scholarly journals Role of Emergency Automated Red Cell Exchange in Sickle Cell Crisis: A Case Report

2020 ◽  
Vol 13 ◽  
pp. 117954762097020
Author(s):  
Anubhav Gupta ◽  
Kiran Chaudhary ◽  
Rajnish Kaushik
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Symptomatic Improvement ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Compound Heterozygous ◽  
Red Cell ◽  
Transfusion Therapy ◽  
Sickle Cell Crisis ◽  
Patient At Risk

For many years main stay of treatment for sickle cell anaemia was transfusion therapy. But repeated transfusions put the patient at risk of iron overload. Automated red cell exchange is an evolving and newer technique which rapidly removes the sickle cells and has benefit in decreasing sickle cell load and related complications. Red cell exchange is a therapeutic procedure in which the patient’s whole blood is processed centrifugally in cell separator. Patient’s red cells are separated from other blood components and removed and replaced with donor red cells and colloids. We report our first experience of automated red cell exchange in 24-year-old female diagnosed case of sickle cell anaemia presented to us with acute chest syndrome with septic shock. Red cell exchange was planned to tide over the acute sickle cell crisis and provide symptomatic improvement. We also highlight that compound heterozygous thalassaemia could be associated with sickle cell disease which could make the diagnosis difficult. New generation automated Apheresis equipment’s provides better monitoring of the procedure that can be useful in severely ill patients also.

scholarly journals Minimising Transfusion Therapy and Alloimmunisation in Patients with Sickle Cell Disorder in the Era of Hydroxyurea

Blood ◽  
2018 ◽  
Vol 132 (Supplement 1) ◽  
pp. 5078-5078
Author(s):  
Louise Smith ◽  
Lucy Stead ◽  
Russell D Keenan ◽  
Rekha Thangavelu
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Conflicts Of Interest ◽  
Cell Phenotype ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Blood Products ◽  
Related Complication ◽  
Transfusion Therapy ◽  
Historical Presence

Abstract In the UK, 700 patients with sickle cell disease are on a transfusion programme1. Red blood cell (RBC) AI occurs in 4.4-76%2 of regularly transfused sickle patients. Contributing factors include repeated transfusions and ethnic differences between sickle cell patients and their donors. This results in higher rates of mismatch in phenotyped and genotyped blood. There is a continued lack of availability of blood products from more compatible ethnic donors. Sickle patients on transfusion programmes are transfused every 3-6 weeks, creating a large burden on healthcare services in terms of time, labour and resources such as extended red cell typing in order to reduce antibody formation. The presence of RBC antibodies in patients can cause significant delays in obtaining cross matched blood and reductions in phenotype matched blood being transfused. The aims of this project were to a) review transfusion use in relation to the use of hydroxyurea (HU) patients treated at Alder Hey (AH) and b) review rates of AI in these patients. At AH we have changed our practice to offer HU from age 9 months as a disease modifying therapy 20-35mg/kg/day. We retrospectively reviewed the use of blood products in our cohort of 60 patients, age between 0-20 years (mean 11yrs). Patients were observed over their life period treated at AH, mean 10.1 patient years, range 0-20years, total of 439 patient years. We studied retrospectively the number of RBC transfusions and the historical presence of antibodies. We then re-tested all patients to determine current antibody status. Prior to commencement of hydroxyurea, 342 units of bloods were transfused to 21 patients over 243 patient years, which is 1.41 units per patient per year. After commencement of hydroxyurea, 114.5 units were transfused to 17 patients in 187.1 patient years, 0.61 units per patient per year. Indications for transfusion included acute chest syndrome, aplastic crisis, prolonged crisis, increased acute anaemia, hydroxyurea induced anaemia, pre-operatively. 3 patients in our cohort had previous antibodies. Upon repeat antibody screening, no patients had detectable RBC antibodies. All patients have an extended red cell phenotype as part of their initial workup on referral to our service. Elective, non-acute patients (transfusion programme, pre-operative, hydroxycarbamide induced anaemia) all receive fully phenotyped blood. This cannot be guaranteed in acute situations where patients receive the best matched blood available. This practice of minimising blood transfusion use in patients with sickle cell anaemia can reduce blood product usage and as a result reduce the demand for ethnic minority transfusion donors. The subsequent donor exposure would therefore also decrease transfusion related complication of AI. 1 National Haemoglobinopathy Registry Annual Report 2016/17 2G. da Cunha Gomes, E & A. F. Machado, L & C. de Oliveira, L & F. N. Neto, J. (2018). The erythrocyte alloimmunisation in patients with sickle cell anaemia: a systematic review: Erythrocyte alloimmunisation in sickle cell anaemia. Transfusion Medicine. 10.1111/tme.12543. Disclosures No relevant conflicts of interest to declare.

Extended, Flexible Program Matching Red Cell Antigens for Patients with Sickle Cell Anemia Provides for Both Chronic and Intermittent Transfusions.

Blood ◽  
2007 ◽  
Vol 110 (11) ◽  
pp. 2899-2899
Author(s):  
Daniel R. Ambruso ◽  
Michele LaSalle-Williams ◽  
Tuan Le ◽  
Laura Cole ◽  
Kathy Hassell ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anemia ◽  
Perfect Match ◽  
Clinical Situation ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Blood Group Antigens ◽  
Red Cell ◽  
Transfusion Therapy ◽  
Antibody Screen

Abstract Introduction: Transfusion of packed red cells (PRBCs) remains the major treatment of severe complications in sickle cell anemia. Patients may require acute, intermittent transfusions for some problems while other, more severe complications are treated with chronic transfusion (≥ 6 months) regimens to replace and suppress production of sickle cells. One of the main complications of transfusion therapy is production of red cell alloantibodies which significantly increases the risk of subsequent transfusions and limits the timely provision of appropriate blood products. Since 1978, we have provided sickle cell patients with extended matching of PRBCs to reduce the rate of alloimmunization. The flexibility of the program is adapted to meet both acute and chronic needs of the patient population. Methods: Records of patients with sickle hemoglobinopathies enrolled in the Colorado Sickle Cell Treatment and Research Center between December 31, 1993 and January 1, 2006 were reviewed under a protocol approved by the COMIRB at UCDHSC. At enrollment, serologic testing was completed on patients for the following blood group antigens: ABO; Rhesus (C,c,D,E,e); Kell (K,k); Duffy (Fya,Fyb); Kidd (Jka,Jkb); Lewis (Lea,Leb); and MNS (M,N,S,s). Donors were typed for the same antigens. For all transfusions, a perfect match was sought. When exact matches at all loci could not be found, mismatches were allowed when necessary for Fyb and MNS because of lower risk of sensitization and for Le because of infrequent hemolytic transfusion reactions. Antibody screens were completed as part of crossmatch technique at the time of each transfusion by standard methods. When an antibody screen was positive, standard antiglobulin and enzyme techniques and cell panels were used to identify the antibody. For the purposes of this study, information about patients with sickle cell anemia, complications, numbers and types of transfusions were reviewed. Results: Over the past 13 years, a total of 6,978 transfusions were provided to 104 patients (mean 68, range 1–519). In this group, 90 patients (86.5%) had HbSS, 11 (10.6%) had HbSC, and 3 (2.9%) had HbS β-thalassemia. Indications for transfusions included vaso-occlusive crisis, aplastic crises, splenic sequestration, and preparation for surgery, acute chest syndrome, stroke and priapism. Fifty-two patients (50%) received simple transfusions only while 33 (32%) had only PRBC exchange and 19 (18%) required both modalities. Of the total group, 42 (40%) were on chronic transfusions and 11 (10%) had both intermittent and chronic transfusions. The protocol and system for delivering matched PRBCs was flexible enough that even when red cells were needed for intermittent transfusions in 62 (60%) patients, delivery of the products was easily accomplished. In only 8 patients receiving 13 units of PRBCs was the clinical situation so urgent that non-antigen matched units was required. Conclusion: PRBCs with extended antigen matching were provided to our population of patients with sickle cell anemia. The program provided blood components for both intermittent and chronic transfusion schemes with very few patients having such urgent conditions that they required products without extended matching.

scholarly journals Red cells in sickle cell crisis: observations on the pathophysiology of crisis

Blood ◽  
1977 ◽  
Vol 49 (6) ◽  
pp. 967-979 ◽  
Author(s):  
EE Rieber ◽  
G Veliz ◽  
S Pollack
Keyword(s):  
Sickle Cell ◽  
Red Cells ◽  
Red Cell ◽  
Molecular Abnormality ◽  
Sickle Cell Crisis ◽  
Sickle Cell Crises

Abstract The pathophysiology of the occurrence and resolution of sickle cell crisis is unknown. The molecular abnormality is constant, while crisis is episodic. In the present study, red cell filterability and sickling with deoxygenation have been measured during sickle cell crises. Recovery from sickle crisis is associated with an increased filterability of the circulating red cell and a decreased susceptibility of the red cell to sickle with deoxygenation (p less than 0.05). The possibility that these changes are responsible for the resolution of crisis is suggested.

Red cells in sickle cell crisis: observations on the pathophysiology of crisis

Blood ◽  
1977 ◽  
Vol 49 (6) ◽  
pp. 967-979
Author(s):  
EE Rieber ◽  
G Veliz ◽  
S Pollack
Keyword(s):  
Sickle Cell ◽  
Red Cells ◽  
Red Cell ◽  
Molecular Abnormality ◽  
Sickle Cell Crisis ◽  
Sickle Cell Crises

The pathophysiology of the occurrence and resolution of sickle cell crisis is unknown. The molecular abnormality is constant, while crisis is episodic. In the present study, red cell filterability and sickling with deoxygenation have been measured during sickle cell crises. Recovery from sickle crisis is associated with an increased filterability of the circulating red cell and a decreased susceptibility of the red cell to sickle with deoxygenation (p less than 0.05). The possibility that these changes are responsible for the resolution of crisis is suggested.

scholarly journals The role of WNK in modulation of KCl cotransport activity in red cells from normal individuals and patients with sickle cell anaemia

2019 ◽  
Vol 471 (11-12) ◽  
pp. 1539-1549
Author(s):  
David C.-Y. Lu ◽  
Anke Hannemann ◽  
Rasiqh Wadud ◽  
David C. Rees ◽  
John N. Brewin ◽  
...  
Keyword(s):  
Oxygen Tension ◽  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Kinase Inhibitors ◽  
Red Cells ◽  
Red Cell ◽  
Sickle Cells ◽  
Kcl Cotransport ◽  
Pre Treatment

AbstractAbnormal activity of red cell KCl cotransport (KCC) is involved in pathogenesis of sickle cell anaemia (SCA). KCC-mediated solute loss causes shrinkage, concentrates HbS, and promotes HbS polymerisation. Red cell KCC also responds to various stimuli including pH, volume, urea, and oxygen tension, and regulation involves protein phosphorylation. The main aim of this study was to investigate the role of the WNK/SPAK/OSR1 pathway in sickle cells. The pan WNK inhibitor WNK463 stimulated KCC with an EC50 of 10.9 ± 1.1 nM and 7.9 ± 1.2 nM in sickle and normal red cells, respectively. SPAK/OSR1 inhibitors had little effect. The action of WNK463 was not additive with other kinase inhibitors (staurosporine and N-ethylmaleimide). Its effects were largely abrogated by pre-treatment with the phosphatase inhibitor calyculin A. WNK463 also reduced the effects of physiological KCC stimuli (pH, volume, urea) and abolished any response of KCC to changes in oxygen tension. Finally, although protein kinases have been implicated in regulation of phosphatidylserine exposure, WNK463 had no effect. Findings indicate a predominant role for WNKs in control of KCC in sickle cells but an apparent absence of downstream involvement of SPAK/OSR1. A more complete understanding of the mechanisms will inform pathogenesis whilst manipulation of WNK activity represents a potential therapeutic approach.

scholarly journals Red cell transfusion and alloimmunization in sickle cell disease

Haematologica ◽  
2021 ◽  
Author(s):  
Grace E. Linder ◽  
Stella T. Chou
Keyword(s):  
Sickle Cell Disease ◽  
Adverse Effects ◽  
Sickle Cell ◽  
Randomized Clinical Trials ◽  
Acute Chest Syndrome ◽  
Red Cell ◽  
Cell Disease ◽  
Transfusion Therapy ◽  
Red Cell Transfusion ◽  
Transfusion Reactions

Red cell transfusion remains a critical component of care for acute and chronic complications of sickle cell disease. Randomized clinical trials demonstrated the benefits of transfusion therapy for prevention of primary and secondary strokes and postoperative acute chest syndrome. Transfusion for splenic sequestration, acute chest syndrome, and acute stroke are guided by expert consensus recommendations. Despite overall improvements in blood inventory safety, adverse effects of transfusion are prevalent among patients with sickle cell disease and include alloimmunization, acute and delayed hemolytic transfusion reactions, and iron overload. Judicious use of red cell transfusions, optimization of red cell antigen matching, and the use of erythrocytapheresis and iron chelation can minimize adverse effects. Early recognition and management of hemolytic transfusion reactions can avert poor clinical outcomes. In this review, we discuss transfusion methods, indications, and complications in sickle cell disease with an emphasis on alloimmunization.

scholarly journals Pathophysiological Relevance of Renal Medullary Conditions on the Behaviour of Red Cells From Patients With Sickle Cell Anaemia

2021 ◽  
Vol 12 ◽  
Author(s):  
David C.-Y. Lu ◽  
Rasiqh Wadud ◽  
Anke Hannemann ◽  
David C. Rees ◽  
John N. Brewin ◽  
...  
Keyword(s):  
Sickle Cell ◽  
Sickle Cell Anaemia ◽  
Renal Medulla ◽  
Red Cells ◽  
Red Cell ◽  
Sickle Cells ◽  
Hypoxic Conditions ◽  
Intracellular Ca ◽  
Concentrate Urine ◽  
Abnormal Haemoglobin

Red cells from patients with sickle cell anaemia (SCA) contain the abnormal haemoglobin HbS. Under hypoxic conditions, HbS polymerises and causes red cell sickling, a rise in intracellular Ca2+ and exposure of phosphatidylserine (PS). These changes make sickle cells sticky and liable to lodge in the microvasculature, and so reduce their lifespan. The aim of the present work was to investigate how the peculiar conditions found in the renal medulla – hypoxia, acidosis, lactate, hypertonicity and high levels of urea – affect red cell behaviour. Results show that the first four conditions all increased sickling and PS exposure. The presence of urea at levels found in a healthy medulla during antidiuresis, however, markedly reduced sickling and PS exposure and would therefore protect against red cell adherence. Loss of the ability to concentrate urine, which occurs in sickle cell nephropathy would obviate this protective effect and may therefore contribute to pathogenesis.

scholarly journals Vaso-occlusion by sickle cells: evidence for selective trapping of dense red cells

Blood ◽  
1986 ◽  
Vol 68 (5) ◽  
pp. 1162-1166 ◽  
Author(s):  
DK Kaul ◽  
ME Fabry ◽  
RL Nagel
Keyword(s):  
Density Gradient ◽  
Sickle Cell ◽  
Morphological Analysis ◽  
Microvascular Obstruction ◽  
Perfusion Pressure ◽  
Peripheral Resistance ◽  
Red Cells ◽  
Arterial Perfusion ◽  
Sickle Cells ◽  
Sickle Cell Crisis

We have characterized the type of red cells from sickle cell patients that were trapped in the course of sickle-cell vaso-occlusion. In addition, the perfusion conditions (arterial perfusion pressure [Pa] and oxygen tension [PO2]) leading to experimentally induced vaso- occlusion in the artificially perfused, innervated mesocecum microvascular preparation were determined. Microvascular obstruction was induced by decrease in Pa; the lower the Pa, the greater the peripheral resistance as well as the extent of obstruction. The cells involved in the obstruction were recovered by vasodilation (secondary to denervation) and increase in Pa. Densitometric analysis of density gradient-separated infused and trapped cells was supplemented with morphological analysis to ascertain the involvement of density classes as well as morphological types seen in oxy and deoxy sickle blood. The trapping phenomenon was sensitive to PO2. Percentage of densest gradient classes, ie, fraction 3 (F3; mainly dense unsicklable SS discocytes [USDs]) and fraction 4 (F4; irreversibly sickled cells [ISCs] and the densest discocytes), showed a significant increase in trapping when perfusion was switched from oxy to deoxy perfusate. Morphological analysis revealed that unsicklable SS discocytes are more effectively trapped when deoxygenated. The deoxygenation of infused cells did not further change the percentage of ISCs trapped, suggesting that ISCs are equally capable of sequestration in the oxy and the deoxy states. The venous effluent showed a selective and significant depletion of dense cells (F4) and ISC counts at all Pa. We conclude that the progressive obstruction of the microcirculation by sickle cells involves selective sequestration of the densest classes of cells and that this mechanism might explain their partial disappearance during painful sickle cell crisis.

scholarly journals Microfluidics in Sickle Cell Disease Research: State of the Art and a Perspective Beyond the Flow Problem

2021 ◽  
Vol 7 ◽  
Author(s):  
Anupam Aich ◽  
Yann Lamarre ◽  
Daniel Pereira Sacomani ◽  
Simone Kashima ◽  
Dimas Tadeu Covas ◽  
...  
Keyword(s):  
Sickle Cell Disease ◽  
Sickle Cell ◽  
Flow Problem ◽  
Red Cells ◽  
Acute Chest Syndrome ◽  
Cell Disease ◽  
Intercellular Interaction ◽  
Blood Disorder ◽  
Resource Setting ◽  
Wide Range

Sickle cell disease (SCD) is the monogenic hemoglobinopathy where mutated sickle hemoglobin molecules polymerize to form long fibers under deoxygenated state and deform red blood cells (RBCs) into predominantly sickle form. Sickled RBCs stick to the vascular bed and obstruct blood flow in extreme conditions, leading to acute painful vaso-occlusion crises (VOCs) – the leading cause of mortality in SCD. Being a blood disorder of deformed RBCs, SCD manifests a wide-range of organ-specific clinical complications of life (in addition to chronic pain) such as stroke, acute chest syndrome (ACS) and pulmonary hypertension in the lung, nephropathy, auto-splenectomy, and splenomegaly, hand-foot syndrome, leg ulcer, stress erythropoiesis, osteonecrosis and osteoporosis. The physiological inception for VOC was initially thought to be only a fluid flow problem in microvascular space originated from increased viscosity due to aggregates of sickled RBCs; however, over the last three decades, multiple molecular and cellular mechanisms have been identified that aid the VOC in vivo. Activation of adhesion molecules in vascular endothelium and on RBC membranes, activated neutrophils and platelets, increased viscosity of the blood, and fluid physics driving sickled and deformed RBCs to the vascular wall (known as margination of flow) – all of these come together to orchestrate VOC. Microfluidic technology in sickle research was primarily adopted to benefit from mimicking the microvascular network to observe RBC flow under low oxygen conditions as models of VOC. However, over the last decade, microfluidics has evolved as a valuable tool to extract biophysical characteristics of sickle red cells, measure deformability of sickle red cells under simulated oxygen gradient and shear, drug testing, in vitro models of intercellular interaction on endothelialized or adhesion molecule-functionalized channels to understand adhesion in sickle microenvironment, characterizing biomechanics and microrheology, biomarker identification, and last but not least, for developing point-of-care diagnostic technologies for low resource setting. Several of these platforms have already demonstrated true potential to be translated from bench to bedside. Emerging microfluidics-based technologies for studying heterotypic cell–cell interactions, organ-on-chip application and drug dosage screening can be employed to sickle research field due to their wide-ranging advantages.

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