Benefits of Simple Exchange Transfusion in Sickle Cell Disease (HbSS) with Vaso-Occlusive Crisis Not Responding to Standard Therapy

Sickle cell disease is an autosomal recessive genetic disease. Vaso occlusive crisis (VOC) is frequently seen in such patients. Painful VOC is usually recurrent, of variable severity due to many factors and its management poses important challenge in the clinical practice. Few patients do not respond to standard therapies and continue to suffer severe pain for prolonged period or land to serious life threatening situation. The red cell exchange by aphaeresis is presumed to be one efficient alternative in this situation which can reduce the level of HbS below 40% - 50%. However, it is costly and not available everywhere. Both circumstances are common in our state where incidence of sickle cell disease is quite high. In such situations simple red cell exchange i.e. removing 1 unit (350 ml) of blood manually (by phlebotomy) and replacement with one unit normal red cell is effective. All of our four cases of SCA with severe acute VOC, are not responding to standard therapy but responded efficiently to this simple red cell exchange transfusion. Our present observation may pave the way of one simple, affordable, and effective measure to reduce the pain of severe acute VOC not responding to standard therapy. Moderate reduction of HbS by 8% - 14% by simple red cell exchange transfusion was associated with relief of pain of acute VOC;a new observation was reported in all our 4 cases which need to be validated by larger controlled studies.

Intensive phototherapy vs.exchange transfusion for the treatment of neonatal hyperbilirubinemia:a multicenter retrospective cohort study

Background:Intensive phototherapy(IPT)and exchange transfusion(ET)are the main treatments for extreme hyperbilirubinemia.However,there is no reliable evidence on determining the thresholds for these treatments.This multicenter study compared the effectiveness and complications of IPT and ET in the treatment of extreme hyperbilirubinemia.Methods:This retrospective cohort study was conducted in seven centers from January 2015 to January 2018.Patients with extreme hyperbilirubinemia that met the criteria of ET were included.Patients were divided into three subgroups(low-,medium-,and high-risk)according to gestational week and risk factors.Propensity score matching(PSM)was performed to balance the data before treatment.Study outcomes included the development of bilirubin encephalopathy,duration of hospitalization,expenses,and complications.Mortality,auditory complications,seizures,enamel dysplasia,ocular motility disorders,athetosis,motor,and language development were evaluated during follow-up at age of 3 years.Results:A total of 1164 patients were included in this study.After PSM,296 patients in the IPT only group and 296 patients in the IPT plus ET group were further divided into the low-,medium-,and high-risk subgroups with 188,364,and 40 matched patients,respectively.No significant differences were found between the IPT only and IPT plus ET groups in terms of morbidity,complications,and sequelae.Hospitalization duration and expenses were lower in the low-and medium-risk subgroups in the IPT only group.Conclusions:In this study,our results suggest that IPT is a safe and effective treatment for extreme hyperbilirubinemia.The indication of ET for patients with hyperbilirubinemia could be stricter.However,it is necessary to have a contingency plan for emergency ET as soon as IPT is commenced especially for infants with risk factors.If IPT can be guaranteed and proved to be therapeutic,ET should be avoided as much as possible.

Preventive Exchange Blood Transfusion in Pregnant Women with Sickle Cell Disease: Maternal and Perinatal Prognosis in a Country with Limited Resources, Burkina Faso

Context: In pregnant women with sickle cell disease, the management with exchange transfusion could be useful in improving the prognosis of mother and child by reducing the level of hemoglobin S less than 40%. Objective: To analyze the maternal and perinatal outcome during the program of the exchange transfusion in pregnant women with sickle cell disease. Patients and Methods: We conducted a prospective study over a period of 18 months. Pregnant women with a major form of sickle cell disease were included. A manual blood exchange transfusion was performed monthly. We monitored the occurrence of maternal and perinatal morbidity during the follow-up. Results: A total of 42 pregnant women with sickle cell disease were monitored. The frequency of infectious episodes and vaso-occlusive crisis was significantly reduced, respectively from 47.6% and 83.3% before the beginning of the blood exchanges transfusion to 11.9% and 16.7% during blood exchanges transfusion program. All newborns were alive at birth with an Apgar score higher or equal to 7 at the 5th minute. The rate of admission of the newborns at neonatal intensive care unit was 9.3%. Maternal mortality was estimated at 7.1% and there was no early neonatal mortality. Conclusion: Prophylactic exchange transfusion reduces infections and the reoccurrence of vaso-occlusive crisis, which has an impact on perinatal prognosis.

Effect of exchange blood transfusion on oxygen saturation of neonates with severe neonatal jaundice by pulse oximetry

Objective:To determine if there was any difference in SpO2 readings during exchange blood transfusion(EBT).Methods:A prospective cross-sectional study of neonates with severe neonatal jaundice requiring EBT was conducted.Oxygen saturation was recorded before,immediately and 15 minutes after EBT by using a pulse oximeter.Results:This study included 30 neonates with 20 males and 10 females.The age ranged from 1 to 12 days with a mean of(5.4±2.9)days.Pre-EBT SpO2 ranged from 90%to 98%with a mean value of(94.3±2.2)%;SpO2 in the end of EBT ranged from 85%to 99%with a mean value of(94.1±3.2)%;SpO2 at 15 minutes after EBT ranged from 77%to 99%with a mean value of(94.8±4.1)%.There was no significant difference between SpO2 values at onset of EBT and either immediately or 15 minutes after EBT(P=0.770 and 0.422,respectively).SpO2 showed no significant difference between neonates who were infused with blood of different storage times(<24 h or≥24 h)at the onset of EBT(P=0.584),immediately(P>0.999)and 15 minutes after EBT(P=0.887).Besides,SpO2 values were compariable in neonates with hematocrit<45%or≥45%at the onset of EBT(P=0.284),immediately(P=0.118)and 15 minutes after EBT(P=0.868).Conclusions:EBT does not affect SpO2 in neonates.