Outcomes of Chronic Automated RBC Exchange Program in Omaha, Nebraska 2015-2020

Blood ◽  
2020 ◽  
Vol 136 (Supplement 1) ◽  
pp. 21-22
Author(s):  
Aleh Bobr ◽  
Scott A Koepsell ◽  
Julie Eclov ◽  
Omar Abughanimeh ◽  
Steven Ebers ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Iron Overload ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Beta Thalassemia ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Before And After ◽  
Ed Visits

Background: Red blood cell exchange (RBCX) is an effective therapy in the treatment of different hemoglobinopathies. The University of Nebraska Medical Center (UNMC) established a chronic RBCX program in November 2015, which took care of patients with multiple hemoglobinopathies. In this study, we aim to evaluate the outcomes of this program. Methods: This is a retrospective study. After an IRB approval, we reviewed the charts of patients who were enrolled in the chronic RBCX program between 11/2015-7/2020 at UNMC. Data was collected to evaluate indications of RBCX, types of hemoglobinopathies, hemoglobinopathies' complications before and after the enrollment in the program, and assessment of hospital visits before and after enrollment in the program. Results: In November 2015, the chronic RBCX program was established in Nebraska. Since the start, 24 patients came through the program and 20 patients are still actively enrolled and undergoing regular exchange transfusions. The four patients who left the program did it for the following reasons: moving out of state, stem cell transplant and change to different treatment modality. Four of 24 patients were beta thalassemia patients (two of them with combined HbE/beta thalassemia). Twenty patients had sickle cell disease with two of them having combined beta thalassemia and HbS and one with alpha thalassemia and HbS. The indications ranged from history of stroke, intracranial vascular stenosis, acute chest syndrome (ACS), iron overload, multiple vascular occlusive crises (VOC) and intolerance of medications with most of the patients having multiple indications from the list above (Figure 1). There are several positive outcomes from being on the program. In the patients who had been on the program for at least one year (n=11), nine started the program with iron overload and all of them had a significant decrease in serum ferritin (average 751 ng/mL) with three patients returning to normal range. In the patients who had been in the program at least six months (n=16), 13 patients started with iron overload with five returning to normal range and average decrease in ferritin of 585 ng/mL. Another positive outcome is the number of emergency department (ED) visits for pain crisis. We noted reduction in ED visits in all patients who were in the program for at least six months (n=14), with the exception of one patient where the visits were likely the part of drug seeking behavior. In fact 12 of 13 patients had one or no ED visits within one year after starting on the chronic exchange program having had from 2-11 visits a year prior. None of the patients in the program experienced more severe complications of sickle cell disease, like stroke and acute chest syndrome, while on the program. Due to high volumes of transfusion, there is a big concern about developing red blood cell antibodies in sickle cell disease patients who in general have higher red blood cell antibody burden. Out of 24 patients in the program, six had pre-existing antibodies. For the duration of the program, no new alloantibodies were discovered in the chronically exchanged patients despite high transfusion volumes (range 14L-30L/year). The transfused blood was matched for Rh and Kell antigens for the patients with no antibody history. The patients with previous antibody history had additional matching for the antigen to which antibody was directed. Conclusion:Automated chronic RBCX transfusion program is safe to perform. It leads to significant reduction in volume overload and ED visits. Performing high volume transfusions outside of acute sickle cell crisis and with Rh and Kell matched units prevents formation of RBC antibodies Disclosures Gundabolu: BioMarin:Consultancy;Bristol Myers Squibb pharmaceuticals:Consultancy.

Sickle crisis in the critically ill

2016 ◽  
Author(s):  
Shilpa Jain ◽  
Mark T. Gladwin
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Systemic Infection ◽  
Red Blood Cell Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Ischaemia Reperfusion Injury ◽  
Transfusion Therapy

Sickle cell disease crises are precipitated by an acute occlusion of microvessels, which can lead to end organ ischaemia reperfusion injury and acute haemolysis. Acute fat emboli syndrome, acute lung injury (the acute chest syndrome), acute pulmonary hypertension, and cor pulmonale, haemorrhagic and occlusive stroke, and systemic infection represent the most common life-threatening complications observed in current ICU practice. General principles of management in all patients admitted to the critical care unit are hydration, antibiotics, pain control, and maintenance of oxygenation and ventilation. Red blood cell transfusion therapy is the treatment of choice for most complications of sickle cell disease requiring intensive care management. Transfusion of sickle negative, leukoreduced red blood cells, phenotypically matched for Rhesus and Kell antigens is the minimum standard of care in sickle cell disease patients as they have a high incidence of red blood cell alloimmunization.

Clinical Effects of Chronic Red Blood Cell Exchange and Different Types of Red Cell Concentrates in Patients with Sickle Cell Disease

Blood ◽  
2008 ◽  
Vol 112 (11) ◽  
pp. 4823-4823
Author(s):  
Sergio Cabibbo ◽  
Agostino Antolino ◽  
Giovanni Garozzo ◽  
Carmelo Fidone ◽  
Pietro Bonomo
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Iron Overload ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Whole Blood ◽  
Clinical Effects ◽  
Red Cell ◽  
Blood Components ◽  
Cell Disease

Abstract For patients with severe SCD not eligible for hydroxyurea, two major therapeutic options are currently available: blood transfusion, and bone marrow transplantation. Either urgent or chronic red blood cell transfusion therapy, is widely used in the management of SCD but determines a progressive increase of ferritin level and is also limited by the development of antibodies to red cell antigens. The introduction of chronic red blood cell exchange and prestorage filtration to remove leucocytes and the use of techniques for multicomponent donation could be a good solutions. Thus, the aims of our studies were to evaluate the clinical effects of the different blood components in terms of annual transfusion needs and the intervals between transfusion, moreover we evaluated the efficacy of chronic red blood cell exchange (manual or automatic with cell separator) in preventing SCD complications and limiting iron overload. In our center we follow 78 patients affected by Sickle Cell Disease. We selected 36 patients occasionally treated with urgent red blood cell exchange because they had less than 2 complications/Year, and 42 patients regularly treated with chronic red blood cell exchange because they had more than 2 complications/Year with Hospital Admission. Moreover among these we selected 10 patients for fulfilling the criteria of continuous treatment at the Centre for at least 48 months with no interruptions, even sporadic and absolute transfusion dependency. All 10 patients were evaluated for a period of 4 years, during which two different systems of producing RCC were used. In the second two the patients were transfused with RCC obtained from filtering whole blood prestorage or with RCC from apheresis filtered prestorage. These products differed from those used in the preceding two years, during which the leucodepletion was obtained by bed-side filtration For all the patients we performed 782 automatic red blood cell exchanges and 4421 units of RCC were transfused. The exchange procedures were extremely well-tolerated by the patients and adverse effects were limited to symptoms of hypocalcaemia during automatic red blood cell exchange with cell separator. After every red blood cell exchange we obtained HbS level < 30%. The10 patients selected received respectively a mean of 6.9 and 6.1 units of RBCs exchanged per automatic procedure, in the first two years and in the second two years. Alloantibody developed in 14 patients but only 2 clinically significant and about the observed frequency of transfusion reactions it was very low. All patients treated with chronic red blood cell exchange had an improvement of the quality of life with a reduced number of complications/year (<2/year) and good compliance and moreover patients had limited iron overload making chelating therapy easier. In conclusion this study was focused on the most suitable characteristics of blood components for use in sickle cell disease patients and the choice of systematically adopting prestorage filtration of whole blood, enabled us to have RCC with a higher Hb concentration than standard. Moreover chronic manual or automatic red blood cell exchange as an alternative approach to simple long-term RBC transfusions give many advantages by being more rapid and tolerable as well as clinically safe and effective and minimize the development of iron overload especially when procedure was carried out with an automatic apparatus. To note that the clinical advantages for patients derived from good selection of the donor and good practices in the production of the blood components

Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy

Blood ◽  
2000 ◽  
Vol 96 (1) ◽  
pp. 76-79 ◽  
Author(s):  
Paul Harmatz ◽  
Ellen Butensky ◽  
Keith Quirolo ◽  
Roger Williams ◽  
Linda Ferrell ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Liver Injury ◽  
Liver Biopsy ◽  
Blood Cell ◽  
Iron Overload ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Liver Iron ◽  
Transfusion Therapy

Chronic transfusion therapy is being used more frequently to prevent and treat the complications of sickle cell disease. Previous studies have shown that the iron overload that results from such therapy in other patient populations is associated with significant morbidity and mortality. In this study we examined the extent of iron overload as well as the presence of liver injury and the predictive value of ferritin in estimating iron overload in children with sickle cell disease who receive chronic red blood cell transfusions. A poor correlation was observed between serum ferritin and the quantitative iron on liver biopsy (mean 13.68 ± 6.64 mg/g dry weight;R = 0.350, P = .142). Quantitative iron was highly correlated with the months of transfusion (R = 0.795, P < .001), but serum ferritin at biopsy did not correlate with months of transfusion (R = 0.308, P = .200). Sixteen patients had abnormal biopsies showing mild to moderate changes on evaluation of inflammation or fibrosis. Liver iron was correlated with fibrosis score (R = 0.50, P = .042). No complications were associated with the liver biopsy. Our data suggest that, in patients with sickle cell disease, ferritin is a poor marker for accurately assessing iron overload and should not be used to direct long-term chelation therapy. Despite high levels of liver iron, the associated liver injury was not severe.

scholarly journals Red blood cell storage duration is not associated with clinical outcomes for acute chest syndrome in children with sickle cell disease

Transfusion ◽  
2015 ◽  
Vol 55 (11) ◽  
pp. 2714-2721 ◽  
Author(s):  
Melanie E. Fields ◽  
Monica L. Hulbert ◽  
Ling Chen ◽  
Ari N. Berlin ◽  
Ron Jackups ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Clinical Outcomes ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Storage Duration ◽  
Cell Storage

Severity of iron overload in patients with sickle cell disease receiving chronic red blood cell transfusion therapy

Blood ◽  
2000 ◽  
Vol 96 (1) ◽  
pp. 76-79 ◽  
Author(s):  
Paul Harmatz ◽  
Ellen Butensky ◽  
Keith Quirolo ◽  
Roger Williams ◽  
Linda Ferrell ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Liver Injury ◽  
Liver Biopsy ◽  
Blood Cell ◽  
Iron Overload ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Cell Disease ◽  
Liver Iron ◽  
Transfusion Therapy

Abstract Chronic transfusion therapy is being used more frequently to prevent and treat the complications of sickle cell disease. Previous studies have shown that the iron overload that results from such therapy in other patient populations is associated with significant morbidity and mortality. In this study we examined the extent of iron overload as well as the presence of liver injury and the predictive value of ferritin in estimating iron overload in children with sickle cell disease who receive chronic red blood cell transfusions. A poor correlation was observed between serum ferritin and the quantitative iron on liver biopsy (mean 13.68 ± 6.64 mg/g dry weight;R = 0.350, P = .142). Quantitative iron was highly correlated with the months of transfusion (R = 0.795, P &lt; .001), but serum ferritin at biopsy did not correlate with months of transfusion (R = 0.308, P = .200). Sixteen patients had abnormal biopsies showing mild to moderate changes on evaluation of inflammation or fibrosis. Liver iron was correlated with fibrosis score (R = 0.50, P = .042). No complications were associated with the liver biopsy. Our data suggest that, in patients with sickle cell disease, ferritin is a poor marker for accurately assessing iron overload and should not be used to direct long-term chelation therapy. Despite high levels of liver iron, the associated liver injury was not severe.

scholarly journals Partial Red Blood Cell Exchange in Children and Young Patients with Sickle Cell Disease: Manual Versus Automated Procedure

2017 ◽  
Vol 30 (10) ◽  
pp. 727 ◽  
Author(s):  
Carlos Escobar ◽  
Marta Moniz ◽  
Pedro Nunes ◽  
Clara Abadesso ◽  
Teresa Ferreira ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Blood Cell ◽  
Iron Overload ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Young Patients ◽  
Limiting Factor ◽  
Cell Disease ◽  
Chronic Complications ◽  
Sickle Hemoglobin

Introduction: The benefits of manual versus automated red blood cell exchange have rarely been documented and studies in young sickle cell disease patients are scarce. We aim to describe and compare our experience in these two procedures.Material and Methods: Young patients (≤ 21 years old) who underwent manual- or automated-red blood cell exchange for prevention or treatment of sickle cell disease complications were included. Clinical, technical and hematological data were prospectively recorded and analyzed.Results: Ninety-four red blood cell exchange sessions were performed over a period of 68 months, including 57 manual and 37 automated, 63 for chronic complications prevention, 30 for acute complications and one in the pre-operative setting. Mean decrease in sickle hemoglobin levels was higher in automated-red blood cell exchange (p < 0.001) and permitted a higher sickle hemoglobin level decrease per volume removed (p < 0.001), while hemoglobin and hematocrit remained stable. Ferritin levels on chronic patients decreased 54%. Most frequent concern was catheter outflow obstruction on manual-red blood cell exchange and access alarm on automated-red blood cell exchange. No major complication or alloimunization was recorded.Discussion: Automated-red blood cell exchange decreased sickle hemoglobin levels more efficiently than manual procedure in the setting of acute and chronic complications of sickle cell disease, with minor technical concerns mainly due to vascular access. The threshold of sickle hemoglobin should be individualized for clinical and hematological goals. In our cohort of young patients, the need for an acceptable venous access was a limiting factor, but iron-overload was avoided.Conclusion: Automated red blood cell exchange is safe and well tolerated. It permits a higher sickle hemoglobin removal efficacy, better volume status control and iron-overload avoidance.

scholarly journals Blood Transfusion in Adult Patients with Sickle Cell Disease: Incidence of Alloimmunization

Blood ◽  
2016 ◽  
Vol 128 (22) ◽  
pp. 4859-4859 ◽  
Author(s):  
Samip Master ◽  
Menchu Ong ◽  
Richard Preston Mansour
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Blood Cell ◽  
Sickle Cell ◽  
Red Blood Cell ◽  
Disease Incidence ◽  
Adult Patients ◽  
Red Blood Cell Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease

Abstract Background: Chronic red blood cell transfusion has been proven to be effective in prevention of strokes, silent cerebral infarcts, acute chest syndrome, recurrent priapism and in pregnancy. The use of regular transfusions to mitigate other morbidities of sickle cell disease (SCD) is evolving. In the silent infarct transfusion (SIT) trial in children, chronic transfusion lead to a significant improvement in quality of life. Some of the common reasons patient with SCD do not get chronic transfusion is fear of alloimmunization, iron over load and risk of viral infections. We did a retrospective analysis of adult patients with SCD who need chronic blood transfusion to determine the incidence of alloimmunization. At our institute all pediatric sickle cell patients needing chronic transfusion are placed on protocol, receive C, E, and K matched blood, and remain on the protocol until they become adults. Methods: We electronically collected data from 180 adult SCD patients who need chronic transfusions and analyzed the data for the number of transfusions received, incidence of allo- immunization and most common antibodies identified. Results: A total of 3967 red blood cell transfusions were administered on 180 adult sickle cell disease patients. Twenty five patients developed antibodies (13.8 %). Fifteen out of the 25 had multiple antibodies (60%). The alloantibodies identified were : anti- K(11), anti- E(12), anti- Fya(5), anti-C (4), anti-V (4), anti- S (3), anti-D (2), anti- Jkb (1), anti-Jsa(1) , and anti- Lutherana (3). Two patients had cold and 5 patients had warm autoantibodies. Conclusion: The policy to place patients with SCD needing chronic transfusion on protocol to receive C, E, and K matched red blood cells has decreased the alloimmunization rate to 13.8 %. We conclude that, fear of alloimmunization should not preclude physicians from using chronic red cell transfusions to prevent complications in sickle cell disease. Disclosures No relevant conflicts of interest to declare.

Delayed Hemolytic Transfusion Reactions in Adults with Sickle Cell Disease: Experience of a Single Institution in the UK

Blood ◽  
2014 ◽  
Vol 124 (21) ◽  
pp. 4079-4079
Author(s):  
Jennifer Vidler ◽  
Kate Gardner ◽  
Aleksandar Mijovic ◽  
Swee Lay Thein
Keyword(s):  
Sickle Cell Disease ◽  
Blood Transfusion ◽  
Blood Cell ◽  
Sickle Cell ◽  
Acute Pain ◽  
Red Blood Cell ◽  
Adult Patients ◽  
Red Blood Cell Transfusion ◽  
Acute Chest Syndrome ◽  
Cell Disease

Abstract Background: Blood transfusion is a key intervention in the management of sickle cell disease (SCD), and is being increasingly used. Nonetheless, transfusion is not without risks; alloimmunisation to red blood cell antigens is a major complication, and can sometimes precipitate a delayed haemolytic transfusion reaction (DHTR), a potentially life-threatening event. Objectives: We describe the prevalence, risk factors and experience of DHTR in a large cohort of adult patients with SCD. Methods: Medical records of the 637 adult patients (all of African descent) regularly attending the SCD specialist clinic at King’s College Hospital, London, were retrospectively reviewed to identify DHTR cases. 362 (57%) were female, with 401 HbSS, 202 HbSC, 29 HbSβ+-thalassaemia (thal), 4 HbSβ0-thal, and 1 HbSSHPFH patients, mean age 36 ± 12 years. Between 1st August 2008 and 31st December 2013, 219 of the 637 patients received red blood cell transfusion, either as simple or exchange transfusion. 123 /219 (56%) of those who received transfusion were female, 84% HbSS genotype. Their Electronic Patient Records were examined, looking for a sharp drop in haemoglobin (Hb) after transfusion. If this was observed, laboratory data were combined with the clinical notes to detect evidence of a DHTR. Results: We identified 25 DHTR episodes (1.2% of all transfusion episodes) in 16 patients (table 1). Six patients had repeat DHTR episodes – 4 twice, one thrice, and one 4 times. Mean age at transfusion was 35.5 ± 14.8 years. Indications for the transfusion that triggered the DHTR, included 20 acute pain episodes (some with acute chest syndrome), 3 pre-operative and 2 chronic exchange program. Mean interval from transfusion to DHTR onset was 11 ± 7 days. Typical presentations of DHTR were fever, pain and hemoglobinuria. Blood results at DHTR diagnosis showed evidence of active haemolysis (mean LDH 1330 IU/L), and Hb drop (mean drop 40.4g/L, range 7 – 88g/L). 84% of episodes showed a severe haemolysis with nadir Hb lower than the pre-transfusion Hb. Mean reticulocyte count at peak haemolysis was 291 x109/L ± 121 x109/L. Eleven of the 25 episodes (44%) resulted in new red cell antibodies; 8 alloantibodies and 3 autoantibodies (table 1). DAT was positive in 16 of 19 (84%) cases where performed. 56% (14/25) of DHTR episodes were not diagnosed during admission, most often they were misdiagnosed as an acute pain crisis. Four of the 11 recognised DHTRs were treated with immunosuppression that included methylprednisolone, immunoglobulin, and, in one case, rituximab. All four of these uneventfully received further blood transfusions. The mean length of hospital stay was 15.9 days. 2/16 patients died, one of stroke, one of multi organ failure, giving a 13% mortality. Discussion: Our data suggest that DHTRs are a severe but uncommon complication of blood transfusion. They are poorly recognised, possibly as their presentation mimics an acute painful crisis. Notably, most of the DHTRs are triggered by RBC transfusions in the acute setting. We recommend a high index of suspicion for DHTR in any SCD patient who has been transfused in the past month and presents acutely to clinicians, as early intervention can be life-saving. Table 1. Table 1. *Exchange Disclosures No relevant conflicts of interest to declare.

Sign in / Sign up

Export Citation Format

Share Document