Blood Res.  2018 Mar;53(1):10-17. 10.5045/br.2018.53.1.10.

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

• Emilse LA
• Cecilia H
• María TM
• Eugenia MM
• Alicia IB
• Lazarte SS

• KMID: 2414359
• DOI: http://doi.org/10.5045/br.2018.53.1.10

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 Bioquímica 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.

Keyword

Hereditary spherocytosis; Cryohemolysis; Erythrocyte osmotic fragility; Hematimetric indexes

MeSH Terms

Anemia
Anemia, Hemolytic
Diagnosis*
Erythrocyte Indices
Erythrocytes*
Hemolysis
Humans
Osmotic Fragility*
Reticulocyte Count
Sensitivity and Specificity

Figure

• 

  Fig. 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.

• 

  Fig. 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.

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