Volume 43, Issue 4 pp. 837-844
ORIGINAL ARTICLE

Interpreting sulfhemoglobin and methemoglobin in patients with cyanosis: An overview of patients with M-hemoglobin variants

Aruna Rangan

Aruna Rangan

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Michelle E. Savedra

Michelle E. Savedra

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Camila Dergam-Larson

Camila Dergam-Larson

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Kenneth C. Swanson

Kenneth C. Swanson

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Jessica Szuberski

Jessica Szuberski

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Ronald S. Go

Ronald S. Go

Department of Hematology and Oncology, Mayo Clinic, Rochester, MN, USA

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Tavanna R. Porter

Tavanna R. Porter

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Sarah E. Brunker

Sarah E. Brunker

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Min Shi

Min Shi

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Phuong L. Nguyen

Phuong L. Nguyen

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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James D. Hoyer

James D. Hoyer

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

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Jennifer L. Oliveira

Corresponding Author

Jennifer L. Oliveira

Division of Hematopathology, Mayo Clinic, Rochester, MN, USA

Correspondence

Jennifer L. Oliveira, 200 1st St SW, Hilton-7-24, Rochester MN, 55905, USA.

Email: [email protected]

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First published: 06 June 2021
Citations: 7

Abstract

Introduction

Methemoglobin (MetHb) and sulfhemoglobin (SHb) measurements are useful in the evaluation of cyanosis. When one or both values are elevated, additional analysis is important to establish the etiology of the disorder. Methemoglobinemia occurs from acquired or hereditary causes with diverse treatment considerations, while true sulfhemoglobinemia is only acquired and treatment is restricted to toxin removal. Some toxic exposures can result in a dual increase in MetHb and SHb. Hereditary conditions, such as M-Hemoglobin variants (M-Hbs), can result in increased MetHb and/or SHb values but are clinically compensated and do not require treatment if they are cyanotic but otherwise clinically well.

Methods

Herein, we report 53 hemoglobin variant cases that have associated MetHb and SHb levels measured by an adapted Evelyn-Malloy laboratory assay method.

Results

Our data indicate M-Hbs cause variable patterns of MetHb and SHb elevation in a fairly reproducible pattern for the particular variant. In particular, α globin chain M-Hbs can mimic acquired sulfhemoglobinemia due to an isolated increased SHb value.

Conclusion

If the patient appears clinically well other than cyanosis, M-Hbs should be considered early in the evaluation process to differentiate from acquired conditions to avoid unnecessary testing and treatment regimens and prompt genetic counseling.

CONFLICT OF INTEREST

The authors have no competing interests.

DATA AVAILABILITY STATEMENT

Data sharing is not applicable to this article as no new data were created or analyzed in this study.

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