Cryohemolysis, erythrocyte osmotic fragility, and supplementary hematimetric indices in the diagnosis of hereditary spherocytosis

Ledesma Achem Miryam Emilse; Haro Cecilia; Terán Magdalena María; Mónaco María Eugenia; Issé Blanca Alicia; Sandra Stella Lazarte

doi:10.5045/br.2018.53.1.10

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Original Article

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Blood Res 2018; 53(1):

Published online March 31, 2018

https://doi.org/10.5045/br.2018.53.1.10

Cryohemolysis, erythrocyte osmotic fragility, and supplementary hematimetric indices in the diagnosis of hereditary spherocytosis

Ledesma Achem Miryam Emilse¹, Haro Cecilia¹, Terán Magdalena María¹, Mónaco María Eugenia², Issé Blanca Alicia¹, Sandra Stella Lazarte¹

Author Info. +

¹Instituto de Bioquímica Aplicada, Facultad de Bioquímica, Química y Farmacia, Universidad Nacional de Tucumán, Tucumán, Argentina.

²Instituto de Biología, Facultad de Bioquímica, Química y Farmacia, Universidad Nacional de Tucumán, Tucumán, Argentina.

Correspondence to : Sandra Stella Lazarte, Ph.D. Instituto de Bioqu?mica Aplicada, Universidad Nacional de Tucum?n, Balcarce 747, San Miguel de Tucum?, Tucum?n, CP4000 Argentina. slazarte@fbqf.unt.edu.ar

Received: June 13, 2017; Revised: July 20, 2017; Accepted: August 30, 2017

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

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Abstract

Background

Hereditary spherocytosis (HS) is a chronic hemolytic anemia characterized by microspherocytes in the peripheral blood and increased erythrocyte osmotic fragility (EOF). This study evaluated the cryohemolysis test (CHT); initial hemolysis (IH); immediate and incubated hemolysis percentage in 5.5 g/L NaCl (H5.5); mean corpuscular hemoglobin concentration (MCHC); red blood cell distribution width (RDW); and Hb/MCHC, Hb/RDW, and MCHC/RDW ratios for the diagnosis of HS.

Methods

Data from 13 patients with HS were evaluated at the Instituto de Bioqu?mica Aplicada and compared with data from 14 unaffected individuals and 11 patients with anemia due to another etiology. Total blood and reticulocyte counts, CHT, and immediate and incubated EOF were performed in all subjects; sensitivity, specificity, efficiency, and Youden index (YI) were calculated.

Results

Eight patients with HS had MCHC ≥345 g/L, 10 had RDW ≥14.5%, 12 had IH >5.0 g/L, 11 had immediate H5.5 ≥5%, and 13 had incubated H5.5 ≥50% (the cut-off value to consider HS). The efficiency and YI were: immediate H5.5 (0.94?0.85), incubated H5.5 (0.89?0.82), IH (0.89?0.78), MCHC (0.87?0.62), CHT (0.84?0.54), and Hb/MCHC (0.71?0.56), respectively. The calculated ratios could distinguish subjects with HS from unaffected individuals (P<0.05), but not those with anemia of another etiology (P>0.05).

Conclusion

Although the CHT and supplementary hematimetric indexes were useful to differentiate individuals with SH from healthy controls, they cannot distinguish from anemias of other etiology. CHT and MCHC, in addition to EOF, are recommended for diagnosing HS patients because of their low cost and efficiency.

Keywords Hereditary spherocytosis, Cryohemolysis, Erythrocyte osmotic fragility, Hematimetric indexes

Article

Original Article

Blood Res 2018; 53(1): 10-17

Published online March 31, 2018 https://doi.org/10.5045/br.2018.53.1.10

Cryohemolysis, erythrocyte osmotic fragility, and supplementary hematimetric indices in the diagnosis of hereditary spherocytosis

Ledesma Achem Miryam Emilse¹, Haro Cecilia¹, Terán Magdalena María¹, Mónaco María Eugenia², Issé Blanca Alicia¹, Sandra Stella Lazarte¹

¹Instituto de Bioquímica Aplicada, Facultad de Bioquímica, Química y Farmacia, Universidad Nacional de Tucumán, Tucumán, Argentina.

²Instituto de Biología, Facultad de Bioquímica, Química y Farmacia, Universidad Nacional de Tucumán, Tucumán, Argentina.

Correspondence to:Sandra Stella Lazarte, Ph.D. Instituto de Bioqu?mica Aplicada, Universidad Nacional de Tucum?n, Balcarce 747, San Miguel de Tucum?, Tucum?n, CP4000 Argentina. slazarte@fbqf.unt.edu.ar

Received: June 13, 2017; Revised: July 20, 2017; Accepted: August 30, 2017

Abstract

Background

Methods

Results

Conclusion

Keywords: Hereditary spherocytosis, Cryohemolysis, Erythrocyte osmotic fragility, Hematimetric indexes

Abstract

Fig 1.

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Figure 1.

CHT and HS discriminator indices in the groups under study.

Abbreviations: CHT, cryohemolysis test; HS, hereditary spherocytosis; MCHC, mean corpuscular hemoglobin concentration; N, normal; RDW, red blood cell distribution width.

Blood Research 2018; 53: 10-17https://doi.org/10.5045/br.2018.53.1.10

Fig 2.

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Figure 2.

Receiver operating characteristic (ROC) curves of the test results for discriminating HS patients from those with other anemias A: ROC curves of CHT, MCHC, and RDW; B: ROC curves of calculated ratios.

Abbreviations: CHT, cryohemolysis test; HS, hereditary spherocytosis; MCHC, mean corpuscular hemoglobin concentration; RDW, red blood cell distribution width.

Blood Research 2018; 53: 10-17https://doi.org/10.5045/br.2018.53.1.10

Table 1 . Results in the normal, HS, and anemia due to other causes groups..

^a)P<0.05 between the normal and patient groups; ^b)P<0.05 between the HS and other anemia groups..

Abbreviations: M, median; R, range; CHT, cryohemolysis test; EOF, erythrocyte osmotic fragility; H50, 50% hemolysis; MCH, mean corpuscular hemoglobin; MCHC, mean corpuscular hemoglobin concentration; MCV, mean corpuscular volume; RDW, red blood cell distribution width..

Table 2 . Sensitivity, specificity, efficiency, and Youden index of CHT, EOF parameters, and discriminative indices..

Abbreviations: CHT, cryohemolysis test; HS, hereditary spherocytosis; MCHC, mean corpuscular hemoglobin concentration; RDW, red blood cell distribution width..