Evaluation of correlation between clinical profile and amount of abnormal hemoglobin in sickle cell hemoglobinopathy

Abstract

Aim and objectives: The present research was undertaken to study the clinical presentation of sickle cell haemoglobinopathy cases and correlates its severity with the subtypes of hemoglobin quantified on high performance liquid chromatography (HPLC).

Method: Total 54 sickle cell anemia (SCA) cases of age >12 years were included in the study. A detailed history, complete clinical examination and hematological investigation were done. From each patient 3 ml of venous blood was withdrawn and mixed with EDTA and sent to pathology laboratory for HPLC. The correlation between clinical profile and quantity of abnormal Hb was determined.

Results: The common presenting symptom was vaso-occlusive crisis (VOC) (30; 55.55%) followed by severe anaemia (22; 40.74%), a vascular necrosis (15; 27.77%) and infections (11; 20.37%). HbF had statistically significant positive correlation with Hb% and mean age of onset of symptoms while had negative correlation with WBC count and frequency of VOC. We found negative correlation between Hb% and HbS level but it was not statistically significant, (p=0.231). Also negative correlation found between mean age of onset of symptoms and HbS level, (p<0.001). There was statistically significant positive correlation between WBC count, frequency of VOC and HbS level (p=0.0067).

Conclusion: Clinical profile of sickle cell anemia is observed to be moderate i.e. less severe in this region as compared to reported in African Countries. HbF levels are higher in SCA patients and high level of HbF has a definitive role in ameliorating the severity of sickle cell anemia. These results point strongly to the recommendation of the therapy to raise the HbF level in patients wherever it is low.