Letter to Editor
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Significance of Early Onset Peaks on High Performance Liquid Chromatography |
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Sunita Sharma, Vandana Puri, Jagdish Chandra 1. Head, Department of Pathology, Lady hardinge Medical College, New Delhi, India. 2. Assistant Professor, Department of Pathology, Lady Hardinge Medical College, New Delhi, India. 3. Director Professor, Department of Pediatrics, Lady Hardinge Medical College, New Delhi, India. |
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Correspondence
Address : Dr. Vandana Puri, Assistant Professor, Department of Pathology, Lady Hardinge Medical College, New Delhi-110001, India. E-mail: drvandanapuri@gmail.com |
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Keywords : Electrophoresis, Hemoglobinopathies, Target cells | |||||||||||
INTRODUCTION | |||||||||||
Sir, High Performance Liquid Chromatography is a rapid and accurate mean for early diagnosis of various hemoglobinopathies. However, availability and applicability of HPLC in Indian scenario is very fragmented and non uniform. Alpha - thalassemias are group of hemoglobinopathies with defective a chain synthesis. Diagnosis of a-thalassaemias trait can be difficult as there are no abnormalities detectable by haemoglobin electrophoresis or high performance liquid chromatography (HPLC). We report here two cases of HbH diagnosed with the help of HPLC and supravital staining. Both the cases were children and presented with pallor and hepatosplenomegaly. Their haematological indices are shown in (Table/Fig 1). On peripheral smear examination, both of them showed microcytic hypochromic red cells with marked anisopoikilocytosis and many polychromatophils. Few target cells and occasional nucleated red cells were also present. HPLC (Biorad variant II) in both these cases showed sharp significant peaks before the start of integration and low HbA2 (Table/Fig 2). Reticulocyte preparation with 1% new methylene blue showed reticulocytosis along with typical golf ball inclusions (Table/Fig 3). These patients were provisionally diagnosed as HbH disease and family studies, Hb electrophoresis and globin chain analyses were advised. Family study was available in only one case (case no 2) as the other child was an orphan. Mother of the child showed normal chromatogram however father of the child showed decreased HbA2 on HPLC along with golf ball inclusions in approximately 10% of red cells. HbH disease is a common entity in Southeast Asia with the frequency varying from 4% to 30% in varying studies (1),(2) however, is usually under-reported in Indian subcontinent (3). HbH dise`ase is caused by the presence of only one functional a gene which occurs due to compound heterozygous state for a 0/ a + thal (--/-a /--/ a T a). Decreased a chains and relative excess of ß chains leads to formation of tetramers of ß globin chains (HbH). This HbH is unstable and precipitates in red cells causing hemolysis. Also high oxygen affinity leads to hypoxia disproportionate to Hb level (4). Hence, in under resourced laboratory, where gene analysis is not possible, reticulocyte preparation showing typical inclusions and HPLC can help in early detection of HbH disease. | |||||||||||
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TABLES AND FIGURES | |||||||||||