewed 09 August 2014] Available from: http://www.bloodjournal.org/content/90/1/398.long?sso-checked=1
  3. PAULI RM, MOTULSKY AG. Risk counselling in autosomal dominant disorders with undetermined penetrance. J Med Genet [online] 1981 Oct, 18(5):340-343 [viewed 09 August 2014] Available from: http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1048754

Management - Specific Treatments

Fact Explanation
Folate therapy Supplementation with folate is recommended in severe and moderate forms of haemolytic anaemia. [1] [2]
Transfusions Red cell transfusions may be required in severely anaemic cases, particularly in the first years of life, and during pregnancy, aplastic crisis and infections. [1] [2]
Splenectomy Required only in severe forms. Pre-vaccination and post-splenectomy prophylaxis is necessary. Laparoscopy technique is now preferred. [3] [4] [5]
References
  1. BARCELLINI W, BIANCHI P, FERMO E, IMPERIALI FG, MARCELLO AP, VERCELLATI C, ZANINONI A, ZANELLA A. Hereditary red cell membrane defects: diagnostic and clinical aspects Blood Transfus [online] 2011 Jul, 9(3):274-277 [viewed 09 August 2014] Available from: doi:10.2450/2011.0086-10
  2. BOLTON-MAGGS PH, STEVENS RF, DODD NJ, LAMONT G, TITTENSOR P, KING MJ, GENERAL HAEMATOLOGY TASK FORCE OF THE BRITISH COMMITTEE FOR STANDARDS IN HAEMATOLOGY. Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol [online] 2004 Aug, 126(4):455-74 [viewed 09 August 2014] Available from: doi:10.1111/j.1365-2141.2004.05052.x
  3. BOLTON-MAGGS PAULA H. B., LANGER JACOB C., IOLASCON ACHILLE, TITTENSOR PAUL, KING MAY-JEAN. Guidelines for the diagnosis and management of hereditary spherocytosis - 2011 update. [online] December, 156(1):37-49 [viewed 09 August 2014] Available from: doi:10.1111/j.1365-2141.2011.08921.x
  4. MORINIS JULIA, DUTTA SANJEEV, BLANCHETTE VICTOR, BUTCHART SHEILA, LANGER JACOB C.. Laparoscopic partial vs total splenectomy in children with hereditary spherocytosis. Journal of Pediatric Surgery [online] 2008 September, 43(9):1649-1652 [viewed 09 August 2014] Available from: doi:10.1016/j.jpedsurg.2008.02.012
  5. ABDULLAH FIZAN, ZHANG YIYI, CAMP MELISSA, ROSSBERG MARK I., BATHURST MELINDA A., COLOMBANI PAUL M., CASELLA JAMES F., NABAWEESI ROSEMARY, CHANG DAVID C.. Splenectomy in hereditary spherocytosis: Review of 1,657 patients and application of the pediatric quality indicators. Pediatr. Blood Cancer [online] 2009 July, 52(7):834-837 [viewed 09 August 2014] Available from: doi:10.1002/pbc.21954