Acquired hemophilia A (AHA) is an acquired bleeding disorder caused by neutralizing antibodies (inhibitors) against Coagulation Factor VIII (FVIII:C), causing sudden hemorrhagic symptoms (i.e., subcutaneous bleeding, intramuscular bleeding, and hematuria). Herein, this study is aimed at presenting a case of AHA diagnosed based on hematuria and reviewing patients who were diagnosed with AHA due to hematuria. A 67-year-old woman was referred to Atsugi City Hospital with painless gross hematuria that began 4 weeks before presentation. Contrast-enhanced computed tomography (eCT) revealed an approximately 2 cm mass in the right renal pelvis, and the patient\'s activated partial thromboplastin time (APTT) was elevated (61.4 s). The day after the endoscopic biopsy, the patient was in shock due to a large retroperitoneal hematoma. Although her condition stabilized after intravenous radioembolization, she underwent emergency surgeries several times because of rebleeding within the next 3 weeks. At that time, APTT was more prolonged at 106.4 s, and the FVIII:C level was 2%. Mixing tests showed an upwardly convex curve after 2-h incubation, indicating the presence of an inhibitor. Factor VIII inhibitor titer was ≥5.1 Bethesda unit (BU)/mL. A combined product of Plasma-Derived Factors VIIa and X (pd-FVIIa/FX), as second-line hemostatic therapy, as well as cyclophosphamide (CYP), were administered after Recombinant Activated Factor VIIa (rFVIIa) had been ineffective. Following this, the Factor VIII inhibitor titer was undetectable, FVIII:C levels were restored, and APTT decreased to within the normal range. Gross hematuria was significantly alleviated. However, the patient died of cytomegalovirus and fungal infections due to prolonged immunosuppressive therapy. Although AHA diagnosed based on hematuria may have a better prognosis than others, there have been occasional cases with severe outcomes. APTT, detected upon initial hematological testing in patients with hematuria, may be a potential indicator of an existing AHA.

Emphysematous cystitis is a rare type of urinary tract infection that is characterized by the accumulation of gas within the walls and lumen of the urinary bladder. In rarer instances, pneumoperitoneum may accompany emphysematous cystitis. When pneumoperitoneum is suspected through imaging studies in patients with emphysematous cystitis, surgical abdominal exploration is frequently performed considering the possibility of bladder perforation or coexistence of intestinal perforation. We successfully managed a case of emphysematous cystitis accompanied with pneumoperitoneum conservatively. A 90-year-old woman hospitalized with a gastric ulcer developed abrupt lower abdominal pain and hematuria. Contrast-enhanced CT revealed gas within the bladder wall, which was consistent with emphysematous cystitis, and pneumoperitoneum. No obvious bowel or bladder perforation was observed in the CT scan. Regarding her high surgical risk and clinical stability, surgical abdominal exploration was not performed, and she was managed conservatively with urethral catheter placement and antibiotics. She recovered with the treatment, and CT imaging obtained on day 18 demonstrated resolution of the bladder wall emphysema and no signs of pneumoperitoneum. We performed a literature review using MEDLINE and Japana Centra Revuo Medicina Web and confirmed 13 previously reported cases of emphysematous cystitis and pneumoperitoneum. Based on the review of these 13 cases and our case, it is difficult to predict the presence of bladder perforation solely based on peritoneal signs in physical findings or ascites on CT scans. Therefore, it would be preferable to perform surgical abdominal exploration to make a definite diagnosis and select an appropriate treatment. However, the fact that at least eight out of the 10 cases managed conservatively survived suggests that there is a specific clinical entity among patients who present with emphysematous cystitis and pneumoperitoneum that can be safely managed conservatively. Further accumulation of cases and research is necessary to determine which cases can be treated conservatively.

Urinary bladder hamartoma is a rare benign proliferation with only 14 cases reported in the literature at present. Urinary bladder hamartoma is composed of a disorderly admixture of normal urinary bladder components, essentially represented by glands lined by transitional epithelium and a variable percentage of fibrous stroma, smooth muscle bundles, and adipose tissue. Urinary bladder hamartomas do not exhibit cytological or architectural abnormalities and show no necrosis or increase in mitotic activity. Clinical manifestations are usually represented by lower urinary tract symptoms, more or less frequently paired with gross hematuria. Several pediatric cases of urinary bladder hamartoma have been reported, sometimes with syndromic associations. Transurethral resection has been curative in all cases reported, with no evidence of recurrence. Here we report an additional rare urinary bladder hamartoma, clinically mimicking urothelial carcinoma, providing a review of the literature regarding this unusual entity.

BACKGROUND: Paraganglioma of genitourinary tract is uncommon, and origin from ureter is even rarer. We aim to present a case of paraganglioma from ureter in a 48-year-old female patient, who presented with gross hematuria.
METHODS: We present a 48-year-old female who complained of gross hematuria for one week. A left ureteral tumor was found by image study. However, hypertension was unexpectedly recorded during diagnostic ureteroscopy survey. Due to persisted gross hematuria and bladder tamponade, she underwent left nephroureterectomy with bladder cuff resection. Blood pressure surged again when the tumor was surgically approached. Ureteral paraganglioma was confirmed according to pathological report. After the surgery, the patient recovered well, and no more gross hematuria was noted. She is now under regular follow-up at our outpatient clinic.
CONCLUSIONS: Ureteral paraganglioma should be kept in mind not only when blood pressure fluctuates during operation, but also before we manipulate the ureteral tumor when gross hematuria is the only sign. Whenever the presumption of paraganglioma is raised, laboratory evaluation and anatomical or even functional imaging should be considered. The concomitant anesthesia consultation before the surgery should not be deferred, either.

Intrauterine device (IUD) is the second most widely used method of contraception worldwide. Up to 14% women prefer IUD for its attractive advantages such as cost effectiveness, high efficiency, and low complication rate. Despite these advantages, however, some complications may occur. One of these complications is uterine perforation and migration of the device to involve adjacent viscera such as peritoneum, bowel, vessels, and rarely bladder. IUD migration into the urinary bladder is uncommon, and only 70 cases are reported in the literature. Recurrent urinary tract infection and bladder calculi are the commonest presentations, and, rarely, women can present with gross hematuria. A high index of suspicion is needed in the evaluation of women who report pregnancy after IUD insertion as it might be the first clue to suspect migration. A forgotten and long-standing IUD increases the risk of uterine perforation and migration. A routine abdominal radiography, cystoscopy, and transvaginal ultrasonography are diagnostic. A computed tomography can also be employed in selected cases to delineate anatomic relations. Urologists should consider a vesical foreign body such as migrated IUD in women with recurrent lower urinary infections. Gross hematuria in a young woman should alert the urologist, and the evaluation should address a detailed contraceptive history. Every migrated IUD should be removed via endoscopy, laparoscopy, or open surgery. Proper follow-up and education of women before and after IUD insertion is also recommended to pick up on complications in time. Here, we report the successful open surgical treatment of a woman who had a forgotten IUD for 15 years and ultimately presented with gross hematuria due to trans-vesical migration. As to our literature search, there was no similar case reported from a urology center from Ethiopia.

OBJECTIVE: To describe the presenting signs and symptoms of patients with urinary tract endometriosis (UTE), appropriate workup, and to review medical and surgical therapies for symptom palliation and definitive management.
RESULTS: UTE is a condition that clinicians should maintain a high index of suspicion for, as symptoms can be easily misdiagnosed from other causes. Surgical resection of implants appears to offer safe and durable symptom relief. Urinary tract endometriosis may present with symptoms overlapping with interstitial cystitis, nephrolithiasis, bladder overactivity, or recurrent urinary tract infections, and may or may not be cyclical in nature. Cyclical gross hematuria is considered pathognomonic, though final diagnosis must be made after a pathologic review. Without proper diagnosis and treatment, consequences such as silent renal loss from asymptomatic obstruction may result. After the diagnosis is made, initial therapy can be undertaken with hormonal treatment to palliate symptoms (most commonly in the form of combined oral contraceptives), followed by surgical resection for a definitive treatment option.

UNASSIGNED: Aberrant cortical adrenal tissues are not generally identified in adults. Herein, we present a very rare case of an ectopic adrenal tumor located in the renal hilum that caused gross hematuria.
UNASSIGNED: A 33-year-old man suddenly presented with asymptomatic gross hematuria. Abdominal computed tomography revealed a 35-mm mass in the left renal hilum encroaching the renal vein. Following the surgical removal with frozen section of the mass, his gross hematuria immediately improved. Pathological analysis of the specimen revealed the features adrenal adenoma. Immunohistochemical staining for key steroidogenic enzymes confirmed the adrenocortical origin without excessive hormone production.
UNASSIGNED: This is the first case of an ectopic adrenocortical adenoma in the renal hilum that caused gross hematuria without hormonal symptoms.

Propafenone hydrochloride has been widely used for the treatment of supraventricular and ventricular arrhythmias. We present a case report of an 81-year-old patient with propafenone-associated gross hematuria.
The Naranjo Adverse Drug Reaction Probability Scale was used to assess causality. We also performed a literature search to find publications that report propafenone-associated gross hematuria.
The Naranjo scale generated a score of 6, suggesting a probable association between gross hematuria and propafenone therapy. Thirteen publications that reported an association between gross hematuria and propafenone administration were found.
A probable association exists between gross hematuria and propafenone.