Abstract
Introduction: Hemolytic anaemias are a group of disorders that cause significant morbidity in children. Method: A cross sectional study was conducted at NRI Medical College for a period of 1 year. All cases of newly diagnosed and old cases of hemolytic anaemia on follow up were included. Results: The study showed beta thalassemia as the most common hemolytic anaemia; followed by malaria, sickle beta thalassemia, thalassemia intermedia, beta thalassemia minor, Sickle cell disease, sickle cell trait, auto immune hemolytic anaemia, and hereditary spherocytosis. The mean hemoglobin at presentation was 5.39 gm/dl. Anthropometric measurements in 32 cases of congenital hemolytic anaemias revealed height < 3rd centile in 9 cases. Weight less than 3rd centile was seen in 11 cases. 13 children had hemolytic facies. Massive splenomegaly causing discomfort, gall stones, heart failure were seen in 2 cases each. In thalassemia major, 10 cases required frequent transfusions, [10-12 per year]. 9 came for less frequent transfusions [6 per year]. Sickle thalassemia, thalassemia intermedia, required one transfusion every 1-2 years. Occasional transfusions were given in sickle cell anaemia, hereditary spherocytosis. Serum ferritin levels varied between 220-1427. Conclusion: Hemoglobin electrophoresis remains the main investigation of choice in diagnosis of hemolytic anaemia. Thalassemia major is the most severe among other hemolytic anaemias encountered in this series. The study emphasizes the need to improve awareness regarding hemoglobinopathies among population, prenatal screening, blood transfusion policies, chelation policies to prevent complications in transfusion dependent patients.
Highlights
Hemolytic anaemias are a group of disorders that cause significant morbidity in children
Inherited RBC defects of structure and metabolism may result in chronic hemolysis state that include hemoglobinopathies like sickle cell anaemia, alpha thalassemia, beta thalassemia, RBC enzyme defect like G6PD, RBC membrane disorder like hereditary spherocytosis [3]
A total of 41 cases of hemolytic anaemia were included in the study
Summary
Hemolytic anaemias are a group of disorders that cause significant morbidity in children. 10 cases required frequent transfusions, [10-12 per year]. Thalassemia intermedia, required one transfusion every 1-2 years. Occasional transfusions were given in sickle cell anaemia, hereditary spherocytosis. Thalassemia major is the most severe among other hemolytic anaemias encountered in this series. Inherited RBC defects of structure and metabolism may result in chronic hemolysis state that include hemoglobinopathies like sickle cell anaemia, alpha thalassemia, beta thalassemia, RBC enzyme defect like G6PD, RBC membrane disorder like hereditary spherocytosis [3]. Malaria is highly endemic in India, presents as hemolytic anaemia with breakdown of RBCs. The mean age of presentation of homozygous thalassemia is 6 months. The only form of treatment available for thalassemia patients are regular transfusions, iron chelation therapy in an selected cases [5]
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