Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso / Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

Early vs delayed insertion of penile prosthesis in patients with refractory priapism: a systematic review and meta-analysis.

INTRODUCTION: Refractory priapism, characterized by persistent and prolonged painful erections despite initial treatment maneuvers, can significantly impair erectile function secondary to ischemia-induced corporal tissue fibrosis. These patients will likely require subsequent penile prosthesis (PP) surgery to regain sexual activity, yet consensus regarding the optimal timing of implantation remains lacking. OBJECTIVES: To evaluate and compare the clinical outcomes associated with early vs delayed PP implantation in individuals with priapism-induced erectile dysfunction (ED). METHODS: We included studies that focused on refractory priapism leading to ED and its management with PP implantation. We assessed cohort study bias with a risk-of-bias tool and case series bias with the modified Newcastle-Ottawa Scale. Pooled odds ratios (ORs) were calculated by a fixed-effect model. RESULTS: We included 9 studies, comprising 4 cohort studies and 5 case series, involving a total of 278 patients. Total complications were higher in the delayed group (OR, 4.16; 95% CI, 2.77-6.26). Fibrosis was significantly more pronounced in the delayed group (OR, 118.18; 95% CI, 20.06-696.32). The odds of erosion, infections, and penile injury did not show statistically significant differences between the groups (OR, 2.52 [95% CI, 0.67-9.49], 0.89 [0.38-2.10], 1.83 [0.79-4.26], respectively). Patients' satisfaction resulted in a pooled OR of 0.15 (95% CI, 0.04-0.49) in favor of the early PP insertion group. CONCLUSION: The results from this study favor an early approach to ED (within 30 days) following ischemic priapism. However, it is important to consider patients' preferences, values, and psychological factors to make an informed decision.

Priapism and chronic myeloid leukemia in an adolescent. Rare debut presentation. A case report. / Priapismo y leucemia mieloide crónica en adolescente. Debut poco frecuente. Reporte de caso.

Priapism is a painful and persistent erection, with or without sexual stimulation. A rare cause of such abnormality is chronic myeloid leukemia. Few cases of priapism as an initial manifestation of this type of leukemia have been reported in adolescent patients. Here we describe the case of a 16-year-old patient who presented with priapism as the initial manifestation of chronic myeloid leukemia. No cavernosal aspiration was performed. A specific hematological treatment was started and, given the persistence of priapism, the patient required 2 corpora cavernosa shunt procedures; despite this treatment, there is a high probability of sequelae.

El priapismo es una erección dolorosa y persistente acompañada o no de estímulo sexual. Una causa poco frecuente de esta anormalidad es la leucemia mieloide crónica. Se han reportado pocos casos de priapismo como manifestación inicial de una leucemia de este tipo en pacientes adolescentes. A continuación, se informa el caso de un paciente de 16 años de edad que presentó priapismo como manifestación inicial de una leucemia mieloide crónica. Durante su evolución, no se realizó aspiración de los cuerpos cavernosos. Se inició tratamiento hematológico específico y, ante la persistencia del priapismo, fue necesario realizar un shunt de cuerpos cavernosos en dos ocasiones, tratamiento a pesar del cual existen altas probabilidades de secuelas.

Therapeutic Leukocytapheresis to relieve priapism due to leukostasis in CML patients: Our experience in a tertiary care centre.

Hyperleukocytosis in leukemic patients may cause tumour lysis syndrome, disseminated intravascular coagulopathy, and leukostasis, resulting in decreased tissue perfusion and increasing the risk of mortality. Since the myeloid blasts are larger than lymphoid blasts and are less deformable, complications of leukostasis are seen more frequently in myeloid leukemia. Priapism is a less common complication associated with leukostasis in leukaemia patients that should be treated as soon as possible to avoid ischemic injuries. Although chemotherapeutic drugs such as hydroxyurea and imatinib are used to treat hyperleukocytosis in CML patients, leukocytapheresis (LCP) can achieve rapid cytoreduction. Prophylactic LCP could not offer any advantage over aggressive chemotherapy, but therapeutic leukocyte depletion has a proven role in patients having symptomatic leukostasis due to high tumour burden. Three patients with ischaemic priapism were reported at our institute's emergency department, where detumescence could not be achieved by distal shunting or aspiration with phenylephrine instillation. The procedure of therapeutic LCP was performed in all three patients on an emergency basis, which resolved painful priapism by rapid cytoreduction.

Penile implants in low flow priapism.

Priapism is a persistent or prolonged erection, in the absence of sexual stimulation, that fails to subside. Prolonged ischaemic or low flow priapism is defined as a full or partial erection persisting for more than 4 h and unrelated to sexual interest or stimulation, characterised by little or no cavernous blood flow. Low flow priapism leads to progressive corporal fibrosis, which could, in turn, lead to long-lasting erectile dysfunction if left untreated. Penile prosthesis implantation is recognised as a management option in refractory and delayed low flow priapism for restoring erectile function with high patient satisfaction rates. However, the ensuing corporal fibrotic scarring poses a surgical challenge to clinicians, given the higher complication rates in this patient subset. Postoperative patient satisfaction has been closely linked to preoperative expectations and perceived loss of penile length. Therefore, thorough patient counselling concerning the risk and benefits of penile implants should be a priority for all clinicians. Moreover, there is a lack of consensus on the ideal prosthesis choice and procedural timing in refractory low flow priapism. In this review, we will examine the existing literature on penile implants in patients with priapism and discuss the options for managing complications associated with penile prosthesis surgery.

"Vitreous Hemorrhage and Long-Lasting Priapism After COVID-19 m-RNA Based Vaccine: A Case Report".

PURPOSE: To report a case of possible multi-district thromboembolic event involving the eye of a patient with several cardiovascular risk factors, following a second inoculation of SARS-CoV-2 m-RNA based vaccine. CASE-REPORT: A 60-year-old man presented with blurred vision in the left eye lasting 1 month but started within 24 hours from the 2nd dose of BNT162b2 vaccine inoculation. He also reported a long-lasting but self-limiting priapism which started about 4 h after the vaccination. Patient's medical history included: acute lymphoblastic leukemia, treated with chemotherapy and HLA-identical sibling donor transplant 18 months earlier; subsequent cytomegalovirus posterior outer retinal necrosis (PORN) resolved with antiviral treatment; type II diabetes and erectile dysfunction. Ocular examination of the affected eye revealed vitreous hemorrhage which limited the observation of details of the fundus. After a 2-week follow-up without any clinical improvement, parsplana vitrectomy (PPV) with cataract extraction was performed. Surgical aspiration of a large preretinal hemorrhage revealed intraretinal flame-shaped hemorrhages and some cotton wool spots. Further intraoperative examination and post-operative fluorescein angiography excluded the rhegmatogenaous and the neovascular origin of the intraocular bleeding. CONCLUSIONS: Due to the several predisposing factors such as diabetes, aspirin assumption, history of blood dyscrasia and infectious retinitis, the relationship between the acute intraocular bleeding and the BNT162b2 inoculation remains difficult to ascertain in this patient. However, the occurrence of lasting priapism and vitreous hemorrhage within 24 h from the vaccination is a critical event which deserves to be mentioned.

Go the Distance: Reproductive Health Care for People with Sickle Cell Disease.

This overview of reproductive and sexual health care concerns for people with sickle cell disease (SCD) addresses clinical concerns that can be complex and are inherently multidisciplinary. Clinicians must be prepared to initiate reproductive health care discussions, as these intimate concerns may not be volunteered by patients. SCD is associated with delayed onset of puberty, sickle pain during menstruation, disease-specific contraceptive considerations, high-risk pregnancy, priapism, erectile dysfunction, and offspring who inherit a hemoglobinopathy trait from affected parents. Reproductive health considerations are underrecognized, undertreated, and understudied. They need attention in primary care and specialty SCD, urology, and obstetrics and gynecology clinics.

Factors Associated With Corporoglandular Shunting for Patients With First-time Ischemic Priapism.

OBJECTIVE: To establish predictive factors of patients who failed intra-cavernosal injection therapy and ultimately required corporoglandular shunting during first-time ischemic priapism episodes. METHODS: A retrospective review was performed of all patients over the age of 18 who presented to our institution with first-time episode of ischemic priapism from 2009 to 2019. Variables assessed included: body mass index, diabetes, hypertension, race, insurance-type, hypertension, etiology, age, duration of erection prior to evaluation, total amount of phenylephrine injected, and use of corporal irrigation. A receiver operating characteristic (ROC) curve was performed utilizing duration of erection and amount of phenylephrine. RESULTS: One-hundred and forty-seven patients met inclusion criteria of which 24 patients required surgical intervention. There were differences associated with mean total phenylephrine used, duration of erection between shunted patients and non-shunted patients with regards to age (P = .38) or etiology (P = .81). Multivariable analysis revealed differences between duration of erection and BMI greater than 25 kg/m2. ROC curve analyses revealed total amount of phenylephrine injected and duration of erection were acceptable and excellent predictors of need for shunt procedures with area under the curves of 0.72 and 0.90, respectively. Optimal cut-off values for each were found to be 950 mcg and 15.5 hours. CONCLUSION: Our study suggests that patients who require greater than 950 mcg of total phenylephrine or present with erections lasting greater than 15.5 hours are significantly more likely to require corporoglandular shunting and should be counseled appropriately as such.

Current Status for Semirigid Penile Prosthetic Devices.

PURPOSE OF REVIEW: The goal of this paper was to evaluate the current use of semirigid penile prosthesis (SRPP), surgical techniques for insertion of SRPP, and how to prevent and approach surgical complications. RECENT FINDINGS: SRPP is a valid option for those who are refractory to medical therapy for erectile dysfunction (ED) and even more appropriate for specific subsets of patient populations. It is important for urologists to know which patient population SRPP is preferred for. Several studies have shown good patient outcomes and patient satisfaction with those who underwent SRPP.

Case Report: Priapism as The Clinical Presenting Feature of Chronic Myeloid Leukemia: Case Report and 20-Year Literature Review.

Priapism in chronic myeloid leukemia (CML) appears to be an infrequent manifestation as well as a crucial emergency. Here, we report an 18-year-old male presenting with a persistent erection of penis for 20 days. We evaluate and compare the reported cases during the past 20 years discussing the management of CML patients experiencing priapism. Cytoreductive therapy followed by leukapheresis, the administration of tyrosine kinase inhibitor, and intra-cavernosal blood aspiration may resolve the symptoms of priapism. Early intervention for cytoreduction and aspiration are the pivotal keys to successfully impeding the complications.

Stuttering Priapism in a Teenage Boy: Lesson to be Learnt.

Untreated priapism can lead to ischemic damage of the penis and impotence. This case report describes a 14-year-old boy who presented with a history of priapism for 2 months, which was undiagnosed, ridiculed, and ignored even by medical practitioners. The underlying etiology was later identified to be chronic myeloid leukemia. Despite the usage of multimodal treatment, it took 7 days for control of priapism. The young boy is now left with an erectile dysfunction. The case highlights that priapism in children is a medical emergency needing aggressive evaluation and treatment.

Penile prosthesis implant in the special populations: diabetics, neurogenic conditions, fibrotic cases, concurrent urinary continence surgery, and salvage implants.

Penile prosthesis implant (PPI) remains an effective and safe treatment option for men with erectile dysfunction (ED). However, PPI surgery can be associated with a higher risk of complications in certain populations. This article provides a critical review of relevant publications pertaining to PPI in men with diabetes, significant corporal fibrosis, spinal cord injury, concurrent continence surgery, and complex salvage cases. The discussion of each category of special populations includes a brief review of the surgical challenges and a practical action-based set of recommendations. While specific patient populations posed considerable challenges in PPI surgery, strict pre- and postoperative management coupled with safe surgical practice is a prerequisite to achieving excellent clinical outcomes and high patient satisfaction rate.

[Ischemic Priapism Associated with Essential Thrombocythemia : A Case Report].

A 71-year-old man visited a local urologist with the complaint of continuous painful erection for three days. Penile cavernosal blood data showed acidosis and hypoxia. Thus he was diagnosed with ischemic priapism. After penile aspiration and injection of phenylephrine, erection was temporary improved. The blood platelet count was 94.5×104/µl and myeloproliferative disease was suspected. He was referred to our hospital and visited us the following day. Since priapism relapsed, we aspirated corpus cavernosum of the penis and injected phenylephrine which was successful. A bone marrow biopsy and genetic test were performed at the department of hematology and the diagnosis of essential thrombocythemia with JAK2 gene mutation was confirmed. By treatment with hydroxyurea, the blood platelet count decreased without priapism recurrence.

Current practice in the management of ischemic priapism: an anonymous survey of ISSM members.

Penile shunting is the standard of care in management of ischemic priapism refractory (IPR) to non-surgical interventions. Due to high rates of impotence, corporal fibrosis, and loss of penile length, recent literature suggests these patients benefit from immediate penile prosthesis (PP) placement. An IRB-exempt anonymous electronic survey of the 2168 members of the International Society for Sexual Medicine (ISSM) was conducted. The survey included demographic information, confidence, and experience-related management of IPR. The aim was to evaluate current practice patterns in management of IPR and to investigate the role of immediate PP implantation in the management of prolonged (>36 h) IPR. The survey response rate was 11.6% (n = 251). Most respondents were urologists (173), from the USA (49.1%), and had completed a fellowship in male sexual medicine, men's health, reconstruction, or andrology (71.1%). The majority (91.3%) see at least one case of prolonged priapism (>36 h) that requires surgical management yearly. When looking at volume in training and after, our respondents had a significantly higher experience with penile prostheses (over 70%, > = 10) as compared with shunts (less than 40%, > = 10). Overall, 70.9% of respondents felt more comfortable with a malleable PP than a shunt. However, penile shunts are still preferred as the first line of surgical management by ~80% of respondents as compared with 12.7% who instead prefer a PP. We also found that under 40% of respondents currently use penile MRI or corporal biopsies in their management of prolonged assessment. This is the first study to assess current clinical practices in management of IPR globally. As in any anonymous self-reported survey-based research, recall and sampling bias are an inherent limitation. Penile shunting for IPR continues to be the preferred treatment despite emerging data. Respondents performed PP surgery routinely and feel more confident placing PP than performing penile shunting procedures.

Penile gangrene: an unusual complication of malignant priapism in a patient with renal cell carcinoma.

A 68-year-old man presented with priapism and penile gangrene. The patient had no history of penis trauma or medications for erectile dysfunction. Corpus cavernosa aspiration cytology were positive for malignant cells. Total penectomy was performed. Enhanced chest and abdominal computed tomography showed a left renal tumor with pulmonary and hepatic metastases. Ultrasound-guided renal biopsy showed clear cell renal cell carcinoma.

Priapismo en la drepanocitosis. Diagnóstico y opciones terapéuticas / Priapism in sickle cell disease. Diagnosis and therapeutics options

El priapismo es una complicación de la anemia drepanocítica y se define como una erección prolongada, dolorosa y persistente del pene de más de 4 horas de duración sin estimulación sexual asociada. El 95 por ciento de las crisis de priapismo en estos pacientes es de tipo isquémico o de bajo flujo y constituyen una emergencia médica que, de no diagnosticarse y tratarse adecuadamente, provoca necrosis del tejido y disfunción eréctil. En este trabajo se revisan el diagnóstico y las opciones terapéuticas actuales y futuras de esta grave complicación(AU)

Priapism is a common complication of sickle cell disease and it is characterized by a prolonged, painful and persistent erection of the penis lasting more than 4 hours without associated sexual stimulation. The 95 percent of priapism crisis in these patients is ischemic type and represents a medical emergency that can provoke erectile tissue necrosis and erectile dysfunction if not treated properly. In this paper we reviewed the diagnosisand the current and perspectives therapeutic options of this severe complication(AU)

The management of the acute ischemic priapism: A state of the art review. / El manejo del priapismo isquémico agudo: una revisión actualizada.

OBJECTIVE: To review the current literature on early penile prosthesis implantation in patients with refractory ischemic priapism (IP). ACQUISITION OF EVIDENCE: A systematic search for the terms "penile prosthesis", "priapism", "impotence", "fibrosis", "downsized prosthesis cylinders", and "patient satisfaction" has been carried out in PubMed, EMBASE, Cochrane, SCOPUS and Science Citation Index databases. SYNTHESIS OF EVIDENCE: Cavernosal tissue damage in IP is time related. Conservative measures and aspiration with or without intracorporeal instillation of α-adrenergic agonists are usually successful in the early stages. Shunt surgery in patients remains debatable, as the lack of response to aspiration and instillation of α-adrenergic agonists indicates that irreversible changes in the cavernosal smooth muscle are likely to have already occurred. Immediate penile prosthesis implantation in patients with refractory IP settles the priapic episode, maintains the long term rigidity necessary to engage in penetrative sexual intercourse and prevents the otherwise inevitable penile shortening. Although complication rates after penile prosthesis implantation in acute priapism are higher than in virgin cases, they are still lower than after implantation in patients with severe corporal fibrosis due to chronic priapism. Regardless of the complication rates, penile prosthesis implantation in refractory IP should be preferred as it allows the preservation of penile length, which is one of the main factors influencing postoperative patient's satisfaction following surgery.