Spectrum of Hereditary Hemolytic Anemia-A Tertiary Care Hospital Based Study
Journal Title: Scholars Journal of Applied Medical Sciences - Year 2017, Vol 5, Issue 6
Abstract
Abstract: Hereditary hemolytic anemias are broad category of hematological disorders that include hemoglobinopathies,enzymopathies and membrane disorders.Though most of the cases are clinically inapparent still hereditary hemolytic anemias particularly hemoglobin disorders are a considerable health problems in India and contributes significantly to morbidity and motality.These group of anemias have various clinical presentations with symptoms and signs.Failure to thrive,anemia,prostration,jaundice,spleenomegaly,cholelithiasis,cardiomegaly,severe life threatening infections and chronic disabilities leading to distress in the families. An analysis of 135 cases of hereditary hemolytic anemias were done in the present study.On the basis of clinical presentation physicalfindings,routine hematological investigations and hemoglobin electrophoresis pattern in hemoglobin defects were carried out to identify the type of hemolytic anemias. This clinicohematological study of hereditary hemolytic anemias showed 34(25.1%)cases of G6PD deficiency.The remaining 101(74.9%) cases were having diseases affecting hemoglobin molecule which included 58cases of sickle cell trait,17cases of sickle cell disease,4() cases of sickle beta thalassemias, 2 cases of Hbs/HPFH,16 cases of thalassemia trait and 4 cases of HbEdisorders.Out of 135 patients 75(55.5%) were males and 60 (44.5%)were females(M:F - 1.9:1). All the patients were from Eastern ODISHA.Family history was positive in 15 patients.Hereditary Hemolytic anemias with membrane defects were not observed in this study.Majority of the patients presented with progressive pallor and hepatospeenomegaly.Peripheral blood smear examination showed varied picture like microcytic hypochromic and normocytic normochromic anemias.Majority of the cases were associated with reticulocytosis.Hemoglobin electrophoresis confirmed the diagnosis in majority of cases.The basic hematological investigations remain the first panel or step towards the approach to diagnose hereditary hemolytic anemia in spite of advanced diagnostic investigations.Hemoglobin electrophoresis will help in confirming the diagnosis. In many cases the hematological findings may be subtle and need careful evaluation including family studies. Keywords:Hereditary hemolytic anemia,clinicohematologicalstudy,hemoglobin electrophoresis.
Authors and Affiliations
Ranjana Giri, Sweta Susmita, M A Khan, Urmila Senapati
Keywords
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