The Prevalence of Hereditary Elliptocytosis in IraqKerbala
Keywords:
Hereditary Elliptocytosis, Red Cell Membrane Disorders.Abstract
Background: Hereditary elliptocytosis is a well-known red cell membrane disorder that is usually asymptomatic with difficulty to determine the actual incidence. Aim: This work aims to assess the prevalence of hereditary elliptocytosis in Iraq-Kerbala. Materials and methods: In this cross-sectional prospective study 10000 persons were examined by hematologist during the period from January 2014 to March 2022 in a specialized legal private laboratory for hematological investigations in Iraq/Kerbala through doing full blood count and blood film. Uncorrected reticulocyte count, serum total bilirubin and indirect bilirubin were done only for cases that are detected to have hereditary elliptocytosis with daily record for the presence or absence of hereditary elliptocytosis. Cases of hereditary elliptocytosis are defined when there are elliptocytes or ovalocytes that constitute 25%-100% of total red cells in stained blood film when other causes of elliptocytosis are excluded. All visitors (males and females, any age) from Iraq-Kerbala who were coming for that private laboratory during the period of study who need complete blood count and blood film for general checking or referred from medical clinics or hospitals for diagnosis or follow up of certain disease were selected for the study.Results: Three cases out of 10000 are discovered to have hereditary elliptocytosis (3/10000). 1/3 cases were discovered incidentally during general checking while the other 2/3 cases had disorders (presented for treatment of another diseases, namely chronic renal failure and urinary tract infection). 1/3 of cases had Anemia while the other 2/3 cases had normal Hb levels. 2/3 of cases (66.66%) had slightly raised uncorrected reticulocyte count, slightly raised serum total bilirubin and serum indirect bilirubin, reflecting the presence of mild hemolysis. Conclusion: The prevalence of hereditary elliptocytosis in Iraq-Kerbala is 3.0/10000 of population.
Downloads
References
Shirlyn Mckenzie, Kristin Landis-Piwowar and Lynne Williams: Clinical
laboratory hematology, 4th ed, chapter 17, hemolytic anemia:
membrane defects by Diana Cochran-Black, DRPH
Kakkar N, Singh GD, Dhanoa J. A case study (hematology): Abnormal
red blood cell morphology in an elderly male. Laboratory Medicine
Sep; 9(35):534-536.
Gallagher PG, Jarolim P: Red cell membrane disorders. In Hoffman R,
Benz E J , Shattil S J et al: (eds): Hematology Basic Principles and
Practice, 3rd ed. Philadelphia, Churchill Livingstone, 2000, p 576.Andolfo I, Russo R, Gambale A, lolascon A. New insights on hereditary
erythrocyte membrane defects. Haematologica. 2016 Nov. 101
(11):1284-1294.
PS Sharmila, Kannupriya, MF Paul: Hereditary Elliptocytosis, case
report. The Journal ofMedical Sciences, April-June 2015; 1(2):41-43.
Bain, Barbara. Red cell membrane disorders. In: Blood Cells: A practical
guide. 4th ed. Wiley-Blackwell; 2006. p. 278-281.
A. Victor Hoffbrand, S. Mitchell Lewis and Edward G.D Tuddenham:
Postgraduate hematology, fourth edition; Wiley Blackwell; 1999;
USA; chapter 7; page 132.
William T. Tse, Patrick G. Gallagher, Patricia B. Jenkins et al: Amino¬
acid substitution in a spectrin commonly coinherited with
nondominant hereditary spherocytosis. American Journal of
Hematology 54:233-241 (1997).
Mohandas N, Gallagher PG. Red cell membrane: past, present, and
future. Blood. 2008; 112(10):3939-3948.
Landis-Piwowar K, Landis J, Keila P. The complete blood count and
peripheral blood smear evaluation. In: Clinical laboratory
hematology. 3rd ed. New Jersey: Pearson; 2015. p. 154-77.
Manchanda N. Anemias: red blood morphology and approach to diagnosis. In: Rodak’s hematology clinical applications and principles. 5th ed. St. Louis, Missouri: Saunders; 2015. p. 284-96.
Gallagher PG. Hereditary elliptocytosis: spectrin and protein 4.1R. Semin Hematol. 2004; 41:142-164.
Paola Bianchi, Narla Mohandas: Hereditary disorders of the red cell membrane and disorders of red cell metabolism: In Postgraduate hematology, Seventh edition; Wiley Blackwell; 2016; New Delhi, India; chapter 8; page 114
Downloads
Published
Issue
Section
License
You are free to:
- Share — copy and redistribute the material in any medium or format for any purpose, even commercially.
- Adapt — remix, transform, and build upon the material for any purpose, even commercially.
- The licensor cannot revoke these freedoms as long as you follow the license terms.
Under the following terms:
- Attribution — You must give appropriate credit , provide a link to the license, and indicate if changes were made . You may do so in any reasonable manner, but not in any way that suggests the licensor endorses you or your use.
- No additional restrictions — You may not apply legal terms or technological measures that legally restrict others from doing anything the license permits.
Notices:
You do not have to comply with the license for elements of the material in the public domain or where your use is permitted by an applicable exception or limitation .
No warranties are given. The license may not give you all of the permissions necessary for your intended use. For example, other rights such as publicity, privacy, or moral rights may limit how you use the material.