Regular automated red cell exchange transfusion in the management of stuttering priapism complicating sickle cell disease

Ekong, Anietie; Berg, Lauren; Amos, R.; Tsitsikas, Dimitris A.

doi:10.1111/bjh.14393

Cited by 11 publications

(7 citation statements)

References 8 publications

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“…Published outcomes for chronic aRCE have included significant reductions in ED visits and hospitalizations for VOC, avoidance of new end-organ damage, improved growth in children, and variable improvements in chronic leg ulcers and priapism. 19,95,[98][99][100][101] In our aRCE program we have observed reductions in hospitalizations and improvements in quality of life. A consistent team approach is key to managing a successful program.…”

Section: Automated Red Cell Exchange Outcomes Multidisciplinary Appro...mentioning

confidence: 88%

Comprehensive guide to managing a chronic automated red cell exchange program in sickle cell disease

Cunard

Gopal

Kopko

et al. 2022

J of Clinical Apheresis

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Sickle cell disease (SCD) is associated with significant morbidity and mortality, and limits both the quality and quantity of life. Transfusion therapy, specifically automated red cell exchange (aRCE), plays a key role in management of SCD and is beneficial for certain indications in the chronic, outpatient setting. The approach to maintain a successful chronic aRCE program for SCD is multifaceted. This review will highlight important considerations including indications for aRCE, patient selection, transfusion medicine pearls, vascular access needs, complications of therapy, aRCE prescription, and therapy optimization.Moreover, the importance of a multidisciplinary approach with frequent communication between the services involved cannot be overstated. Ultimately, the underlying goal of a chronic RCE program is to improve the quality of life and longevity of patients with SCD.

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Section: Automated Red Cell Exchange Outcomes Multidisciplinary Appro...mentioning

confidence: 88%

Comprehensive guide to managing a chronic automated red cell exchange program in sickle cell disease

Cunard

Gopal

Kopko

et al. 2022

J of Clinical Apheresis

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“…We previously reported an excellent response of cases with severe and drug resistant SP to a-RCE [17]. This involved five patients, four of whom were on the programme for a different primary indication (three SSP and one LU), while one patient was started specifically to manage his SP.…”

Section: Priapismmentioning

confidence: 99%

Automated Red Cell Exchange in the Management of Sickle Cell Disease

Tsitsikas

Badle

Hall

et al. 2021

JCM

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Red cell transfusion represents one of the cornerstones of the chronic management of sickle cell disease, as well as its acute complications. Automated red cell exchange can rapidly lower the number of circulating sickle erythrocytes, without causing iron overload. Here, we describe our experience, having offered this intervention since 2011. A transient reduction in the platelet count by 61% was observed after the procedure. This was not associated with any haemorrhagic complications. Despite exposure to large volumes of blood, the alloimmunisation rate was only 0.027/100 units of red cells. The absence of any iron loading was confirmed by serial Ferriscans, performed over a number of years. However, patients with advanced chronic kidney disease showed evidence of iron loading due to reduced innate haemopoiesis and were subsequently switched to simple transfusions. A total of 59% of patients were on regular automated red cell exchange with a history of recurrent painful crises. A total of 77% responded clinically, as evidenced by at least a 25% reduction in their emergency hospital attendance for pain management. The clinical response was gradual and increased the longer patients stayed on the program. The earliest sign of clinical response was a reduction in the length of stay when these patients were hospitalised, indicating that a reduction in the severity of crises precedes the reduction in their frequency. Automated red cell exchange also appeared to be beneficial for patients with recurrent leg ulcers and severe, drug resistant stuttering priapism, while patients with pulmonary hypertension showed a dramatic improvement in their symptoms as well as echocardiographic parameters.

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“…Some small recent studies have shown that exchange to a low HbS level (in one study ≤30% 20 and in another study ≤16% 21 ), with Hb level of approximately 10 g/dL in the acute setting, has been helpful in resolving priapism. [20][21][22] One institution represented by the authors noted that chronic RCEs seemed to be helpful in preventing recurrent episodes of priapism in at least two patients.…”

Section: Indications For Rcementioning

confidence: 99%

Red blood cell exchange in patients with sickle cell disease—indications and management: a review and consensus report by the therapeutic apheresis subsection of the AABB

et al. 2018

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Although a consensus as to protocol details may not be possible, there are areas of agreement in the management of these patients, for example, that it is optimal to avoid hyperviscosity and iron overload, that a target Hb S level in the range of 30% is generally desirable, and that RCE as an acute treatment for pain crisis in the absence of other acute or chronic conditions is ordinarily discouraged.

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Regular automated red cell exchange transfusion in the management of stuttering priapism complicating sickle cell disease

Cited by 11 publications

References 8 publications

Comprehensive guide to managing a chronic automated red cell exchange program in sickle cell disease

Comprehensive guide to managing a chronic automated red cell exchange program in sickle cell disease

Automated Red Cell Exchange in the Management of Sickle Cell Disease

Red blood cell exchange in patients with sickle cell disease—indications and management: a review and consensus report by the therapeutic apheresis subsection of the AABB

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