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Clinical-Hematological Profile of Indian Hemoglobin Sickle Cell D in Punjab Patients

Pandey S, Ranjan R, Seth T, Shah V, Mishra RM, Pandey SW and Saxena R

Hb D heterozygous is a clinically silent condition, but co-inheritance of Hb D with Hb S or beta thalassemia produces clinically significant conditions like sickle cell anemia and chronic hemolytic anemia of moderate severity. Complete blood count and red cell indices were measured by automated cell analyzer. Quantitative assessment of hemoglobin Hb F, HbA, HbA2, HbS and HbD was performed by high performance liquid chromatography (HPLC) DNA extraction done by phenol-chloroform method. Molecular study for common alpha deletions were done by Gap –PCR. Patients with Hemoglobin S-D disease were show the severity of the disease. These patients had sickle crises and moderate hemolytic anemia. Frequency of anemia (84.61%), Splenomegaly (61.53%) and Jaundice (44.15%)were high in the HbSD patient. 38.46% patients were transfusion dependent. HbS-D patients red cell indices were suggestive like sickle cell anemia patients while their clinically behavior showed a degree of variability.