Clinical and laboratory parameters, risk factors predisposing to the development of priapism in sickle cell patients

Abstract

Although sickle cell disease is very common in Oman, priapism is a relatively rare complication of this disease. This study was aimed to identify the clinical and laboratory risk factors that predispose sickle cell disease patients to priapism. In a retrospective, case-control study, data on 21 male sickle cell disease patients, with priapism, were compared to 20 age and sex-matched sickle cell disease patients without priapism from the hospital medical records. Specifically, the demographical, hematological, biochemical, and clinical parameters including complications attributable to priapism were studied. Means for continuous measures and independent t-test were used to evaluate the association between the parameter studied and the occurrence of priapism. Priapism occurrence was associated with low basal hemoglobin (Hb), along with an increase in other parameters such as white blood cell count (WBC) ( P = 0.010), platelet count ( P = 0.001), reticulocyte count ( P < 0.001), mean corpuscular volume (MCV) ( P < 0.001), and mean corpuscular hemoglobin (MCH) ( P < 0.001). Biochemical laboratory parameters revealed an association with an increased total bilirubin ( P < 0.001). Patients with priapism were more likely to have acute chest syndrome, stroke, pulmonary hypertension, asplenia ( P = 0.006), gallbladder stones, and consequently cholecystectomy. Blood exchange and Penile aspiration were the most frequent medical therapies ( P = 0.010 and P < 0.001 respectively). Patients with sickle cell disease who presented with priapism were young adults with low Hb, high WBC’s, platelets, reticulocytes, MCV, MCH, bilirubin, and LDH. These findings suggest an association of priapism with increased hemolysis. Impact statement Sickle cell disease is prevalent throughout the world with a 5.8% incidence of sickle cell trait among the Omani population, yet priapism was a relatively rare complication. Penile erection and detumescence are complex physiological processes, which require a delicate neurohormonal and cardiovascular response. In this cohort, young SCD patients developed priapism with a low incidence of complications. These patients had high WBCs, platelets, bilirubin, LDH, and reticulocytes with low hemoglobin as compared to controls, suggesting an increased hemolytic process. Favorable outcome was dependent on early intervention, with almost half of these patients needing surgical intervention.

Iron Overload in Patients With Heavily Transfused Sickle Cell Disease—Correlation of Serum Ferritin With Cardiac T2* MRI (CMRTools), Liver T2* MRI, and R2-MRI (Ferriscan®)

“…A yearly increase as the result of newborn cases is about 120-150 (3). Thus, SCD is a major public health problem in the Sultanate of Oman, with a high rate of morbidity and mortality (4)(5)(6)(7)(8)(9)(10)(11)(12).…”

Platelet volume parameters as a tool in the evaluation of acute ischemic priapism in patients with sickle cell anemia

“…These conflicting studies could be related to differences in various factors such as study design, sickle cell genotype, precipitating factors, genetic factors, effects of intervention, and laboratory methods, all of which can change the hematological values of priapic patients with SCA (8,16,27). In general, the prognostic value of a complete blood count includes other biomarkers (eosinophil, and reticulocyte count), has been associated with the development of acute IP in men with SCA (8,11,13). Furthermore, in this report, patients with priapism > 17.9 hours had a positive correlation for predicting erectile tissue recovery.…”

“…Because of the vasculopathy, chronic hemolysis, and veno-occlusive pathogenesis of SCA, multiple hematologic markers are needed to predict the outcome of IP in SCA patients. The role of platelet count (PLT) and mean platelet volume (MPV) in these veno-occlusive mechanisms is well documented (7)(8)(9)(10)(11). Previous studies confirmed the association between IP, defined as a vasculogenic disease, and platelet activation leading to higher MPV (9,10).…”

Neutrophils in sickle cell disease: Exploring their potential role as a therapeutic target

“…[5][6][7][8][9][10] More recently, also retinopathy, priapism, and an increased risk of venous thromboembolism (VTE) were shown to be associated with increased neutrophil counts. [11][12][13][14] Lydian A. de Ligt and Aafke E. Gaartman contributed equally.…”

“…Neutrophil counts positively correlated with SCD‐related complications such as stroke, acute chest syndrome, and early death 5–10 . More recently, also retinopathy, priapism, and an increased risk of venous thromboembolism (VTE) were shown to be associated with increased neutrophil counts 11–14 …”