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  Table of Contents    
LETTER TO EDITOR  
Year : 2011  |  Volume : 54  |  Issue : 2  |  Page : 407
Role of HPLC in the detection of HbH disease


Department of Pathology, MAMC, New Delhi, India

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Date of Web Publication27-May-2011
 

How to cite this article:
Wadhwa R, Singh T. Role of HPLC in the detection of HbH disease. Indian J Pathol Microbiol 2011;54:407

How to cite this URL:
Wadhwa R, Singh T. Role of HPLC in the detection of HbH disease. Indian J Pathol Microbiol [serial online] 2011 [cited 2019 Dec 15];54:407. Available from: http://www.ijpmonline.org/text.asp?2011/54/2/407/81590


Sir,

We have gone through the paper entitled "Detection of Hb variants and hemoglobinopathies in Indian population using HPLC: a report of 2600 cases." [1] HPLC forms a rapid and accurate tool in the early detection and management of various hemoglobin disorders. We have also used cation exchange HPLC for the characterization of the hemoglobin profile in various thalassemia syndromes and hemoglobinopathies in our laboratory in the last 1 year. Our cases included 63 cases of β-thalassemia trait, 6 cases of thalassemia major, 5 cases of thalassemia intermedia, 2 cases of HbS/β-thalassemia, 8 cases of HbE/β-thalassemia, and 4 cases of HbH disease. Other hemoglobinopathies detected included HbSS, HbEE, HbDD, HbD Punjab trait, HbD Iran trait, HbS trait, HbE trait, HbSE, and HbJ.

Besides various abnormal hemoglobin fractions described by the author which can be detected by HPLC, we could diagnose four cases of HbH disease. HbH disease is seen most commonly in the Asian population (South East Asia, Mediterranean, and parts of Middle East). [2]

Hemoglobin H has extremely high affinity for oxygen and is therefore not useful for oxygen exchange, leading to tissue hypoxia disproportionate to the level of hemoglobin. [3]

It is prone to oxidation leading to the formation of intracellular inclusions. The instability of HbH is a major cause of anemia, as precipitates of oxidized HbH form in older red cells, which are then removed by splenic macrophages leading to hemolysis. HbH disease shows considerable variability in clinical and hematological severity. [4] The most relevant features are microcytic hypochromic anemia, hepatosplenomegaly, and mild hemolytic facies. [2],[5] The most sensitive method to detect HbH is inclusion bodies easily recognizable in a reticulocyte preparation (using brilliant cresyl blue) in last 10 years. [6] The presence of HbH was confirmed by HPLC in four of these cases; HPLC showed a significant sharp peak before the start of integration in all the four cases [Figure 1].
Figure 1: HPLC chromatogram obtained on Bio-Rad Variant β-thal short program for HbH. A sharp peak, before the start of integration, in the first minute of elution indicates HbH, F Concentration = 0.2%, A2 Concentration = 2.0*%

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HbH disease is an underdiagnosed entity in the Indian subcontinent. We feel that a careful evaluation for HbH inclusions on reticulocyte preparation would help in diagnosing these cases and also in assessing the prevalence of α-thalassemia in India.

 
   References Top

1.Sachdev R, Dam AR, Tyagi G. Detection of Hb variants and hemoglobinopathies in Indian Population using HPLC: Report of 2600 cases. Indian J Pathol Microbiol 2010;53:57-62.  Back to cited text no. 1
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2.Borgna-Pignatti C, Galanello R. Thalassemias and related disorders: Quantitative disorders of hemoglobin synthesis. In: Greer JP, Foerster J, Lkens JN, Rodgers GM, Paraskevas F, Glader B, editors. Wintrobe's clinical hematology. 11 th ed. Philadelphia: Lippincott Williams and Wilkins; 2004. p. 1319-65.  Back to cited text no. 2
    
3.Benesch RE, Ranney HM, Benesch R, Smith GM. The Chemistry of Bohr effect. II. Some properties of hemoglobin H. J Biol Chem 1961;236:2926-9.  Back to cited text no. 3
    
4.Kattamis C, Tzozos S, Kanavakis E, Synodinos J, Metaxotoumavrommati A. Correlation of clinical phenotype to genotype in hemoglobin H disease. Lancet 1988;1:442-4.  Back to cited text no. 4
    
5.Balgir RS. The burden of hemoglobinopathies in India and the challenges ahead. Curr Sci 2000;79:1536-47.  Back to cited text no. 5
    
6.Borgna-Pignatti C, Galanello R. Thalassemias and related disorders: Quantitative disorders of hemoglobin synthesis. In: Greer JP, Foerster J, Lkens JN, Rodgers GM, Paraskevas F, Glader B, editors. Wintrobe's clinical hematology. 11 th ed. Philadelphia: Lippincott Williams and Wilkins; 2009. p. 1093.  Back to cited text no. 6
    

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Correspondence Address:
Rachna Wadhwa
Department of Pathology, MAMC New Delhi - 110 002
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.81590

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This article has been cited by
1 Detection of Abnormal Hemoglobin Variants by HPLC Method: Common Problems with Suggested Solutions
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International Scholarly Research Notices. 2014; 2014: 1
[Pubmed] | [DOI]



 

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