Granulocyte-colony stimulating factor administration among hemoglobin S trait donors: A single center experience from the Eastern Mediterranean region.

BACKGROUND AND OBJECTIVE: Assessment of Hemoglobin S trait donors has gained importance together with the increased allogeneic peripheral stem cell transplant activity for sickle cell disease in the regions where the disease is prevalent. Outcomes of Granulocyte-Colony Stimulating Factor (G-CSF) administration are obscure for hemoglobin S trait donors. This study aims at investigating the incidence of hemoglobin S carrier status and outcomes of G-CSF administration among donors who live in Eastern Mediterranean region. MATERIAL AND METHOD: The cross-sectional, single-center cohort study was performed with 147 donors between January 2013 and March 2017. Prevalence of hemoglobin S trait was estimated and subjects with or without Hemogobin S trait were compared with regard to stem cell characteristics, early and late clinical outcomes after G-CSF administration. RESULTS: Eleven out of 147 donors (7.48%) were found as hemoglobin S trait. G-CSF administration was successfully completed and yielded good harvesting results in hemoglobin S trait donors. No statistically significant difference was found between groups with regard to early and late side effects, stem cell characteristics. Blood pressures and QTc values were within normal ranges in both groups. Groups were similar with regard to CD34 values. CONCLUSION: G-CSF seems safe in hemoglobin S trait donors. Their being eligible as donors would increase the chance of the patients for allogeneic stem cell transplantation in high prevalence regions. Further studies are required to reveal the safety profile of G-SCF in hemoglobin S carriers in different regions.

Quantitation of hemoglobins using Sebia Capillarys-2 capillary electrophoresis (CE) for A1c: Comparison to results using CE for hemoglobins.

Background: Measurement of A1c using the Sebia Capillarys-2 capillary electrophoresis (A1c CE) involves relative quantitative measurements of peaks for hemoglobins A1c, A, A2. We examined correlation of A1c CE results with results of CE analysis for hemoglobins (Hb CE) for homozygous A and S-trait patients. We specifically examined whether abnormalities in A2 or the A/S ratio by A1c CE alone would reasonably be the basis for recommendation of red cell indices for evaluation of possible thalassemia. Methods: Selection of patients was from results for A1c CE, exhibiting either a normal pattern or a pattern consistent with S-trait. We then examined correlation of results of quantitation for A, S and A2 between A1c CE and Hb CE. Results: %A2 by A1c CE (y) had high correlation with %A2 by Hb CE (x): y = 0.88 x; r = 0.948. %A2 in S-trait patients was right-shifted in comparison to normals by 0.5%. For S-trait patients, the A/S ratio by A1c CE (y) had high correlation with the A/S ratio by Hb CE (x): y = 1.02 x; r = 0.995. Conclusions: Given high correlation of results between A1c CE and Hb CE, patent elevation of A2 by A1c CE for either normal or S-trait patients is a reasonable basis for recommendation of red cell indices for evaluation of possible beta thalassemia. For S-trait patients, patent abnormality in the A/S ratio by A1c CE is a reasonable basis for recommendation of red cell indices for evaluation of possible alpha or beta thalassemia.

Acceptability to and Engagement With a Virtual Sickle Cell Trait Education Program (SCTaware): Single-Center Prospective Study.

BACKGROUND: Public health programs are tasked with educating the community on health topics, but it is unclear whether these programs are acceptable to learners. Currently, these programs are delivered via a variety of platforms including in-person, virtually, and over the telephone. Sickle cell trait (SCT) education for parents of children with this trait is one of many education programs provided by the Ohio Department of Health. The novel SCTaware videoconference education program was developed by a research team after central Ohio's standard program transitioned from in-person to telephone-only education during the COVID-19 pandemic. OBJECTIVE: Our objectives were to investigate the acceptability of the format and engagement with the SCTaware education and assess parental worry about having a child with SCT before and after receiving SCTaware. METHODS: This was a single-center, prospective study of English-speaking parents of children <3 years of age identified to have hemoglobin S trait by newborn screening. Parents who previously received SCT education by telephone, were able to be contacted, and had access to an electronic device capable of videoconferencing were eligible to complete surveys after receiving the virtual SCTaware education program. The SCTaware educator also completed a survey to assess participant engagement. Data were summarized descriptively and a McNemar test was used to compare parental worry before and after receiving SCTaware. RESULTS: In total, 55 participants completed follow-up surveys after receiving standard SCT telephone education and then completing SCTaware. Most (n=51) participants reported that the SCTaware content and visuals were very easy to understand (n=47) and facilitated conversation with the educator (n=42). All of them said the visuals were respectful and trustworthy, helped them understand content better, and that their questions were addressed. Nearly two-thirds (62%, n=34) reported that the pictures appeared very personal and applied to them. The educator noted most participants (n=45) were engaged and asked questions despite having to manage distractions during their education sessions. Many participants (n=33) reported some level of worry following telephone-only education; this was significantly reduced after receiving SCTaware (P<.001). CONCLUSIONS: Our results suggest that SCTaware is acceptable and engaging to parents. While telephone education may make SCT education more accessible, these findings suggest that many parents experience significant worry about their child with SCT after these sessions. A study to evaluate SCTaware's effectiveness at closing parents' SCT knowledge gaps is ongoing.

Acute visual loss following dapsone-induced methemoglobinemia and hemolysis.

OBJECTIVE: While methemoglobinemia is a possible complication of chronic dapsone therapy or of acute overdose, serious adverse manifestations related to methemoglobin formation remain rare. We present an unusual case with severe ischemic retinal injury. CASE REPORT: A 30-year-old African woman presented with a sudden decrease of visual acuity secondary to retinal ischemia. She was chronically treated with dapsone (50 mg/day) for a dermatologic disease and denied any drug overdose. However, the determination of serum dapsone level on admission revealed a largely supratherapeutic concentration (20,044 µg/ml compared with 1-3.5 ± 0.5 µg/ml for therapeutic levels). The methemoglobin level at admission was 32% (sulfhemoglobin 1.2%), with hemoglobin level, 7.4 g/dl, schistocytes count, 2-5%, lactate dehydrogenase level, 580 IU/l, and haptoglobin level, < 10 mg/dl. The patient had both alpha-thalassemia and sickle cell trait. She was treated with methylene blue, vitamin C, and exchange transfusion. There was no improvement in visual symptoms over time. CONCLUSIONS: In a patient with supratherapeutic serum levels of dapsone, the severity of visual injury was associated with dapsone-induced methemoglobinemia and hemolysis, and perhaps also with some hematologic predisposing factors.