Year : 2019 | Volume
: 10 | Issue : 4 | Page : 134--140
Double heterozygosity for hemoglobin S and D Punjab in Basra, Iraq: A Clinical and hematological study of 42 patients
Zuhair Abd Ali Al-Barazanchi1, Subeh Salim Abdulateef2, Meaad Kadhum Hassan3, Rawshan Zuhair Jaber5
1 Department of Laboratory, Basra Children Specialty Hospital, Al-Nahrain Medical College, Baghdad, Iraq
2 Department of Pathology and Forensic Medicine, Al-Nahrain Medical College, Baghdad, Iraq
3 Department of Pediatrics, College of Medicine, University of Basra; Center for Hereditary Blood Diseases, Basra Health Directorate, Basra, Iraq
BACKGROUND: Patients with compound heterozygosity for sickle hemoglobin (HbS) and hemoglobin DPunjab (Hb SDPunjab) may present with a variable clinical course and may be indistinguishable from those with homozygous HbS.
OBJECTIVES: The objective was to identify Hb SDPunjab phenotypes and the association of fetal Hb with disease severity.
PATIENTS AND METHODS: This descriptive, cross-sectional study included 42 (17 males and 25 females) patients with double heterozygosity for HbS and D. In addition to full clinical data, the complete blood count and Hb quantitation were assessed. Statistical analyses were performed using SPSS software version 20.0.
RESULTS: The mean age at diagnosis was 4.6 ± 0.7 years. Pallor and acute painful episodes (>90%) were the most common presenting symptoms, followed by jaundice (76.2%). Six patients (14.3%) had acute splenic sequestration crises and five (11.9%) had cardiac complications. Of the 42 studied patients, 17 (40.5%) had nonsevere disease and 25 (59.5%) had severe disease. Patients with severe disease had statistically significantly more blood transfusions (47.4 ± 10.27) than those with nonsevere disease (17.14 ± 1.82, P < 0.001). No significant association was reported between HbF level and either the number of blood transfusions or acute painful episodes requiring hospitalization/year.
Conclusion: Double heterozygosity for Hb S and DPunjab in Basra results in SCD with a severe phenotype, and the Hb F level did not modulate the disease severity.
Dr. Meaad Kadhum Hassan
Department of Pediatrics, College of Medicine, University of Basra, Basra
|How to cite this article:|
Ali Al-Barazanchi ZA, Abdulateef SS, Hassan MK, Jaber RZ. Double heterozygosity for hemoglobin S and D Punjab in Basra, Iraq: A Clinical and hematological study of 42 patients.J Appl Hematol 2019;10:134-140
|How to cite this URL:|
Ali Al-Barazanchi ZA, Abdulateef SS, Hassan MK, Jaber RZ. Double heterozygosity for hemoglobin S and D Punjab in Basra, Iraq: A Clinical and hematological study of 42 patients. J Appl Hematol [serial online] 2019 [cited 2020 Sep 25 ];10:134-140
Available from: http://www.jahjournal.org/article.asp?issn=1658-5127;year=2019;volume=10;issue=4;spage=134;epage=140;aulast=Ali;type=0