Stuttering Priapism in a Patient with Sickle Cell Trait Treated with Automated Red Cell Exchange Transfusion

Sickle cell trait (SCT) is typically an asymptomatic carrier state, but sickling complications can occur under extreme conditions. Priapism is known to be associated with sickle cell disease (SCD); The link with SCT is less well established. We report the case of a 19-year old man with SCT presenting with prolonged priapism and a refractory, stuttering course requiring multiple invasive procedures over a five day period, with no clear alternative triggers. In light of ongoing, stuttering priapism, he underwent RBC exchange transfusion with decrease of hemoglobin S (HbS) from 45.8% to 11.7%. This was followed by immediate and sustained cessation of stuttering priapism, with no further episodes at five months. Multiple cases of priapism associated with SCT have been reported in the literature. In most cases invasive interventions were required but RBC exchange was not attempted. RBC exchange has been reported in two people with exertional rhabdomyolysis in the context of SCT, with improvement in one case. In patients with SCT and priapism, conservative measures are used to treat brief episodes, but invasive management is required for persistent or prolonged episodes. RBC exchange transfusion may be considered for treatment of refractory, stuttering priapism in individuals with SCT.

Differences in polysomnographic, nocturnal penile tumescence and penile doppler ultrasound findings in men with stuttering priapism and sleep-related painful erections

Men with Stuttering Priapism (SP) and sleep-related painful erections (SRPE) experience bothersome nocturnal painful erections resulting in poor sleep. The aim of this study is to observe common features and differences between men with SP and SRPE based on polysomnography, nocturnal penile tumescence (NPT), and penile doppler ultrasound (PDU). This is a prospective cohort study of 20 participants divided into two groups (Group 1 = SP [n = 12]; Group 2 = SRPE [n = 8]) with bothersome painful nocturnal erections. All participants were referred to the sleep disorder clinic to be assessed and consented for overnight polysomnography with simultaneous NPT recording and to complete validated sleep, sexual dysfunction and health-related quality of life questionnaires. Unstimulated PDU was also performed. Abnormal Polysomnographic findings (reduced sleep efficiency, total sleep time, and awake after sleep onset) were identified in both groups suggesting poor sleep. Men with SP had significantly longer erections (60.0 vs 18.5; p = 0.002) and took longer to detumesce once awake (25.7 vs 5.4 min; p = 0.001) than men with SRPE. They also had significantly higher peak systolic and end diastolic velocities on unstimulated PDU with an abnormal low resistance waveform identified. No sleep pathology was identified in men with SP. This implies a local (penile) etiology in men with SP. Men with SRPE had a normal resting PDU and abnormal sleep architecture with REM awakenings and significantly more Periodic limb movements (p = 0.04) than men with SP suggesting a central (sleep-related) cause in men with SRPE. Sexual dysfunction and poor HR-QoL was identified on validated questionnaires in both groups. SP and SRPE are rare entities that share similar symptoms (painful nocturnal erections and poor sleep) but dissimilar features of nocturnal erection onset, duration and resolution with different polysomnographic features which may allude to a different pathophysiology.

Automated Red Cell Exchange in the Management of Sickle Cell Disease

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.

Priapism with Essential Thrombocythemia Review of Literature, Where We Stand and Where We Are Heading

Introduction Priapism is a persistent erection of the penis that is not associated with sexual stimulation or desire lasting more than 4 hours. Priapism can be classified as ischemic, non-ischemic, or stuttering. Stuttering priapism is defined as recurrent priapism lasting less than 4 hours. Commonly, no cause is identified, secondary causes include medications specially intracorporal injections or medical conditions such as hematological diseases particularly sickle cell disease, but it is uncommon to be seen with essential thrombocythemia (ET). It adversely affects the quality of life, sexual function, and physical wellness of the affected patients besides the risk of penile fibrosis and permanent erectile dysfunction. The underlying pathophysiology is not clearly understood but the mechanism involves disturbed nitrous oxide, phosphodiesterase enzyme activity, and disturbed auto-regulation of the penile circulation. Method Literature searched in google scholar, PubMed, and Scopus search engines with keywords priapism, essential thrombocythemia, essential thrombocytosis results found from 1960 to 2020. Results Using the keywords above we found 11 cases of ET devolved or presented with priapism. With the case in our center (not published) the total number of cases will be 12. The cases arranged by chronological order. Almost all age groups are involved from 7 years to 71 years. The table shows hematological parameters, type of mutation, and the management of priapism. Discussion In ET the mechanism of priapism is probably related to high platelet count and platelets sludging in the corpora cavernosa, similar to RBC sludging in sickle cell disease and WBC sludging in leukemia. Subsequent stasis and disturbed NO and cGMP signaling. This might be precipitated by increased viscosity or release of free hemoglobin secondary to hemolysis similar to sickle cell anemia as many patients had low hemoglobin levels (table-1). Looking at the table with the available data most hemoglobin levels are in the lower normal limits, but no patient had high hemoglobin levels, opposite to what can be expected that polycythemia might enhance the viscosity of the blood and enhance thrombosis. Observing other MPN who developed priapism, in one study 430 patients with CML 8 patients developed priapism at diagnosis, they had lower hemoglobin levels compared to CML patients who didn't develop priapism. The mean hemoglobin level in patients with priapism was 8.4 ± 2.3 g/dL, compared to 10.8 ± 2.5 g/dL in CML patients control who didn't develop priapism. To the best of our knowledge, there are no reports of cases of adults with polycythemia Vera who devolved priapism which might indicate that anemia has a synergistic effect with leukocytosis in the pathogenesis of priapism in CML patients. But, it is difficult to generalize this observation to ET. However, anemia can enhance thrombosis and the risk of thromboembolism by different mechanisms not related to reactive thrombocytosis. Some patients presented after years from the onset of priapism. This may indicate the complaint of priapism is underreported especially for patients with stuttering priapism who develop erection for a shorter duration of time and subsides spontaneously. While patients with ischemic priapism have prolonged, painful erection usually seeks medical attention from the first time. This can be the reason that CML reports of priapism are relatively more common 1.9 % compared to ET because CML type is predominantly ischemic. At the same time, ET can be ischemic or stuttering. Most of these cases are reported before 2008 WHO criteria for ET, which explains why no genetic studies done for the cases. This case is the first reported case of CLAR mutation associated with priapism. In the table 6/12 had splenomegaly or splenectomy a ratio close to general ET patients. Conclusion Priapism is a rare presentation and complication of ET. It can present as ischemic or stuttering types. ET with stuttering priapism might be underreported. The main risk factors aren't clear due to old and few reported cases. Splenomegaly, age, and type of mutation appear to have little impact. But the characteristic features are the high platelet count and lower hemoglobin level. Disclosures No relevant conflicts of interest to declare.

Stuttering priapism post C-spine schwannoma excision: case report

Priapism is prolonged penile erection that persists beyond or is unrelated to sexual stimulation and this is associated with significant psychological, socioeconomic and physical morbidity. It is a urologic emergency as delayed intervention may result in penile fibrosis and consequent erectile dysfunction. The aim of this paper is to present our experience in the management of the rare occurrence of stuttering priapism in a 32 years old man following surgical excision of cervical schwannoma during the early postoperative period and review relevant literature on management of this urologic entity. Stuttering priapism may complicate cervical spine tumor excision but may demonstrate complete patient recovery.