RBC Exchange Pheresis for Priapism in Sickle Cell Disease

Rifkind, Stephen; Waisman, James; Thompson, R P; Goldfinger, Dennis

doi:10.1001/jama.1979.03300210043021

Cited by 44 publications

(10 citation statements)

References 11 publications

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“…Accordingly, the number of children who joined our program increased steadily, and many of the patients had severe complications of SCD. Second, several reports described the use of some forms of partial or complete RBC exchange transfusion in children and adults to treat severe complications of SCD, especially sickle cell anemia (SCA), with favorable outcomes for some patients …”

Section: The Haemonetics Centrifugal Intermittent Apheresis Systemmentioning

confidence: 99%

From total blood exchange to erythrocytapheresis and back to treat complications of sickle cell disease

Ballas

2017

Transfusion

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Erythrocytapheresis is an important procedure in the management of certain complications of sickle cell disease, including acute stroke, stroke prevention, acute chest syndrome, and multiorgan failure. Erythrocytapheresis in sickle cell disease simply entails the removal of the patient's red blood cells containing the abnormal sickle hemoglobin and replacing them with normal red blood cells carrying normal hemoglobin. In these procedures, the patient's plasma is not exchanged but is returned to the patient. Several studies have demonstrated that the plasma of patients with sickle cell disease contains several components that increase blood viscosity and initiate or promote vaso-occlusion. These factors include increased levels of globulins, especially immunoglobulin G, acute-phase reactants, fibrinogen, coagulation factors, inflammatory mediators, and heme in the steady state and increase further during painful crises. This may explain why, in certain complications of sickle cell disease, such as acute chest syndrome, hepatic crisis, and priapism, erythrocytapheresis by itself may not be effective despite repetitive cycles of red blood cell exchange. The use of therapeutic plasma exchange in addition to erythrocytapheresis in these situations seems to be useful in resolving them more efficiently. The role of therapeutic plasma exchange in the management of certain complications of sickle cell disease needs further evaluation. This commentary addresses the role of therapeutic plasma exchange in the management of complications of sickle cell disease.

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Section: The Haemonetics Centrifugal Intermittent Apheresis Systemmentioning

confidence: 99%

From total blood exchange to erythrocytapheresis and back to treat complications of sickle cell disease

Ballas

2017

Transfusion

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“…The investigators concluded that routine use of transfusion in the treatment of priapism was not supported. 39 In a small number of case reports and series, erythrocytapheresis has been associated with variable clinical response, ranging from resolution within 6-8 hours 40 to no resolution. 41 Some sickle cell patients with priapism who undergo RCE have also experienced a serious neurological complication known as Aspen's syndrome (which is an acronym for association of sickle cell disease, priapism, exchange transfusion, and neurological events).…”

Section: Priapismmentioning

confidence: 99%

Red cell exchange: special focus on sickle cell disease

Kim

2014

Hematology

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The primary function of red blood cells (RBCs) is to deliver oxygen from the lungs to tissues. Tissue hypoxia occurs when the oxygen-carrying capacity of RBCs is compromised due primarily to 3 causes: (1) a reduction in circulating RBC mass, (2) an increase in circulating RBC mass, or (3) abnormal hemoglobin (Hb) that either does not sufficiently release oxygen to tissues (high-oxygen-affinity hemoglobin) or occludes the microvasculature due to deformed RBCs (sickled RBCs). To improve oxygenation in patients with reduced or increased RBC mass, RBC administration (simple transfusion) or RBC removal (RBC depletion) is performed, respectively. However, for patients with abnormal Hb, RBCs containing abnormal Hb are removed and replaced by healthy volunteer donor RBCs by red cell exchange (RCE). RCE can be performed by manual exchange or by automated exchange using a blood cell separator (erythrocytapheresis). In this review, indications for RCE in sickle cell disease using the evidence-based American Society for Apheresis categories 1 are presented and the rationale for RCE in each disorder are discussed. Simple transfusion versus RCE and manual RCE versus automated RCE are compared. Finally, this review briefly presents some of the challenges of performing erythrocytapheresis in small children and discusses various choices for central venous access during RCE. 2 Learning Objectives• To describe indications for RCE • To define goals for RCE for stroke prophylaxis in sickle cell disease • To discuss benefits versus risks of automated RCE compared with simple transfusion and manual RCE.

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“…Case studies have suggested improvement in some patients with acute priapism after exchange 71,72 or simple transfusion therapy. 73,74 However, no controlled trial has been performed to establish the effectiveness of transfusion therapy in comparison with hydration, analgesia, stilbestrol, 27 or hydralazine 75,76 therapies for the treatment of acute priapism.…”

Section: Priapismmentioning

confidence: 99%