How do we monitor hemoglobin S in patients who undergo red blood cell exchange and take voxelotor?

Godbey, Elizabeth A.; Anderson, Michele R.; Bachmann, Lorin M.; Sanford, Kimberly W.; Wieditz, Kaitlyn; Roseff, Susan D.

doi:10.1111/trf.16405

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(1 citation statement)

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“…Lastly, the decision to report “total” HbS or HbS without HbS-Voxelotor complexes could lead to a confusion in the number of units of RBCs a patient receives in an exchange transfusion setting and, subsequently, estimate its efficiency. Godbey et al ( 9 ), have also reported on this dilemma in their patients coming to the apheresis clinic for the RBC exchange program, who were also on Voxelotor therapy. Thus, additional studies into the structure-function relationship of the hemoglobin-Voxelotor complexes are necessary to understand how quantitative HbS results should be reported in the presence of this agent.…”

Section: Discussionmentioning

confidence: 99%

Influence of Voxelotor–hemoglobin complexes in the estimation of hemoglobin S levels by the current standard of care laboratory evaluation techniques

et al. 2023

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BackgroundSickle cell disease is an inherited disorder characterized by the presence of sickle hemoglobin (HbS). The process of Hb molecule polymerization is a pivotal step in the sickling process. Voxelotor, a recently approved novel therapeutic agent, is known to interfere with polymerization. We aim to study the impact of Voxelotor on Hb variants analysis using high performance liquid chromatography (HPLC).Material and methodsWe are reporting the impact of Voxelotor on Hb variants analysis using HPLC after an informed consent and medical research committee approval. Data was collected from eight patients who are enrolled in the GBT440-034OL study using electronic medical records, to evaluate the Hb levels, hemolytic markers and the clinical response.ResultsOur patients were well-balanced for gender, with a mean age of 31.1 years (19–50). Six patients showed a significant improvement in the Hb level, with reduced reticulocytes, bilirubin, LDH and an improved clinical outcome. Interestingly, these patients showed the appearance of a split band of Hb S and D on HPLC impacting significantly on HbS level. Two patients did not show any improvement on laboratory parameters, and no changes on their HPLC analysis.ConclusionsWe report here eight patients on Voxelotor therapy, six of which showed improved hemolytic markers and anemia and demonstrated the appearance of HbD peak on the HPLC chromatogram. Therefore, the absence of HbD on HPLC or other laboratory methods for estimating HbS in patients on Voxelotor therapy, gives the clinician a possible hint regarding the patient's compliance with the drug.

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Section: Discussionmentioning

confidence: 99%