Hemoglobin K-Woolwich (Hb KW) is a rare hemoglobin variant with very few cases reported. It is most prevalent in West African countries, particularly Nigeria, Ghana, and the Ivory Coast. Some reports suggest Hb KW may...Hemoglobin K-Woolwich (Hb KW) is a rare hemoglobin variant with very few cases reported. It is most prevalent in West African countries, particularly Nigeria, Ghana, and the Ivory Coast. Some reports suggest Hb KW may be a clinically benign trait, whereas others indicate it may behave similarly to a β+ thalassemia. The combination of hemoglobin S and hemoglobin KW (Hb S/KW) is a rare double heterozygous disorder with little known clinical characteristics. We report the hematologic and clinical data on three patients with Hb S/KW to help describe the characteristics of this patient population. The first two cases represent first cousins, ages 3 and 2 years. They are clinically asymptomatic. They have normal hemoglobin and mean corpuscle volume (MCV) levels without reticulocytosis. The third case is of a 14-year-old male who is non-anemic with no microcytosis. He has been clinically well except for abdominal pain upon dehydration. On hemoglobin electrophoresis, these patients have Hb S levels slightly higher than typically observed with sickle cell trait and a delay of hemoglobin F to adult levels. There exists a need for more reports to better delineate the clinical course and management of these patients.展开更多
Purpose: The accuracy of spectrophotometric hemoglobin (SpHb) measurement with a pulse co-oximeter was analyzed in cases of intraoperative massive hemorrhage and compared with Hb levels determined through blood gas an...Purpose: The accuracy of spectrophotometric hemoglobin (SpHb) measurement with a pulse co-oximeter was analyzed in cases of intraoperative massive hemorrhage and compared with Hb levels determined through blood gas analysis (LabHb) as a reference. We aimed to determine the extent to which in vivo adjustment (ADJ) for results of initial blood gas analysis would improve the accuracy of SpHb measurement. Methods: Data on LabHb and SpHb levels were collected from cases with hemorrhage of 1000 g or more. Correlations and Bland-Altman analyses were used to determine the associations between data before and after ADJ. Results: ADJ slightly improved the correlation coefficient (Pearson r) between SpHb and LabHb levels from 0.65 to 0.72. In Bland-Altman analysis, ADJ reduced the bias from 1.76 ± 1.47 g/dL to 0.64 ± 1.43 g/dL, while the 95% limits of agreement of -1.12 to 4.64 g/dL (range: 5.77 g/dL) without ADJ improved to -2.16 to 3.44 g/dL (range: 5.59 g/dL) with ADJ. In four-quadrant analysis, other than samples in the exclusion zone, the total number analyzed was 326, and the concordance rate was 82%. Conclusion: Although the accuracy of SpHb measurement improves on ADJ, SpHb measurement cannot substitute for LabHb levels, and it is necessary to determine Hb levels with blood gas analysis in the laboratory. It remains unclear which measurement is superior for determining when to initiate blood transfusion to achieve better outcomes. To comply with conventional methods, LabHb measurements may be necessary.展开更多
文摘Hemoglobin K-Woolwich (Hb KW) is a rare hemoglobin variant with very few cases reported. It is most prevalent in West African countries, particularly Nigeria, Ghana, and the Ivory Coast. Some reports suggest Hb KW may be a clinically benign trait, whereas others indicate it may behave similarly to a β+ thalassemia. The combination of hemoglobin S and hemoglobin KW (Hb S/KW) is a rare double heterozygous disorder with little known clinical characteristics. We report the hematologic and clinical data on three patients with Hb S/KW to help describe the characteristics of this patient population. The first two cases represent first cousins, ages 3 and 2 years. They are clinically asymptomatic. They have normal hemoglobin and mean corpuscle volume (MCV) levels without reticulocytosis. The third case is of a 14-year-old male who is non-anemic with no microcytosis. He has been clinically well except for abdominal pain upon dehydration. On hemoglobin electrophoresis, these patients have Hb S levels slightly higher than typically observed with sickle cell trait and a delay of hemoglobin F to adult levels. There exists a need for more reports to better delineate the clinical course and management of these patients.
文摘Purpose: The accuracy of spectrophotometric hemoglobin (SpHb) measurement with a pulse co-oximeter was analyzed in cases of intraoperative massive hemorrhage and compared with Hb levels determined through blood gas analysis (LabHb) as a reference. We aimed to determine the extent to which in vivo adjustment (ADJ) for results of initial blood gas analysis would improve the accuracy of SpHb measurement. Methods: Data on LabHb and SpHb levels were collected from cases with hemorrhage of 1000 g or more. Correlations and Bland-Altman analyses were used to determine the associations between data before and after ADJ. Results: ADJ slightly improved the correlation coefficient (Pearson r) between SpHb and LabHb levels from 0.65 to 0.72. In Bland-Altman analysis, ADJ reduced the bias from 1.76 ± 1.47 g/dL to 0.64 ± 1.43 g/dL, while the 95% limits of agreement of -1.12 to 4.64 g/dL (range: 5.77 g/dL) without ADJ improved to -2.16 to 3.44 g/dL (range: 5.59 g/dL) with ADJ. In four-quadrant analysis, other than samples in the exclusion zone, the total number analyzed was 326, and the concordance rate was 82%. Conclusion: Although the accuracy of SpHb measurement improves on ADJ, SpHb measurement cannot substitute for LabHb levels, and it is necessary to determine Hb levels with blood gas analysis in the laboratory. It remains unclear which measurement is superior for determining when to initiate blood transfusion to achieve better outcomes. To comply with conventional methods, LabHb measurements may be necessary.
