Study of clinical presentation, laboratory profile, transfusion requirements and transfusion-related infectious complications in adult patients of congenital hemolytic anemia in a tertiary care center

Abstract

Congenital hemolytic anemia (CHA) especially hemoglobinopathies is quite prevalent in certain ethnic groups in India and commonly seen in pediatric age group. Adult presentations are being seen due to better treatment options and increased life span. This study was conducted to determine the clinical profile, laboratory parameters, transfusion requirements, and transfusion-related infectious complications of adult patients with CHA. A total of 84 patients of CHA aged more than 12 years were enrolled and studied over 18 months period. 63% patients were males with mean age of 19.98±4.99 SD years and maximum (57%) in the age group of 13-20 years. Family history of CHA was present in 19 (22.61%) patients and consanguinity in 16 (19.04%) cases which were statistically significant (P<0.05). Easy fatigability (76.19%) was the most common symptom followed by dyspnea on exertion (36.19%), whereas pallor (98.80%) was the commonest finding on examination followed by spleenomegaly (40.47%). The mean average (mean±SD) values for hemoglobin was 7.37±1.23gm/dl, Red blood cell count was 4.71±0.44 million/μL, Mean Corpuscular Volume was 77±5.05 fL, Mean Corpuscular Hemoglobin concentration was 33±1.38 g/dl, Mean Corpuscular Hemoglobin was 23±1.22 pg and mean Mentzer’s index was 16.59±2.24. The most common type of CHA in our study was β thalassemia major (58%), followed by β thalassemia intermedia (17%) and Sickle cell homozygous (13%). 45% of CHA patients required monthly transfusion (p <0.05) especially sickle cell homozygous (90%) and thalassemia major (65%) patients. 24 (28.57%) patients were Hepatitis C virus-positive and 1 was HIV virus-positive. Among adult patients with CHA, β thalassemia major, intermedia and sickle cell anemia were predominant diagnoses. Consanguinity played a role in one-fifth of patients. A majority of the patients had monthly blood transfusion requirements. The high prevalence of hepatitis C virus infection is a major concern.