Improved exercise performance after exchange transfusion in subjects with sickle cell anemia

Miller, DM; Winslow, R. M.; Hj, Klein; Wilson, KC; Brown, FL; Statham, NJ

doi:10.1182/blood.v56.6.1127.1127

Cited by 45 publications

(12 citation statements)

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“…Therefore, transcutaneous SaO 2 was considered to accurately reflect the true SaO 2 in this study. The mean Tc-SO 2 in the patients prior to erythrocytapheresis in this study was 96.2 ± 2.8%, a relatively high value [2,11], and the mean P50 was 30.4 ± 2.2 mm Hg, a relatively low value for patients with SCD [6,12]. The reason may be that patients were being maintained on chronic erythrocytapheresis therapy, and have higher baseline Hb levels than non-transfused patients, which reduces the level of 2,3-DPG.…”

Section: Discussionmentioning

confidence: 47%

Effect of erythrocytapheresis on arterial oxygen saturation and hemoglobin oxygen affinity in patients with sickle cell disease

Uchida¹,

Rackoff²,

Ohene‐Frempong³

et al. 1998

Am. J. Hematol.

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An important purpose of blood transfusion in patients with sickle cell disease is to improve arterial oxygen saturation (SaO2) and thereby reduce red cell sickling. To investigate the degree of improvement in SaO2 by blood transfusion, we determined the hemoglobin oxygen affinity, transcutaneous oxygen saturation (Tc-SO2), and pulse rate before and after automated partial exchange transfusion (erythrocytapheresis). In 13 patients with sickle cell disease who underwent 24 erythrocytapheresis procedures, the mean oxygen tension at half saturation (P50) was significantly reduced from 30.4 +/- 2.2 to 26.0 +/- 1.6 mm Hg (P< 0.01) immediately after exchange transfusion. Mean Tc-SO2 values increased from 96.2 +/- 2.8 to 98.5 +/- 2.1% (P< 0.01). Approximately 50% of the increase in Tc-SO2 after erythrocytapheresis could be explained by the increase in hemoglobin oxygen affinity. An increase in arterial oxygen pressure (PaO2) following erythrocytapheresis, suggested by the calculated PaO2 in this study, may explain some of the increase in Tc-SO2. We conclude that improvement in Tc-SO2 in patients with sickle cell disease resulted from changes in hemoglobin oxygen affinity as well as blood oxygen pressure following erythrocytapheresis.

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Section: Discussionmentioning

confidence: 47%

Effect of erythrocytapheresis on arterial oxygen saturation and hemoglobin oxygen affinity in patients with sickle cell disease

Uchida¹,

Rackoff²,

Ohene‐Frempong³

et al. 1998

Am. J. Hematol.

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“…Transfusion studies have shown that this is related to the reduced oxygen carrying capacity associated with anemia [5-81. Transfusion to a higher hemoglobin results in improved exercise performance [7]. Partial exchange transfusion of SS patients with normal donor blood to raise hemoglobin A concentrations without increasing the total hemoglobin level has also been shown to increase exercise capability [8], suggesting that sickle erythrocytes contribute to poor exercise capacity.…”

Section: Introductionmentioning

confidence: 99%

Cardiovascular function during rest and exercise in patients with sickle-cell anemia and coexisting alpha thalassemia-2

1996

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Cardiac function was measured at rest and during exercise in 9 patients with sickle-cell anemia (SS) and coexisting homozygous alpha thalassemia-2 (alpha thal-2). Results were compared with 18 sickle cell patients with normal alpha globin genes, who were matched to the study group by age, gender, and size, and to published normal values. SS alpha thal-2 patients were less anemic: 9.9 +/- 1.0 vs 8.2 +/- 1.2 gm/dl for SS alone (P<.05). Left ventricular dimensions were normal in SS alpha thal-2 (4.9 +/- 0.7 cm), but increased in SS (5.4 +/- 0.7, cm P=.05) (normal range, 3.7-5.6 cm). Left ventricular wall thickness was, however, dramatically increased in the SS alpha thal-2 patients (free wall, 1.8 +/- 0.6 cm; septum, 1.6 +/- 0.4 cm), though SS controls had normal wall thickness (free wall, 1.0 +/- 0.2 cm; septum, 1.0 +/- 0.2 cm, P<.001) (normal range, 0.6-1.1 cm). At rest, Doppler indices of systolic function were not significantly different between sickle groups and normal values. SS alpha thal-2 patients did have abnormal diastolic filling at rest, as evidenced by a reduced ratio of early/late diastolic filling, 1.4 +/- 0.3 vs. 2.0 +/- 0.5 for SS controls (P<.01), and 1.8 +/- 0.4 for normals. An analysis of covariance suggested that this abnormality persisted after taking into account the previously demonstrated hypertrophy. During exercise, SS alpha thal-2 patients had higher heart rates and blood pressures than SS controls in spite of performing the same or less work. This resulted in a higher double product (an estimate of oxygen consumption) in SS alpha thal-2 patients (37,470 +/- 2,310 mm Hg-BPM) than in SS controls (33,310 +/- 1,490 mm Hg-BPM, P<.01). Work capacity, peak heart rate, and blood pressure were all abnormally decreased in both sickle-cell groups when compared to normal. Cardiac abnormalities noted at rest and during exercise in SS alpha thal-2 patients suggest a role of microvascular occlusion and a protective effect of decreased hemoglobin.

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Section: Discussionmentioning

confidence: 99%

“…Studies that showed improvement in exercise capacity with higher Hgb in SCA patients have increased Hgb by transfusion with Hgb A blood 20,21 . In the exchange transfusion experiment attempting to keep Hgb the same (but actually Hgb increased by 1.4 g/dL), the effect of Hgb A was directly queried and results suggested improved exercise capacity was associated with Hgb A 21 . In contrast, in a study of children on long‐term exchange transfusion maintaining Hgb S approximately 30%, average peak VO 2 was still less than 80% compared to healthy controls, but the transfused patients also had significantly lower Hgb (9.3 ± 0.5 g/dL) than healthy controls 32 …”

Section: Discussionmentioning

confidence: 99%

“…Indeed, CPET before and after transfusion in patients with chronic anemia and in anemic presurgical patients showed approximately 10% rise in peak VO 2 after transfusion 18,19 . In SCA patients, exercise studies improved after exchange transfusion, which partially replaced hemoglobin S blood with hemoglobin A blood 20,21 . These studies suggest low hemoglobin in SCA is likely one factor contributing to decreased exercise capacity, and increasing hemoglobin might improve VO 2 .…”

Section: Introductionmentioning

confidence: 92%

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Effect of voxelotor on cardiopulmonary testing in youths with sickle cell anemia in a pilot study

Phan

Hershenson

Caldarera

et al. 2023

Pediatric Blood & Cancer

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In Memoriam: Laura Caldarera passed away in early 2022 after a nearly year-long battle with aggressive cancer. As manager of the stress testing lab and preventive cardiology clinic at Child Cardiology Associates, she spent the majority of her career helping and assessing pediatric and adult congenital patients to improve their exercise capacity with a goal of improving their overall health and life expectancy. She was passionate, smart, and a true advocate for patients and families. She is greatly missed.

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Improved exercise performance after exchange transfusion in subjects with sickle cell anemia

Cited by 45 publications

References 0 publications

Effect of erythrocytapheresis on arterial oxygen saturation and hemoglobin oxygen affinity in patients with sickle cell disease

Effect of erythrocytapheresis on arterial oxygen saturation and hemoglobin oxygen affinity in patients with sickle cell disease

Cardiovascular function during rest and exercise in patients with sickle-cell anemia and coexisting alpha thalassemia-2

Effect of voxelotor on cardiopulmonary testing in youths with sickle cell anemia in a pilot study

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