Skip to main content
SpringerLink
Log in

Comparison and evaluation of three screening tests of hereditary spherocytosis in Chinese patients

  • Original Article
  • Published:
Annals of Hematology Aims and scope Submit manuscript

Abstract

The objective of this study is to compare and evaluate the diagnostic value of hereditary spherocytosis (HS) by three screening tests, comparing mean spherical corpuscular volume (MSCV) to mean corpuscular volume (MCV), mean corpuscular hemoglobin concentration (MCHC), and flow cytometric osmotic fragility test. Peripheral blood was collected from 237 participators diagnosed at the First Affiliated Hospital of Guangxi Medical University, including 56 hereditary spherocytosis patients, 86 thalassemia patients, and 95 healthy people. The samples were examined by three tests, and the three screening tests were evaluated by the sensitivity and specificity of tests. The sensitivity was only 41.07 %, and specificity was 94.47 % when using MCHC >355 g/L as diagnostic criteria. The sensitivity was 89.28 %, and specificity was 96.14 % when using MSCV < MCV as the optimum cutoff point. When using the residual red cell percentage <23.6 % as the diagnostic threshold in flow cytometric osmotic fragility test, the sensitivity was 85.71 % and the specificity was 97.24 %. Flow cytometry osmotic fragility test or comparing MSCV to MCV combined with smear examination of peripheral red blood cells morphology can be a simple, practical, and accurate hereditary spherocytosis (HS) laboratory screening method.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3

Similar content being viewed by others

References

  1. Perrotta S, Gallagher PG, Mohandas N (2008) Hereditary spherocytosis. Lancet 372(9647):1411–1426

    Article  CAS  PubMed  Google Scholar 

  2. Hayag-Barin JE, Smith RE, Tucker FC Jr (1998) Hereditary spherocytosis, thrombocytosis, and chronic pulmonary emboli: a case report and review of the literature. Am J Hematol 57(1):82–84

    Article  CAS  PubMed  Google Scholar 

  3. Kar R, Saxena R, Pati HP (2008) Hereditary spherocytosis with high fetal hemoglobin: an interesting case. Hemoglobin 32(5):520–523

    Article  CAS  PubMed  Google Scholar 

  4. Afshar A (2012) A case of Kienbock’s disease and hereditary spherocytosis anaemia. J Hand Surg Eur 37(5):466–467

    Article  Google Scholar 

  5. Oliveira MC, Fernandes RA, Rodrigues CL, Ribeiro DA, Giovanardi MF, Viana MB (2012) Clinical course of 63 children with hereditary spherocytosis: a retrospective study. Rev Bras Hematol Hemoter 34(1):9–13

    Article  PubMed Central  PubMed  Google Scholar 

  6. Bianchi P, Fermo E, Vercellati C, Marcello AP, Porretti L, Cortelezzi A et al (2012) Diagnostic power of laboratory tests for hereditary spherocytosis: a comparison study in 150 patients grouped according to molecular and clinical characteristics. Haematologica 97(4):516–523

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  7. Kutter D (2005) Hereditary spherocytosis is more frequent than expected: what to tell the patient? Bull Soc Sci Med Grand Duche Luxemb 1:7–22

    PubMed  Google Scholar 

  8. Bolton-Maggs PH, Stevens RF, Dodd NJ, Lamont G, Tittensor P, King MJ et al (2004) Guidelines for the diagnosis and management of hereditary spherocytosis. Br J Haematol 126(4):455–474

    Article  CAS  PubMed  Google Scholar 

  9. Bolton-Maggs PH, Langer JC, Iolascon A, Tittensor P, King MJ (2012) General Haematology Task Force of the British Committee for Standards in H. Guidelines for the diagnosis and management of hereditary spherocytosis—2011 update. Br J Haematol 156(1):37–49

    Article  PubMed  Google Scholar 

  10. Won DI, Suh JS (2009) Flow cytometric detection of erythrocyte osmotic fragility. Cytometry B Clin Cytom 76(2):135–141

    Article  PubMed  Google Scholar 

  11. Warang P, Gupta M, Kedar P, Ghosh K, Colah R (2011) Flow cytometric osmotic fragility—an effective screening approach for red cell membranopathies. Cytometry B Clin Cytom 80(3):186–190

    Article  PubMed  Google Scholar 

  12. Demir A, Yarali N, Fisgin T, Duru F, Kara A (2002) Most reliable indices in differentiation between thalassemia trait and iron deficiency anemia. Pediatr Int Off J Japan Pediatr Soc 44(6):612–616

    Article  Google Scholar 

  13. Beyan C, Kaptan K, Ifran A (2007) Predictive value of discrimination indices in differential diagnosis of iron deficiency anemia and beta-thalassemia trait. Eur J Haematol 78(6):524–526

    Article  CAS  PubMed  Google Scholar 

  14. Broseus J, Visomblain B, Guy J, Maynadie M, Girodon F (2010) Evaluation of mean sphered corpuscular volume for predicting hereditary spherocytosis. Int J Lab Hematol 32(5):519–523

    Article  CAS  PubMed  Google Scholar 

  15. Lin FQTJ, Ju Y, Qiu YL (2010) The value of mean spherical corpuscular volume in diagnosis of hereditary spherocytosis. J Pract Med 26(14):2537–2539

    Google Scholar 

  16. Zarandona JM, Yazer MH (2006) The role of the Coombs test in evaluating hemolysis in adults. CMAJ Can Med Assoc J J Assoc Med Can 174(3):305–307

    Article  Google Scholar 

  17. Eber SW, Pekrun A, Neufeldt A, Schroter W (1992) Prevalence of increased osmotic fragility of erythrocytes in German blood donors: screening using a modified glycerol lysis test. Ann Hematol 64(2):88–92

    Article  CAS  PubMed  Google Scholar 

  18. Vettore L, Zanella A, Molaro GL, De Matteis MC, Pavesi M, Mariani M (1984) A new test for the laboratory diagnosis of spherocytosis. Acta Haematol 72(4):258–263

    Article  CAS  PubMed  Google Scholar 

  19. Mittler U, Radig K, Kluba U, Aumann V, Roppnack R (1993) Experience with the glycerol lysis test in acid medium in diagnosis of hereditary spherocytosis. Kinderarztliche Praxis 61(6):219–222

    CAS  PubMed  Google Scholar 

  20. Hoffmann JJ, Swaak-Lammers N, Breed WP, Strengers JL (1991) Diagnostic utility of the pre-incubated acidified glycerol lysis test in haemolytic and non-haemolytic anaemias. Eur J Haematol 47(5):367–370

    Article  CAS  PubMed  Google Scholar 

  21. King MJ, Zanella A (2013) Hereditary red cell membrane disorders and laboratory diagnostic testing. Int J Lab Hematol 35(3):237–243

    Article  PubMed  Google Scholar 

  22. Park ES, Jung HL, Kim HJ, Park SS, Bae SH, Shin HY et al (2013) Hereditary hemolytic anemia in Korea from 2007 to 2011: a study by the Korean Hereditary Hemolytic Anemia Working Party of the Korean Society of Hematology. Blood Res 48(3):211–216

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  23. Da Costa L, Galimand J, Fenneteau O, Mohandas N (2013) Hereditary spherocytosis, elliptocytosis, and other red cell membrane disorders. Blood Rev 27(4):167–178

    Article  PubMed  Google Scholar 

Download references

Acknowledgments

This study was supported by the National Natural Science Foundation of China (No. 81360263) and the Provincial Natural Science Foundation of Guangxi (2011GXNSFA018290).

Conflict of interest

The authors declare that they have no conflict of interest.

Author information

Authors and Affiliations

  1. Affiliated Tumor Hospital of Guangxi Medical University, Nanning, Guangxi, China

    Yi-feng Tao

  2. Department of Clinical Laboratory, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China

    Zeng-fu Deng, Lin Liao & Fa-quan Lin

  3. Department of Pediatric Laboratory, The First Affiliated Hospital of Guangxi Medical University, Nanning, Guangxi, China

    Yu-ling Qiu & Wen-qiang Chen

Authors
  1. Yi-feng Tao
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Zeng-fu Deng
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Lin Liao
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Yu-ling Qiu
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Wen-qiang Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Fa-quan Lin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Fa-quan Lin.

Additional information

The authors Yi-feng Tao and Zeng-fu Deng contributed equally to this work.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Tao, Yf., Deng, Zf., Liao, L. et al. Comparison and evaluation of three screening tests of hereditary spherocytosis in Chinese patients. Ann Hematol 94, 747–751 (2015). https://doi.org/10.1007/s00277-014-2270-2

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00277-014-2270-2

Keywords

Search

Navigation