Safety and efficacy of blood exchange transfusion for priapism complicating sickle cell disease

Abstract

Background: Priapism is unwanted painful penile erection that affects about 36% of boys and men with sickle cell disease (SCD) most of whom have sickle cell anemia. Clinically, priapism could be stuttering, minor, or major. The first two types are mild, last < 4 h, are usually treated at home, have good prognosis with normal sexual function. The major type of priapism lasts >4 h, associated with severe pain, requires hospitalization; often does not respond to medical treatment and may require shunt surgery. Untreated major priapism and surgical intervention often cause impotence. In this study, we report our 15‐year experience in treating adult patients with SCD and major priapism with blood exchange transfusion after being refractory to other medical therapies. Methods: Adult male African Americans patients with SCD and major priapism were enrolled in this study and followed for 15 years. A Haemonitics V‐50 machine was initially used for whole blood exchange and was later replaced with Cobe Spectra machine for RBC exchange. Results: We used 239 blood exchanges requiring 1,136 RBC units. We maintained a post‐exchange hemoglobin level of about 10 g/dL and hemoglobin S level < 30%. None of the patients had any neurological complications such as headache, seizures, neurological deficits, or obtundation post‐exchange. Conclusion: Together, the data indicate that blood exchange transfusion for the treatment of patients with SCD and major priapism is efficacious and safe. J. Clin. Apheresis 31:5–10, 2016. © 2015 Wiley Periodicals, Inc.

Indwelling ports for prophylactic RBC exchanges in sickle cell patients: Comparison of bard and vortex ports

“…Recurrence rates of 46‐90% are especially concerning. While less commonly performed, other possible indications for scheduled RCE in SCD which appear efficacious include recurrent, severe episodes of acute chest syndrome, vaso‐occlusive pain crises, and priapism . Most patients receiving prophylactic RBC exchanges are maintained on an approximate schedule of exchange every 3‐5 weeks to keep their hemoglobin S (HbS) level below 30%.…”

Sickle cell disease: Classification of clinical complications and approaches to preventive and therapeutic management

“…Thus, the neurologic complications were most likely due to transfusion-induced hyperviscosity. A larger study of blood exchange transfusion in patients with priapism, which maintained the post-exchange Hb level similar to baseline values, showed no neurologic complication in any of the patients [129]. …”

Efficacy and Safety of Manual Partial Red Cell Exchange in the Management of Severe Complications of Sickle Cell Disease in a Developing Country

“…In a literature review conducted by Kato [16], he found that RCE and other drug treatments were not efficient in this indication and delayed the urologic management. However Ballas and Lyon had obtained clinic regression by cytapheresis by maintaining an HbS rate lower than 30% [17]. …”