Hemoglobin lansing: A novel hemoglobin variant causing falsely decreased oxygen saturation by pulse oximetry

Sarikonda, Kiran V.; Ribeiro, Rubens S.; Herrick, Jennifer L.; Hoyer, James D.

doi:10.1002/ajh.21452

Cited by 20 publications

(11 citation statements)

References 11 publications

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“…Six variant hemoglobins due to α‐globin gene missense mutations (Hbs Lansing [33], Titusville [35], Bonn [19], Delaware, and M‐Iwate [36], and a novel hemoglobin [37]), and five variant hemoglobins due to β‐globin gene missense mutations (Hbs Hammersmith [38], Cheverly [40], Okazaki, Regina, and Köln [21,45]) are tabulated in Table II (Supporting Information). Carriers of these variant hemoglobins presented with unexpectedly low SpO 2 but normal SaO 2 .…”

Section: Resultsmentioning

confidence: 99%

Unexpectedly low pulse oximetry measurements associated with variant hemoglobins: A systematic review

et al. 2010

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Pulse oximetry estimates arterial blood oxygen saturation based on light absorbance of oxy‐ and deoxy‐hemoglobin at 660 and 940 nm wavelengths. Patients with unexpectedly low SpO2 often undergo cardio‐pulmonary testing to ascertain the cause of their hypoxemia. However, in a subset of patients, a variant hemoglobin is responsible for low SpO2 measurements. The extent of this problem is unclear. We performed a systematic literature review for reports of low SpO2 associated with variant hemoglobins. We also reviewed unpublished cases from an academic hemoglobin diagnostic reference laboratory. Twenty‐five publications and four unpublished cases were identified, representing 45 patients with low SpO2 and confirmed variant hemoglobin. Fifty‐seven family members of patients had confirmed or suspected variant hemoglobin. Three low oxygen affinity variant hemoglobins had concordantly low SpO2 and SaO2. Eleven variant hemoglobins were associated with unexpectedly low SpO2 measurements but normal SaO2. Hemoglobin light absorbance testing was reported in three cases, all of which showed abnormal absorption spectra between 600 and 900 nm. Seven other variant hemoglobins had decreased SpO2, with unreported or uncertain SaO2. Twenty‐one variant hemoglobins were found to be associated with low SpO2. Most variant hemoglobins were associated with spuriously low SpO2. Abnormal absorption spectra explain the discrepancy between SpO2 and SaO2 for some variants. The differential diagnosis of possible variant hemoglobin ought to be considered in asymptomatic patients found to have unexpectedly low SpO2. The correct diagnosis will help to spare patients from unnecessary investigations and anxiety. Am. J. Hematol., 2010. © 2010 Wiley‐Liss, Inc.

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Section: Resultsmentioning

confidence: 99%

Unexpectedly low pulse oximetry measurements associated with variant hemoglobins: A systematic review

et al. 2010

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“…Uncommon Hb variants have been reported to cause spuriously decreased SpO 2 readings, including Hb Lansing, 24 Hb Bonn, 25 Hb Koln, 26,27 Hb Hammersmith, 28 and Hb Cheverly. 29 Sarikonda et al 24 reported a father and daughter with SpO 2 readings that were more than 10% lower than SaO 2 measurements due to an abnormal Hb variant (Hb Lansing) which accounted for w11% of their total Hb. Assessment of oxygen affinity was normal, suggesting that interference of absorbance of red and/or near-IR light by the abnormal Hb accounted for the spurious reduction in SpO 2 .…”

Section: Inherited Forms Of Abnormal Hemoglobinmentioning

confidence: 99%

Pulse oximetry: Understanding its basic principles facilitates appreciation of its limitations

Chan

2013

Respiratory Medicine

526

382

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Pulse oximetry has revolutionized the ability to monitor oxygenation in a continuous, accurate, and non-invasive fashion. Despite its ubiquitous use, it is our impression and supported by studies that many providers do not know the basic principles behind its mechanism of function. This knowledge is important because it provides the conceptual basis of appreciating its limitations and recognizing when pulse oximeter readings may be erroneous. In this review, we discuss how pulse oximeters are able to distinguish oxygenated hemoglobin from deoxygenated hemoglobin and how they are able to recognize oxygen saturation only from the arterial compartment of blood. Based on these principles, we discuss the various conditions that can cause spurious readings and the mechanisms underlying them.

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“…In pulse oximetry, the measured value of oxygen saturation only agrees with the actual oxygenated hemoglobin if no dysfunctional hemoglobins are present in the blood [ 2 , 3 , 26 ] . In the hemoglobin anomalies Hb Köln [ 20 ] , Hb Cheverly [ 15 ] , HbM Iwate [ 23 ] and Hb Lansing [ 36 ] , pulse oximetry does not refl ect actual oxygen saturation and comparison of pulse oximetry measurements and results from blood gas analysis revealed in parts considerable discrepancies, whereby pulse oximetry regularly detects false low oxygen saturation. The point mutation of Hb Bonn H87D in exon 2 of the α1 gene is found at the…”

Section: False Low Oxygen Saturation the Example Of Hemoglobin Bonnmentioning

confidence: 99%

Oxygen Saturation in Pulse Oximetry in Hemoglobin Anomalies

Zur¹,

Bağcı

Ludwig³

et al. 2012

Klin Padiatr

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Pulse oximetry is an essential diagnostic method in pediatric emergency medicine and pediatric intensive care. However, if undetected hemoglobin anomalies are the underlying cause measurements of low oxygen saturation can be interpreted incorrectly or may lead to unnecessary examinations. In 2 recently discovered hemoglobin anomalies, Hb Bonn and Hb Venusberg, this resulted in extensive and repeat cardiopulmonary examinations. This review aims to provide an overview of hemoglobin anomalies causing low oxygen saturation.We describe the methods required for differential diagnosis of hemoglobin anomalies, such as hemoglobin electrophoresis, High Performance Liquid Chromatography, hemoglobin gene sequencing and spectral photometry, and the difficulties with the interpretation of results. Furthermore, with a review of the literature we provide an extensive overview of hemoglobin anomalies which result in low oxygen saturation measurement in pulse oximetry. With the examples of Hb Bonn, a novel hemoglobin mutation of the proximal α1-globin, which results in false low pulse oximetry measurements of oxygen saturation, and Hb Venusberg, a low oxygen-affine hemoglobin mutation of the β-globin, we highlight the difficulties arising from the respective case histories.In pediatric medicine, hemoglobin anomalies must be included in the diagnosis as a possible underlying cause of low oxygen saturation in case of ambiguous or conflicting pulse oximetry findings.

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Hemoglobin lansing: A novel hemoglobin variant causing falsely decreased oxygen saturation by pulse oximetry

Cited by 20 publications

References 11 publications

Unexpectedly low pulse oximetry measurements associated with variant hemoglobins: A systematic review

Unexpectedly low pulse oximetry measurements associated with variant hemoglobins: A systematic review

Pulse oximetry: Understanding its basic principles facilitates appreciation of its limitations

Oxygen Saturation in Pulse Oximetry in Hemoglobin Anomalies

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