Manual erythroexchange in sickle cell disease: multicenter validation of a protocol predictive of volume to exchange and
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ORIGINAL ARTICLE
Manual erythroexchange in sickle cell disease: multicenter validation of a protocol predictive of volume to exchange and hemoglobin values Barbara Gianesin 1 & Valeria Maria Pinto 1 & Maddalena Casale 2 & Paola Corti 3 & Carmelo Fidone 4 & Sabrina Quintino 1 & Vincenzo Voi 5 & Gian Luca Forni 1 Received: 16 March 2020 / Accepted: 15 July 2020 / Published online: 21 July 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Manual erythroexchange (MEEX) was proven to be effective and safe in the management of sickle cell disease (SCD). The goal is to quickly reduce the percentage of hemoglobin S (HbS%). A national survey of the Italian Society for Thalassemia and Hemoglobinopathies (SITE) observed a great variability among MEEX protocols none of which were found to be predictive of the values of HbS% and hemoglobin (Hb) after the exchange. Two equations to estimate the HbS% and Hb values to be obtained after MEEX were developed based on the results of the MEEX procedures in place in the centers participating in the present study. A standard protocol was subsequently defined to evaluate the volumes to exchange to obtain the target values of HbS% and Hb. The protocol was tested in 261 MEEX performed in SCD patients followed in the 5 participating centers that belong to the Italian Hemoglobinopathy Comprehensive Care Network, with the support of the SITE. The results showed a correlation between the estimated and measured values of HbS% and Hb (Rp 0.95 and 0.65 respectively, p < 0.001). A negligible bias was found for the prediction of HbS% and a bias of 1 g/dl for Hb. From consecutive MEEX, a rate of increase of HbS% between two exchanges of around 0.4% per day (p < 0.001) was measured. This protocol was shown to be effective and safe, as all patients reached the target value of HbS%. All the MEEX procedures were carried out with single venous access. No adverse events or reactions such as hypotension or electrolyte imbalance were reported nor were any complaints concerning the procedures received from patients. Keywords MEEX . Sickle cell disease . Volumes exchanged . HbS% . Hb
Introduction Sickle cell disease (SCD) is the most relevant hemoglobinopathy worldwide in terms of frequency and social impact and has recently been recognized as a global public health problem * Gian Luca Forni [email protected] 1
Hemoglobinopathies and Congenital Anemia Center, Ospedale Galliera, Genoa, Italy
2
Department of Woman Child and General Surgery, University of Campania “Luigi Vanvitelli”, Naples, Italy
3
Pediatrics Unit, Monza e Brianza per il Bambino e la sua Mamma Foundation (FMBBM), Monza, Italy
4
Thalassemia Center, SIMI Ragusa, Ragusa, Italy
5
Department of Clinical and Biological Sciences, University of Turin, Turin, Italy
by the World Health Organization and the United Nations [1, 2]. SCD is a hereditary red cell disorder characterized by the presence of a point mutation on the β-globin chain, resulting in the synthesis of the pathological hemoglobin S (HbS) [3, 4] w
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