Automated Erythrocytopheresis for Relief of Priapism in Sickle Cell Hemoglobinopathies

Abstract

Priapism, a complication of male patients with sickle hemoglobinopathies, has been managed by a variety of surgical and nonsurgical forms of therapy that often are unsuccessful. The application of automated erythrocytopheresis (red blood cell exchange) by continuous-flow and semicontinuous-flow procedures appears to offer distinct advantages in the treatment of complications resulting from sickle hemoglobinopathies. The successful application of erythrocytopheresis for the relief of priapism in a patient with hemoglobin SC disease is presented and probably represents the first case reported on the use of automated red blood cell exchange procedures in the treatment of this condition. Data and results of automated erythrocytopheresis in 4 additional patients are presented. The advantages and disadvantages of erythrocytopheresis in the treatment of priapism (and other complications of sickle hemoglobinopathies) are discussed and the method is compared to other modes of therapy.

Myth: Blood transfusion is effective for sickle cell anemia—associated priapism

“…Of these, 4 were unobtainable for review. The remaining 122 papers were reviewed for relevance, resulting in a total of 14 case reports or studies meeting the above inclusion criteria [6][7][8][9][10][11][12][13][14][15][16][17][18][19] and 5 papers describing adverse sequelae. [20][21][22][23][24] These papers contained information on 42 individuals who were then stratified by treatment modality into the following categories:…”

Priapism in Sickle Cell Disease: The Case for Early Implantation of the Penile Prosthesis

“…The mainstay emergency room management of sicklecell-associated priapism incorporates analgesia, hydra tion, alkalization, oxygenation, and hypertransfusion of packed red blood cells [6], Rapid transfusion dilutes the percentage of sickle hemoglobin in circulation and sup presses the bone marrow release of additional abnormal red cells, thereby increasing oxygen delivery to the corpo ra [10], Additional conservative measures that have been reported in the literature include ice packs, hot soaks, ene mas, transrectal diathermy, estrogens, hyperbaric oxygen, radiation, anticoagulation, fibrinolytic therapy, vasodila tors, antimuscarinics, anxiolytics, spinal anesthesia with hypotension, and erythrocytophoresis [2,3,11], Partial exchange transfusion to reduce hemoglobin S to 30% or less has been recommended when ischemic priapism per sists beyond 24 h [12], Partial exchange transfusion may be associated with severe neurologic complications, possi bly due to changes in blood viscosity and cerebral blood flow or coexisting coagulation abnormalities aggravated by release of reperfusion metabolites from the corpora [9], Conservative measures are most effective in the pediatric population [2], When these measures fail, invasive management may include aspiration and saline irrigation of the corpora (with or without adrenergic drugs) and shunt procedures from the corpus cavemosum to the glans penis, corpus spongiosum or saphenous vein [13], Unfortunately, sickle cell priapism is prone to recurrence following these proce dures [2], Success with hormonal manipulation to prevent recurrent episodes of priapism in sickle cell patients using diethylstilbestrol and gonadotropin-releasing hormone analogues (leuprolide and goserelin acetates) has been reported recently [14,15]. Success with diethylstilbestrol was achieved in 9 of 11 patients, but priapism recurred in several.…”

Red cell exchange: special focus on sickle cell disease

“…Despite conflicting outcomes with RCE, erythrocytapheresis with the goal of reducing the HbS level to Ͻ30% and achieving a postexchange Hct of ϳ30% may be considered if early intervention with irrigation fails. 43 Acute multiorgan failure syndrome…”