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Hereditary spherocytosis diagnosed with extremely low glycated hemoglobin compared to plasma glucose levels

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Abstract

Glycated hemoglobin (HbA1c) is an important indicator of glycemic control in patients with diabetes. High-performance liquid chromatography (HPLC) is the most commonly used method for measuring HbA1c levels; as HPLC measures all hemoglobin types, the values can be influenced by hemoglobin variants. Moreover, as HPLC-HbA1c levels are low in some diseases, including hemolytic anemia, it may be difficult to differentiate hemoglobin variants from these diseases based on HPLC-HbA1c levels alone. Similar HbA1c values using both HPLC and immunoassays (IAs) are noted for these diseases, while discrepancies are noted in the case of hemoglobin variants. Herein, we describe our process of differential diagnosis for hereditary spherocytosis, the most common inherited hemolytic anemia, in a 56-year-old man presenting with a low HPLC-HbA1c level compared to the glucose concentration, concomitant with anemia, jaundice, hyperbilirubinemia, cholelithiasis, and splenomegaly. There was a discrepancy between HbA1c levels measured with HPLC and IAs and glycated albumin levels. The possibility of hemoglobin variants was unlikely, based on the chromatography and isoelectric focusing results. The haptoglobin levels and reticulocyte counts were low and high, respectively. The direct and indirect Coomb’s tests were negative. The presence of spherocytes on blood smears and flow cytometric analysis of the eosin-5-maleimide binding test supported a diagnosis of hereditary spherocytosis. We recommend that when a discrepancy between HPLC-HbA1c levels and glucose concentrations is noted, clinicians should consider hemolysis or hemoglobin variants as the diagnosis. It should be considered that a discrepancy between HbA1c levels measured with HPLC and IAs does not specifically exclude hemolysis.

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Acknowledgements

We thank Dr.Yukio Hattori (Department of Clinical Laboratory, Saiseikai Yamaguchi General Hospital), Dr. Yasuhiro Yamashiro (Department of Health Sciences, Yamaguchi University Graduate School of Medicine), and the staff of Fukuyama Medical Laboratory for their support in performing laboratory tests and assistance in the diagnosis of this patient. We also thank Editage for their technical assistance by way of editing a draft of this manuscript.

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Author notes

  1. Takuya Okamoto and Hisato Shima contributed equally to this work.

Authors and Affiliations

  1. Department of Laboratory, Kawashima Hospital, 1-39 Kitasakoichiban-cho, Tokushima, Tokushima, 770-0011, Japan

    Takuya Okamoto, Chiaki Masaki, Hiroaki Tada & Norimichi Takamatsu

  2. Department of Kidney Disease, Kawashima Hospital, 1-39 Kitasakoichiban-cho, Tokushima, Tokushima, 770-0011, Japan

    Hisato Shima, Manabu Tashiro, Tomoko Inoue & Jun Minakuchi

  3. Department of Diabetes Care Medicine, Kawashima Hospital, 1-39 Kitasakoichiban-cho, Tokushima, Tokushima, 770-0011, Japan

    Yoshihiko Noma & Machiko Komatsu

  4. Department of Internal Medicine, Kawashima Hospital, 1-39 Kitasakoichiban-cho, Tokushima, Tokushima, 770-0011, Japan

    Hiroyuki Azuma & Keiko Miya

Authors
  1. Takuya Okamoto
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  2. Hisato Shima
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  3. Yoshihiko Noma
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  4. Machiko Komatsu
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  5. Hiroyuki Azuma
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  6. Keiko Miya
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  7. Manabu Tashiro
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  8. Tomoko Inoue
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  9. Chiaki Masaki
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  10. Hiroaki Tada
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  11. Norimichi Takamatsu
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  12. Jun Minakuchi
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Corresponding author

Correspondence to Hisato Shima.

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All procedures followed were in accordance with the ethical standards of the responsible committee on human experimentation (institutional and national) and with the Helsinki Declaration of 1964 and later versions.

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Informed consent was obtained from the patient included in this study.

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Okamoto, T., Shima, H., Noma, Y. et al. Hereditary spherocytosis diagnosed with extremely low glycated hemoglobin compared to plasma glucose levels. Diabetol Int 12, 229–233 (2021). https://doi.org/10.1007/s13340-020-00456-4

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  • DOI: https://doi.org/10.1007/s13340-020-00456-4

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