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. 1982 Sep;137(3):186–190.

Hemoglobin E in Indochinese Refugees

Thomas M Cunningham
PMCID: PMC1274062  PMID: 7147932

Abstract

Hemoglobin E, a β-chain variant commonly found in Southeast Asian populations, is relatively unfamiliar to American physicians. The hematologic features of 55 patients with hemoglobin E (8 homozygote, 46 heterozygote and 1 genotype E/βthal) are described and compared with previous series. The patients were immigrants from Cambodia, Laos, Thailand and Vietnam. These studies support the association of this β-globin variant with microcythemia and suggest that it mimics the “thalassemia picture” of microcythemia out of proportion to the degree of anemia. Splenomegaly was not a characteristic. Oxidative hemolysis may occur under conditions that deplete reduced glutathione. The simultaneous presence of the β-thalassemia trait appears to significantly worsen the prognosis.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Benz E. J., Jr, Berman B. W., Tonkonow B. L., Coupal E., Coates T., Boxer L. A., Altman A., Adams J. G., 3rd Molecular analysis of the beta-thalassemia phenotype associated with inheritance of hemoglobin E (alpha 2 beta2(26)Glu leads to Lys). J Clin Invest. 1981 Jul;68(1):118–126. doi: 10.1172/JCI110226. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Bhamarapravati N., Na-Nakorn S., Wasi P., Tuchinda S. Pathology of abnormal hemoglobin diseases seen in Thailand. I. Pathology of beta-thalassemia hemoglobin E disease. Am J Clin Pathol. 1967 Jun;47(6):745–758. doi: 10.1093/ajcp/47.6.745. [DOI] [PubMed] [Google Scholar]
  3. Blackwell R. Q., Blackwell B. N., Huang J. T., Thephusdin C. Haemoglobin J and E in a Thai kindred. Nature. 1965 Aug 14;207(998):767–768. doi: 10.1038/207767a0. [DOI] [PubMed] [Google Scholar]
  4. Blackwell R. Q., Huang J. T., Chien L. C. Haemoglobin E in Vietnamese. Nature. 1965 Aug 14;207(998):768–768. doi: 10.1038/207768a0. [DOI] [PubMed] [Google Scholar]
  5. Bunn H. F., Meriwether W. D., Balcerzak S. P., Rucknagel D. L. Oxygen equilibrium of hemoglobin E. J Clin Invest. 1972 Nov;51(11):2984–2987. doi: 10.1172/JCI107125. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. CHATTERJEA J. B. MALARIA AND HAEMOGLOBIN E. Lancet. 1964 Nov 28;2(7370):1185–1185. doi: 10.1016/s0140-6736(64)92724-2. [DOI] [PubMed] [Google Scholar]
  7. CHERNOFF A. I., MINNICH V., NANAKORN S., TUCHINDA S., KASHEMSANT C., BANGKOK, THAILAND, CHERNOFF R. R. Studies on hemoglobin E. I. The clinical, hematologic, and genetic characteristics of the hemoglobin E syndromes. J Lab Clin Med. 1956 Mar;47(3):455–489. [PubMed] [Google Scholar]
  8. FLATZ G., PIK C., SUNDHARAGIATI B. MALARIA AND HAEMOGLOBIN E IN THAILAND. Lancet. 1964 Aug 22;2(7356):385–387. doi: 10.1016/s0140-6736(64)90392-7. [DOI] [PubMed] [Google Scholar]
  9. Fairbanks V. F., Gilchrist G. S., Brimhall B., Jereb J. A., Goldston E. C. Hemoglobin E trait reexamined: a cause of microcytosis and erythrocytosis. Blood. 1979 Jan;53(1):109–115. [PubMed] [Google Scholar]
  10. Fairbanks V. F., Oliveros R., Brandabur J. H., Willis R. R., Fiester R. F. Homozygous hemoglobin E mimics beta-thalassemia minor without anemia or hemolysis: hematologic, functional, and biosynthetic studies of first North American cases. Am J Hematol. 1980;8(1):109–121. doi: 10.1002/ajh.2830080112. [DOI] [PubMed] [Google Scholar]
  11. Feldman R., Rieder R. F. The interaction of hemoglobin E with beta thalassemia: a study of hemoglobin synthesis in a family of mixed Burmese and Iranian origin. Blood. 1973 Nov;42(5):783–791. [PubMed] [Google Scholar]
  12. Forget B. G. Molecular genetics of human hemoglobin synthesis. Ann Intern Med. 1979 Oct;91(4):605–616. doi: 10.7326/0003-4819-91-4-605. [DOI] [PubMed] [Google Scholar]
  13. Frischer H., Bowman J. Hemoglobin E, an oxidatively unstable mutation. J Lab Clin Med. 1975 Apr;85(4):531–539. [PubMed] [Google Scholar]
  14. May A., Huehns E. R. The oxygen affinity of haemoglobin E. Br J Haematol. 1975 Jun;30(2):177–184. doi: 10.1111/j.1365-2141.1975.tb00532.x. [DOI] [PubMed] [Google Scholar]
  15. NA-NAKORN S., BANGKOK, THAILAND, MINNICH V., CHERNOFF A. I. Studies on hemoglobin E. II. The incidence of hemoglobin E in Thailand. J Lab Clin Med. 1956 Mar;47(3):490–498. [PubMed] [Google Scholar]
  16. Perutz M. F., Lehmann H. Molecular pathology of human haemoglobin. Nature. 1968 Aug 31;219(5157):902–909. doi: 10.1038/219902a0. [DOI] [PubMed] [Google Scholar]
  17. TUCHINDA S., RUCKNAGEL D. L., MINNICH V., BOONYAPRAKOB U., BALANKURA K., SUVATEE V. THE COEXISTENCE OF THE GENES FOR HEMOGLOBIN E AND ALPHA-THALASSEMIA IN THAIS, WITH RESULTANT SUPPRESSION OF HEMOGLOBIN E SYNTHESIS. Am J Hum Genet. 1964 Sep;16:311–335. [PMC free article] [PubMed] [Google Scholar]
  18. Traeger J., Wood W. G., Clegg J. B., Weatherall D. J. Defective synthesis of HbE is due to reduced levels of beta E mRNA. Nature. 1980 Dec 4;288(5790):497–499. doi: 10.1038/288497a0. [DOI] [PubMed] [Google Scholar]
  19. Wasi P., Disthasongchan P., Na-Nakorn S. The effect of iron deficiency on the levels of hemoglobins A2 and E. J Lab Clin Med. 1968 Jan;71(1):85–91. [PubMed] [Google Scholar]
  20. Wasi P., Na-Nakorn S., Suingdumrong A. Studies of the distribution of haemoglobin E, thalassaemias and glucose-6-phosphate dehydrogenase deficiency in north-eastern Thailand. Nature. 1967 Apr 29;214(5087):501–502. doi: 10.1038/214501a0. [DOI] [PubMed] [Google Scholar]
  21. Wasi P., Sookanek M., Pootrakul S., Na-Nakorn S., Suingdumrong A. Haemoglobin E and alpha-Thalassaemia. Br Med J. 1967 Oct 7;4(5570):29–32. doi: 10.1136/bmj.4.5570.29. [DOI] [PMC free article] [PubMed] [Google Scholar]

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