Improved method for automated red cell exchange in sickle cell disease

An improved method for intermittent-flow erythrocytapheresis in patients with sickle cell disease is reported. The method, a modification of the standard red cell exchange procedure for the Haemonetics 30S unit, dilutes with physiologic saline the patient's blood as it flows from the draw line and before it reaches the centrifugation bowl. The blood dilution (approximately 1.6 parts saline to 1 part blood) is used only during the first two passes, when the proportion of sickle erythrocytes in the patient's blood is still high. Only that amount of bowl supernatant (saline-diluted plasma) necessary to maintain extracorporeal volume below 500 ml is returned to the patient. The method described largely prevents the clumping of sickle erythrocytes in the centrifugation bowl, a complication frequently encountered with the Haemonetics 30S unit. Thus, changing the bowl between passes is avoided. Furthermore, the sickle red cells can be collected with the first pass and cryopreserved for possible future uses including the option of autotransfusion.     

Blood bank issues associated with red cell exchanges in sickle cell disease

Sickle cell disease (SCD) patients are prone to develop complications that include stroke, acute chest syndrome, and other crises. Some of these complications require chronic transfusion therapy or red cell exchange (RCE), either for therapeutic or prophylactic reasons. Due to a discrepancy of red cell antigens between African Americans and Caucasians (majority blood donors), the incidence of alloantibody formation is very high, which makes it difficult to find compatible red cell units, especially for urgent RCE. Some of the above conditions require immediate oxygen delivery to the tissues. Thus, SCD patients undergoing RCE should receive red blood cells with special attributes that include matching for Rh and Kell blood group antigens; RBCs should be fresh in order to provide (1) immediate oxygen delivery and (2) longer surviving cells to reduce the interval between RCE. Also, these units should be pre-storage leukoreduced to prevent febrile non-hemolytic reactions and screened for sickle cell traits to avoid transfusing red cells containing HbS. This requires a concerted effort between the apheresis unit, the local blood bank, and the central blood supplier.     

Use of red blood cell exchange for treating acute complications of sickle cell disease

Sickle cell disease (SCD) is a life-threatening chronic condition primarily caused by genetic mutation. The disease is characterized by intermittent vaso-occlusive events and chronic hemolytic anemia. Acute complications in patients with SCD are difficult to manage due to the pathophysiological nature of the disease. Transfusion therapy is the cornerstone of management of acute complications and significantly reduces SCD morbidity and mortality. Red cell exchange (RCE), which is characterized by low iron accumulation and volume overload, has been widely used for transfusion therapy in recent years.     

Safety,tolerability, and outcomes of regular automated red cell exchange transfusion in the management of sickle cell disease

We report here our experience with regular automated red cell exchange transfusion for the management of chronic complications of sickle cell disease in 50 patients in our institution from June 2011 to December 2014. The mean sickle hemoglobin level was 44% and 8.5% pre‐ and post‐transfusion, respectively. Platelets were reduced by a mean 70% during the procedure with a count of less than 50 × 10⁹/l in 6% of cases. The alloimmunization rate was 0.065/100 units of red cells with no hemolytic reactions. Patients with no iron overload at baseline showed no evidence of iron accumulation with a mean liver iron concentration of 1.6 mg/g dry tissue and 1.9 mg/g dry tissue at baseline and 36 months, respectively. All six patients with pre‐existing iron overload and on chelation therapy, showed a gradual reduction of their liver iron concentration and two patients could discontinue chelation during the follow‐up period. Seventy percentage of patients who were on the programme for recurrent painful crises showed a sustained reduction in the number of emergency hospital attendances; the mean number of days in hospital for emergency treatment was 103 in the year prior to commencing ARCET and reduced to 62 (40%) after the first 12 months, 51 (50%) after 24 months, and 35 days (66%) after 36 months. J. Clin. Apheresis 31:545–550, 2016. © 2015 Wiley Periodicals, Inc.     

Therapeutic plasmapheresis and red blood cell exchange in a sickle cell trait patient with rhabdomyolysis

We report a case of a 16‐year‐old African‐American male with sickle cell trait and a past medical history significant for asthma that was transferred to our hospital for management of respiratory failure. On the fourth day of hospitalization, the patient was found to have increased creatine kinase (CK) levels and urine myoglobin levels consistent with rhabdomyolysis. No clear etiology was identified. Aggressive standard hydration and urine alkalization were applied without response. On the sixth day of hospitalization, the patient underwent a 1–1.5 plasma volume therapeutic plasma exchange (TPE) resulting in a transient reduction of serum CK and myoglobin by 50%, which became elevated once again within 4 h. Since his clinical presentation resembles exertional rhabdomyolysis documented in patients with sickle cell trait, RBC exchange was performed. The patient tolerated the procedure without complications. In addition to his improved overall condition, the patient's post‐exchange CK and serum myoglobin levels dropped dramatically without rebound. To our knowledge, this case represents the first reported case of TPE followed by RBC exchange in a SCT patient with rhabdomyolysis. J. Clin. Apheresis 2012. © 2012 Wiley Periodicals, Inc.     

Process mapping of the urgent red cell exchange procedure for patients with severe complications of sickle cell disease at a centralized hemapheresis service

Sickle cell disease (SCD) patients require urgent red cell exchange (RCE) procedures for acute chest syndrome (ACS), demanding a coordinated effort of multiple clinical services. Execution of RCE is a multistep process from the time the procedure is requested to the time the procedure is initiated. A retrospective review of patients with SCD requiring urgent RCE for ACS and stroke from 2012 to 2017 was performed at a centralized hemapheresis service covering a multihospital healthcare system. A total of 30 urgent RCE procedures performed on 28 patients were evaluated. The time required for red blood cell (RBC) preparation was the longest step in the process (median 3.8 hours). Furthermore, RBC preparation time was longer for sickle cell patients with RBC alloimmunization compared with nonalloimmunized patients (8.6 vs 3.8 hours, P = .03). One mortality event occurred in Ab− group. This study identified potentially modifiable factors, which impact the time to implementation of RCE in one service area. It highlights the importance of a structured and coordinated approach for the efficient and timely delivery of this vital treatment modality.     

The implementation of automated red blood cell exchange (erythrocytapheresis) as a treatment modality in sickle cell disease patients: Single center experience

BackgroundImprovements of health infrastructure, preventive care and clinical management is important to reduce the morbidity and mortality of sickle cell disease (SCD).ObjectiveThis prospective, investigator-initiated non-randomized open-label intervention, single centre study describes the implementation of the automated erythrocytapheresis in low-middle income country as a treatment modality for SCD patients to improve the standard of care and highlights its benefits and challenges.MethodologyEligible patients with SCD who had overt stroke, abnormal/conditional transcranial doppler (TCD), or other indications were subjected to regular automated erythrocytapheresis program.ResultsFrom 18th Dec 2017 till 17th Dec 2022, 21 subjects were enrolled; seventeen (80.9 %) were Egyptian and four (19.1 %) were non-Egyptian (three Sudanese and one Nigerian). Totalling 133 sessions had been performed mainly in working hours with fluctuating frequency per month. All sessions maintained isovolumic status and were performed using central venous access. The target HbS concentration was set from the start; the mean final FCR % fraction was 51, most of the session (n = 78, 58.7 %) were able to achieve target FCR. The majority of session pass smoothly with no adverse event (n = 81, 60.9 %), except for certain challenges as shortage of the required blood (n = 38), hypotension (n = 2), hypocalcaemia (n = 2).ConclusionAutomated erythrocytapheresis is safe and effective modality for management of patients with sickle cell disease.     

Erythrocyte‐exchange with the OPTIA™ cell separator in patients with sickle‐cell disease

Erythrocyte–exchange (EEX) has proven to be a very useful tool in sickle‐cell disease (SCD) patients either during acute painful crisis unresponsive to hydration and/or analgesia or as a prophylactic treatment in high risk patients in those who do not tolerate hydroxyurea (HU), with the aim of lowering HbS levels. EEX may be performed either by using continuous‐ or discontinuous flow devices, the former being of choice in children or in low‐weight patients. Thus, a low extracorporeal blood volume (EBV) could allow for a better and safer procedure management. In this study we compared EEX procedure performed with the recently released OPTIA device with EEX procedures performed using the COBE Spectra device (EBV 185 vs 270 mL, respectively). Twenty‐one EEX (4 as emergency treatment) were performed in 12 patients with the Spectra device and 25 (9 as emergency treatment) in 15 patients with the OPTIA device. All the procedures were well tolerated and uneventful. We did not observe significant differences between the two devices as to pre‐ and post‐EEX parameters, namely in target hematocrit and in HbS reduction. Noteworthy, due to the lowest EBV allowed by the OPTIA device, an EEX procedure performed in a 13 Kg‐ child did not require a preliminary priming of the circuit. In conclusion, the OPTIA device proved to be as effective as the Spectra device in treating SCD patients either during sickling crisis or as prophylactic therapy. The OPTIA device can be safely used in the pediatric setting since it allows a lower EBV. J. Clin. Apheresis, 28:411–415, 2013. © 2013 Wiley Periodicals, Inc.     

A pseudoaneurysm within a subperiosteal collection in a patient with sickle cell disease

Sickle cell disease involves long bones in the form of infection or subperiosteal collections. Rare pseudoaneurysm/aneurysm formation is also known to occur in the intracranial and visceral territories. We report a small subperiosteal pseudoaneurysm that developed within a subperiosteal abscess in the tibia in a patient with sickle cell disease. This case adds to the known spectrum of musculoskeletal abnormalities resulting from this condition. © 2009 Wiley Periodicals, Inc. J Clin Ultrasound, 2010