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Journal of Medical Genetics logoLink to Journal of Medical Genetics
. 1974 Sep;11(3):275–279. doi: 10.1136/jmg.11.3.275

Haemoglobin H disease and β-thalassaemia: Clinical haematological and electrophoretic studies in a family from South Lebanon

Munib J Shahid 1,, Farid P Khouri 1, Itaf F Sahli 1
PMCID: PMC1013142  PMID: 4372355

Abstract

A family is described in which four sibs are affected with haemoglobin H disease. To our knowledge, this is the first instance where this disorder has been encountered in the Lebanon. In fact only a few cases have so far been reported from the Arab world.

All four sibs had typical haemoglobin H bands on electrophoretic examination, and characteristic intracorpuscular inclusion bodies were demonstrated in a variable proportion of their erythrocytes, as well as in cells from a younger sib and from the mother. The latter also had elevation of the Hb-A2 fraction, and it is suggested that the above family has a combination of α- and β-thalassaemia.

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Selected References

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

  1. GOLDBERG C. A. A new method for starch gel electrophoresis of human hemoglobins, with special reference to the determination of hemoglobin A2. Clin Chem. 1958 Dec;4(6):484–495. [PubMed] [Google Scholar]
  2. GOUTTAS A., FESSAS P., TSEVRENIS H., XEFTERI E. Description d'une nouvelle variété d'anémie hémolytique congénitale; etude hématologique, électrophorétique et génétique. Sang. 1955;26(9):911–919. [PubMed] [Google Scholar]
  3. HUEHNS E. R., SHOOTER E. M. HUMAN HAEMOGLOBINS. J Med Genet. 1965 Mar;2(1):48–90. doi: 10.1136/jmg.2.1.48. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. MOTULSKY A. G. Genetic and haematological significance of haemoglobin H. Nature. 1956 Nov 10;178(4541):1055–1056. doi: 10.1038/1781055b0. [DOI] [PubMed] [Google Scholar]
  5. Na-Nakorn S., Wasi P., Pornpatkul M., Pootrakul S. N. Further evidence for a genetic basis of haemoglobin H disease from newborn offspring of patients. Nature. 1969 Jul 5;223(5201):59–60. doi: 10.1038/223059a0. [DOI] [PubMed] [Google Scholar]
  6. RIGAS D. A., KOLER R. D., OSGOOD E. E. New hemoglobin possessing a higher electrophoretic mobility than normal adult hemoglobin. Science. 1955 Mar 11;121(3141):372–372. doi: 10.1126/science.121.3141.372. [DOI] [PubMed] [Google Scholar]
  7. SINGER K., CHERNOFF A. I., SINGER L. Studies on abnormal hemoglobins. I. Their demonstration in sickle cell anemia and other hematologic disorders by means of alkali denaturation. Blood. 1951 May;6(5):413–428. [PubMed] [Google Scholar]
  8. WASI P., NA-NAKORN S., SUINGDUMRONG A. HAEMOGLOBIN H DISEASE IN THAILAND: A GENETICAL STUDY. Nature. 1964 Nov 28;204:907–908. doi: 10.1038/204907a0. [DOI] [PubMed] [Google Scholar]

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