CLINICO-PATHOLOGICAL CORRELATION OF SICKLE HEMOGLOBIN (HBS)

Introduction:  Present study relates to the results of clinical examination of 10 patients with sickle hemoglobin. 

Material and Methods:All the patients belonged to TharuChaudhary community of Nepal.  The patients were clinically examined.   Sickling test was done using Sodium metasulphite.  Hemoglobin agarose gel electrophoresis was done at alkaline pH.  Total hemoglobin was estimated by cyanhemoglobin method. 

Results: Sickling test was positive in all the patients.  In addition, electrophoresis revealed detection of HbS in all the patients.  Four of 10 patients had homozygous sickle cell disease (βglobin genotype SS).  Five other patients had heterozygous sickle cell trait (βglobin genotype AS).  Another patient had double heterozygous sickle cell β+thalassemia; high level of fetal hemoglobin (16.4%) was also detected in this patient.

Discussion: Capillary thromboses might have resulted in minor infarcts, resulting in pain in chest and abdomen. 

Conclusion:The patients belonged to mid-western region of Nepal; this region is infested with vivax malaria.  High frequency of malaria in this region might have resulted in biological advantage for sickle hemoglobin patients.