Articles

Study of D/G Hemoglobin incidence in a sample population (single institution)

Abstract

Background:  The hemoglobin disorders constitute the most prevalent group of monogenic disease A findings consis¬tent with this is a hemoglobin/G variant indicating that the patient has HbD/G trait. Hb D/G traits are clinically benign. In this study, the epidemiology of HbD/G was reviewed in a single institution in Iran.
Methods and Materials: We conducted the epidemiological study at Aliasghar children's hospital and all patients with HbD/G were entered to this study. The hematological values, hemoglobin electrophoresis, peripheral blood smear and clinical findings were collected in the data special form.
Results: 
Among 11825 hemoglobin analysis performed, we detected 101 cases of hemoglobin D/G. There were 55.4% male and 44.6% female, median age 9±13.36 yrs. Homozygous of D/G was found in seven pa¬tients with splenomegaly, jaundice, mild anemia and Reticulocytosis. Heterozygote patients were as¬ymptomatic. There wasn't any correlation between HbD/G with serum ferritin, MCV, Hb and sex.
Conclusion: In Iran HbD/G is relatively benign condition with mild anemia, Poikylocytosis and minimal hemolysis.

Lukens JN: The abnormalhemoglobins: general princi- ples In Lee GR, Foerster J, et al (eds): Wintrobes Clinical Hematology, 10 th ed.Philadelphia: Lippincott Wil- liams& Wilkins. 1999:1329-1345

Sturgeon P, Itano HA: Clinical manifestation of inher- ited abnormal hemoglobins. The interaction of HbS with HbD. Blood1955; 10:389

Schneider RG, Ueda S, Alperin JB, et al: Hemoglobin Los Angeles in two Caucasian families: Hemoglobin SD disease and hemoglobin D Thalassemia. Blood 1968; 32:250-259

Cawein MJ, Lappat EJ, Brangle RW, et al: Hemoglo- bin S-D disease. Ann Intern Med1966; 64:62-70

Cohen PS, Israel MA: Basic molecular biology for the pediatric Hematologist/Oncologist .Am J Pediatr Hema- tol oncol 1989; 11:467

Honig GR, Adams JG: Human hemoglobin genetics. Vienna, Springer-verlag, 1986

Weatherall DJ, Clegg Jb, Higgs DR, et al: The Hemo- globinopathies. In: Scriver CR, Beaudet AL, Sly WS, et al: The metabolic basis of inherited disease. 6th ed. New York, McGraw-Hill, 1989

Beutler E: The sickle cell diseases and related disor- ders: Williams Hematology, 6ih ed .New York; McGraw- Hill, 2001: 581-605

Wang WC, Lukens JC: Sickle cell anemia and other sickling syndromes.Wintrobes Clinical Hematology, 10 th ed.Philadelphia: Lippincott Williams& Wil- kins.1999:1329-1345

Harmening DM: Hemolytic anemias, intracorposcular defects: the Hemoglobinopathies. Clinical Hematology and Fundamentals of Hemostasis, 3rd ed. Philadelphia: FA Davis, 1997:173-192

Rabinovitch A: Hemoglobinopathy survey set HG- B.Northfield, MN: CAP, 1992:2-3

Ahmed M, Stuhrmann M, Bashawri L, Kuhnau W: The beta – globin genotype in hemoglobin D. Ann- Hematol .2001 NOV; 80(11): 629-33

Almeida AM, Henthorn JS, and Davies SC: Neonatal screening for Hemoglobinopathies. Br –J- Hematol, 2001 Jan; 112 (1): 32-35

Thornburg CD, Zimmerman SA, Schultz WH, and Ware RE: An infant with homozygous Hemoglobin D Iran, J-Ped- Hematol –Oncol.2001 Jan; 23(1): 67-8

Angastiniotis M, Modell B: Global epidemiology of Hemoglobin disorders: Annals, 1998; 850:251-269

Ahmed M, Petrou M: Screening extended families for genetic hemoglobin disorders in Pakistan; NEJM, 2002, vol 347(15):1162-1168

Primary health care approaches for the prevention and control of congenital and genetic disorders: report of a WHO meeting, Cairo, Egypt, 6-8 December 1999. Ge- neva: World Health Organization, 2000. (Document no. WHO/HGN/WG/OO.l.)

Alwan A, Modell B. Community control of genetic and congenital disorders. EMRO technical publication series 24 Alexandria, Egypt: WHO Regional Office for the Eastern Mediterranean, 1997

Bittles AH. The role and significance of consanguin- ity as a demographic variable. Popul Dev Rev 1994; 20:561-84

Khattak MF, Saleem M. Prevalence of heterozygous (beta)-thalassaemia in the northern areas of Pakistan Assoc] 992; 42:32-4.

The laboratory diagnosis of Hemoglobinopathies. Br J Hematol 998;] 0 I: 783-92.

Steinberg MH, Adams JG III. Haemoglobin A (J}: origin, evolution and aftermath. Blood 1991; 78:2165- 77.

Ahmed S, Petrou M, Saleem M. Molecular genetics of (beta)-thalassaemia in Pakistan: a basis for prenatal diagnosis. 3r J Haematol 1996; 94:476-82.

Mouzouras M, Camba L, Ioannou P, Modell B, Con- stantinides P, Gale R. Thalassaemia as a model of reces- sive netic disease in the community. Lancet 1980; 2:5

Ra.iab A, Patton MA. Analysis of the population structure in Oman. Community Genet 1999; 2:23-6.

Steensma DP, Hoyer JD: Hereditary Red Blood Cell disorders; Mayo Clininc Proceedings. 2001, 76(3):285- 293

Persons DA, TisdaleJF: Gene therapy for the hemo- globin disorders; Seminar in hematology, 2004, 41:279- 286

Files
IssueVol 2, No 1 (2005) QRcode
SectionArticles
Keywords
Hemoglobin D/G Hemoglobinopathis Iran

Rights and permissions
Creative Commons License This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
How to Cite
1.
Arjmandi K, Faranoush M, Bahoush G, Vossough P, Hedayatiasl A, Ansari S, Shahgholi E. Study of D/G Hemoglobin incidence in a sample population (single institution). Int J Hematol Oncol Stem Cell Res. 1;2(1):23-25.