Myelodysplastic syndrome occurring after enfortumab vedotin treatment for metastatic urothelial carcinoma.

Introduction: Enfortumab vedotin is an antibody-drug conjugate targeting Nectin-4 for the treatment of advanced urothelial carcinoma in patients previously treated with platinum-containing chemotherapy and immune checkpoint inhibitors. Common adverse events include rashes, peripheral neuropathy, and hyperglycemia. However, there are no reports on the development of myelodysplastic syndrome during enfortumab vedotin therapy in clinical settings. Case presentation: A 72-year-old male patient experienced prolonged and severe thrombocytopenia 18 weeks after the start of enfortumab vedotin therapy for metastatic urothelial carcinoma, requiring daily platelet transfusions. Bone marrow examination and chromosomal analysis confirmed the diagnosis of myelodysplastic syndrome. Treatment with eltrombopag proved to be effective. Conclusion: This is the first report of the development of myelodysplastic syndrome during enfortumab vedotin therapy in a clinical setting. Although rare, myelodysplastic syndrome can occur during enfortumab vedotin therapy.

Utility of combining multiple parameters of 123I-IMP SPECT and voxel-based morphometry MRI using a multiparametric scoring system for differentiating dementia with Lewy bodies from Alzheimer's disease.

BACKGROUND: Brain magnetic resonance imaging voxel-based morphometry (VBM) and perfusion single-photon emission computed tomography (SPECT) are useful for differentiating dementia with Lewy bodies (DLB) from Alzheimer's disease (AD). PURPOSE: To determine whether combining multiple parameters of VBM and SPECT using a multiparametric scoring system (MSS) improves diagnostic accuracy in differentiating DLB from AD. MATERIAL AND METHODS: In total, 23 patients with DLB and 57 patients with AD underwent imaging using a voxel-based specific regional analysis system for AD (VSRAD), an easy Z-score imaging system, and a Z-Graph using three-dimensional stereotactic surface projection. The cutoff values were determined using the receiver operating characteristic curve to differentiate DLB from AD for all parameters. Patients were scored 1 (DLB) or 0 (AD) for each statistically significant parameter, according to a threshold. The total score was determined for each case to obtain a cutoff value for the MSS. RESULTS: The mean Z-scores in the medial temporal lobes using the VSRAD were significantly lower in patients with DLB than in those with AD. Each Z-score of the summed Z-scores in all four segmented regions of the occipital lobes using the Z-Graph was significantly higher in patients with DLB than in those with AD. Among the five parameters, the highest accuracy was 80% for the Z-score of the summed Z-scores in the left medial occipital lobe. For the MSS, a cutoff value of four improved the diagnostic accuracy to 82%. CONCLUSION: MSS was more accurate than any single parameter of VBM or SPECT in differentiating DLB from AD.

Lessons from a case with stiff left atrial syndrome complicated by iatrogenic inter-atrial shunts: a case report.

Background: In patients with stiff left atrial (LA) syndrome, reservoir function is significantly impaired due to extensive LA fibrosis; consequently, the increase in LA pressure during haemodynamic stress is prominent, easily leading to pulmonary venous hypertension and subsequent pulmonary congestion, and eventually results in intractable heart failure. Case summary: A 79-year-old female with mitral stenosis and atrial fibrillation underwent valve replacement, Cox-Maze IV procedure, LA plication, and appendage ligation 4 years prior to presentation. Thereafter, she underwent a total of two catheter ablation procedures for recurrent atrial tachycardia. Transthoracic echocardiography revealed two continuous colour jets across the interatrial septum, with a peak pressure gradient of 23 mmHg, which was consistent with the residual puncture hole at the catheter ablation procedures. Although transoesophageal echocardiography showed no evidence of prosthetic valve dysfunction, the pulmonary venous flow signal showed a significantly blunted systolic forward flow, extremely small retrograde reversal flow during atrial contraction, and prominent diastolic flow velocities, all of which indicated significantly impaired LA function. Cardiac catheter examination revealed a characteristic pulmonary capillary wedge pressure waveform, which consisted of a steep ascending limb of v wave with a large peak, consistent with stiff LA syndrome. Discussion: Treatment of patients with stiff LA syndrome is quite challenging and restricted to the use of diuretics only, which has limited efficacy and eventually results in intractable heart failure. In this case, owing to the inter-atrial pressure-relieving gateway, the patient was only mildly symptomatic despite the existence of a non-compliant LA.

Where has the lymphoma gone? Pericardial effusion adenosine deaminase may play a key role in diagnosing primary effusion lymphoma-like lymphoma: a case report.

Background: Primary effusion lymphoma (PEL) is a non-Hodgkin lymphoma that is exclusively generated by body cavity effusion. Primary effusion lymphoma develops in patients infected with human immunodeficiency virus (HIV) and is associated with the human herpes virus (HHV)-8 infection. However, there are sporadic cases without HHV-8 infections or any history of immunodeficiency, called 'PEL-like lymphoma'. Case summary: An 83-year-old man was admitted to our institution because of shortness of breath, fatigue, and facial oedema. Laboratory findings were unremarkable, including negative results for HIV antibodies. Transthoracic echocardiography revealed massive pericardial effusion surrounding the entire heart, which resulted in the early diastolic collapse of the right ventricular free wall, indicating elevated intra-pericardial pressure. He underwent pericardial centesis and 700 mL of pericardial fluid was drained. Adenosine deaminase (ADA) in the pericardial effusion showed an abnormally high value of 221 U/L. Cytological examination revealed a cellular population compatible with diffuse large B-cell lymphoma with prominent blastic characteristics and negative for HHV-8 latent nuclear antigens. Thus, the patient was diagnosed with HHV-8 unrelated HIV-negative PEL-like lymphoma. He was followed for more than 10 months in complete remission after a single pericardial drainage without any chemotherapy. Discussion: Exhaustive drainage of the lymphomatous effusion may induce complete remission in some patients with PEL-like lymphoma. Furthermore, the ADA value in the pericardial effusion may serve as a valuable guide to facilitate the accurate diagnosis of PEL-like lymphoma.

Type 1 diabetes mellitus after cord blood transplantation from an unrelated donor with a disease-sensitive haplotype.

Allogeneic hematopoietic stem cell transplantation (allo-HSCT), a curative treatment for hematopoietic neoplasms, often causes various autoimmune disease-like conditions. In contrast, allo-HSCT-related type 1 diabetes mellitus is extremely rare. Herein, we report a case of allo-HSCT-related type 1 diabetes mellitus in a patient who had undergone cord blood transplantation (CBT) as a treatment for acute myeloid leukemia. The patient's human leukocyte antigen was replaced with the donor type after transplantation. The donor had a disease-sensitive haplotype. To the best of our knowledge, this is the first reported case of type 1 diabetes mellitus following CBT.

Diastolic heart failure is a new clinical entity of trastuzumab-induced cardiotoxicity: a case report.

Background: Cancer therapy-related cardiac dysfunction (CTRCD) is defined as a decrease in the left ventricular ejection fraction (LVEF) of >10% to a value below the lower limit of normal or relative reduction in global longitudinal strain (GLS) >15% from baseline after cancer treatment. However, the possibility of the development of isolated diastolic dysfunction has never been considered in the clinical presentation of CTRCD. Case summary: An 81-year-old woman was admitted to our institution presenting with prominent bilateral leg oedema, orthopnoea, and 8 kg of weight gain after administration of the anti-human epidermal growth factor receptor 2 (HER-2) antibody, trastuzumab, for HER-2-positive breast cancer. Transthoracic echocardiography showed a preserved LVEF of 62% without a significant reduction in GLS compared with results obtained before anti-HER-2 targeted therapy. Doppler echocardiography distinctly revealed a newly developed significant left ventricular diastolic dysfunction with evidence of elevated filling pressure. After successful achievement of volume reduction, the patient underwent cardiac catheter examination, revealing an elevated pulmonary artery wedge pressure of 18 mmHg. Subsequently, trastuzumab was discontinued and the patient was treated with diuretics, arteriodilators, and venodilators, until the signs and symptoms of heart failure completely disappeared. Discussion: In the management of CTRCD, including pretreatment screening, cardiotoxicity monitoring, follow-up after anti-cancer agents, and evaluation of the effectiveness of the therapy, too much emphasis has been paid exclusively to the development of systolic dysfunction; however, perspectives for diastolic dysfunction may be needed. A comprehensive multidisciplinary team approach composed of breast surgeons, oncologists, onco-cardiologists, and echocardiography specialists is required.

Treatment Strategy for Fatal Arrhythmias in Ebstein's Anomaly Combined With Leadless Pacemaker and S-ICD Implantations.

The management of heart rhythm disorders in patients with adult congenital heart disease and limited vascular access is challenging. We present the case of a 38-year-old woman with Ebstein's anomaly who underwent implantation of a combination of a leadless pacemaker and a subcutaneous implantable cardioverter-defibrillator to manage fatal arrhythmias. (Level of Difficulty: Intermediate.).

A Case of Esophagojejunal Variceal Rupture after Total Gastrectomy and Esophagojejunostomy Successfully Treated with Percutaneous Transhepatic Obliteration under Dual-balloon Occlusion of Feeding and Draining Veins.

We present the case of a man in his 60s with bleeding esophagojejunal varices occurring after gastrectomy for gastric carcinoma. Percutaneous transhepatic portography depicted the esophagojejunal varices originated from the jejunal vein and drained into the azygos vein. A 5-French occlusion balloon catheter was wedged into the jejunal vein and a 3-French occlusion balloon catheter into one drainage channel of the esophagojejunal varices via the azygos vein. Selective antegrade jejunal venography under dual-balloon occlusion revealed entire esophagojejunal varices with good stagnated and well-opacified contrast medium. Subsequently, 12 mL of 5% ethanolamine oleate-contrast medium mixture was slowly injected into the esophagojejunal varices. He was discharged without complications one week after the procedure, and abdominal computed tomography demonstrated the disappearance of the esophagojejunal varices six months after the procedure.

High Signal Intensity of the Cochlear Modiolus on Unenhanced T1-Weighted Images in Classical Infratentorial Superficial Siderosis.

Background: Superficial siderosis (SS) results from chronic bleeding in the subarachnoid space. SS can be classified as infratentorial SS (i-SS) and supratentorial SS (s-SS). The cochlear modiolus (CM) normally shows low signal intensity (SI) on Tl-weighted images (T1WI). We noticed persistently high SI of the CM on unenhanced thin-sliced T1WI in patients with i-SS. The purpose of this study was to evaluate the correlation between SS and high SI of the CM on unenhanced T1WI. Methods: This retrospective study analyzed three cases with i-SS, eight cases with s-SS, and 23 normal controls (NC) evaluated on unenhanced thin-sliced T1WI with a three-dimensional spoiled gradient-recalled echo sequence. CM-T1SI scores of 0, 1, and 2 indicated low, iso, and high SI, respectively. In cases with scores of 2 evaluated several times, all scores were reviewed for each case. The CM-T1SI ratio was defined as the contrast ratio between the CM and the cerebellum. Differences between the three groups were statistically analyzed based on the CM-T1SI score and ratio. Receiver operative curve (ROC) analysis was used to determine the cut-off values for differentiating the i-SS group from the NC group based on the CM-T1SI ratio. Results: Two patients with i-SS had a score of 2 on all evaluations. The CM-T1SI score and ratio differed significantly between the i-SS and NC groups. The accuracy of the CM-T1SI ratio for discriminating i-SS from NC was 98.9% at a cutoff value of 0.628. Conclusion: High SI of the CM on unenhanced TIWI can be an additional characteristic finding of i-SS.

Ability of Left Atrial Distensibility After Radiofrequency Catheter Ablation to Predict Recurrence of Atrial Fibrillation.

This study sought to assess the left atrial (LA) functional recovery after radiofrequency catheter ablation (RFCA) for atrial fibrillation (AF) and to evaluate the determining factor of procedural success of RFCA, using a novel preload stress echocardiography. A total of 111 patients with AF were prospectively recruited. The echocardiographic parameters were obtained during the leg-positive pressure (LPP) maneuver, both at baseline and midterm after RFCA. As an index of LA distensibility, the LA expansion index was calculated as (LAVmax - LAVmin) × 100 / LAVmin. During a median follow-up period of 14.2 months, AF recurrence was observed in 23 patients (20.7%). In LA functional parameters at baseline, only the Δ LA expansion index was significantly larger in the success group (16 ± 11% vs 4 ± 9%, p <0.05). At midterm follow-up, the Δ LA expansion index significantly increased to 32 ± 19% (p <0.05), together with structural LA reverse remodeling only in the success group. Moreover, the Δ stroke volume index during the LPP stress test significantly increased only in the success group (from 2.3 ± 1.3 ml/m2 to 3.1 ± 4.8 ml/m2, p <0.05). In a multivariate analysis, left ventricular ejection fraction (hazard ratio 0.911, p <0.05) and baseline Δ LA expansion index (hazard ratio 0.827, p <0.001) were independent predictors of AF recurrence. In conclusion, the baseline Δ LA expansion index during LPP stress is a reliable marker for predicting procedural success after RFCA. Moreover, maintenance of sinus rhythm resulted in an improvement of the preload reserve after RFCA.

Serum high-density lipoprotein cholesterol level has a significant prognostic impact on outcomes of follicular lymphoma patients.

We investigated the potential of nutritional and inflammatory parameters as prognostic factors for follicular lymphoma (FL), and also examined the predictive value of the early progression of disease within 24 months of first-line chemo-immunotherapy (POD24). We retrospectively analyzed 46 patients with FL admitted to Teikyo University Hospital and treated with chemo-immunotherapy between May 2009 and July 2019. Physical characteristics, blood parameters, and markers or scores for consumptive/inflammatory and nutritional conditions were used as variables. Nine parameters correlated with poor overall survival (OS) in univariate analysis: An Eastern Cooperative Oncology Group (ECOG) scale performance status (PS) ≥2, five or more involved nodal sites, positive bone marrow (BM) involvement, a serum albumin level <3.5 g/dL, CRP >0.5 mg/dL, lactate dehydrogenase (LD) higher than the upper normal limit (UNL), high-density lipoprotein cholesterol (HDL-C) <40 mg/dL, modified Glasgow prognostic score of 1-2, and the geriatric nutritional risk index <82. In multivariate analysis, ECOG PS ≥2, positive BM involvement, and a serum HDL-C level <40 mg/dL remained significant for poor progression-free survival. One-year OS rate after receiving salvage chemotherapy was lower in the POD24 group (50%) and POD24 correlated with ECOG PS ≥2, positive BM involvement, a serum lactate dehydrogenase >UNL, and HDL-C <40 mg/dL by Fisher's exact test. These results indicate that low serum HDL-C levels appear to be important for predicting the risk of POD24 and the worse prognosis of FL.

Comprehensive Palliative Care Needs in Outpatients with Chronic Heart Failure: A Japanese Cross-Sectional Study.

Background: The type and frequency of palliative care needs of chronic heart failure (CHF) patients have not been determined in Japan. Objectives: The aim of this study was to comprehensively assess the prevalence and characteristics of palliative care needs of CHF outpatients. Methods: Patients were recruited for this cross-sectional study from June 1 to August 31, 2020, at the Kobe University Hospital. An Integrated Palliative care Outcome Scale (IPOS) and an original questionnaire developed by multidisciplinary experts were answered once by patients themselves or with the assistance of their family. Results: A total of 101 patients (63 males and 38 females) were included. The most common distressing symptoms were dyspnea (29%; 95% confidence interval [CI] 21-39]), drowsiness (29%; 95% CI 21-39), poor mobility (25%; 95% CI 17-35), insomnia (25%; 95% CI 17-35), and anxiety (24%; 95% CI 17-35). Eighty percent (95% CI 70-87) of patients were willing to have an end-of-life (EOL) discussion. When we compared New York Heart Association class I/II with III/IV patients, the frequency of distressing symptoms was associated with the severity of the disease, but both groups exhibited a willingness for having an EOL discussion or knowing the future course of their diseases. Conclusions: Dyspnea, drowsiness, insomnia, and anxiety were frequent symptoms in CHF outpatients in Japan. Beyond distressing symptoms, most ambulatory heart failure patients have a need for EOL discussion, which was not associated with disease stage. Assessing comprehensive and multidimensional palliative care needs, including needs for EOL discussion, is advisable among outpatients with CHF.

Advanced magnetic resonance imaging findings of cerebellar hemangioblastomas: A report of three cases and a literature review.

On conventional magnetic resonance imaging (MRI), hemangioblastomas typically appear as mural nodules with an adjacent surrounding cyst or a solid mass in the cerebellum. However, hemangioblastomas sometimes cannot be reliably distinguished using this imaging technique from other tumors, especially pilocytic astrocytomas and metastatic tumors, because of their similar imaging findings and locations. Herein, we report three cases of cerebellar hemangioblastomas and review their findings on conventional and advanced MRI, including diffusion-weighted imaging (DWI), dynamic susceptibility-weighted contrast-enhanced perfusion-weighted imaging (DSC-PWI), and magnetic resonance spectroscopy (MRS). Solid contrast-enhanced lesions of hemangioblastomas showed increased apparent diffusion coefficient values on DWI, increased relative cerebral blood volume ratio on DSC-PWI, and high lipid/lactate peak on MRS. Therefore, advanced MRI techniques can be helpful in understanding the pathological and metabolic changes of hemangioblastomas and may be useful for their characterization.

Sheath shape pattern during leadless pacemaker implantation.

PURPOSE: Options for shaping the delivery sheath of leadless pacemakers (LPs) based on the cardiac anatomy of patients are limited. We predicted the shape of the LP sheath during implantation using preoperative computed tomography (CT) and intraoperative fluoroscopy. METHODS: Forty-eight patients with implanted LPs due to symptomatic bradyarrhythmia were divided into two groups, α-loop and non-α-loop, based on the shape of the LP delivery sheath head at implantation. Angles between the inferior vena cava (IVC) and the interventricular septum (IVST), and the IVC and right ventricular apex (RVA) were measured by CT. The relationship between the final sheath shape and position of the IVC and the right or left side of the line drawn vertically from the deflection point of the sheath in the LAO view on fluoroscopy was assessed. RESULTS: Angles between the IVC and IVST (44.4 ± 5.9° vs. 50.2 ± 6.8°) and IVC and RVA (52.5 ± 5.3° vs. 58.8 ± 7.8°) on CT were significantly (p < 0.01) smaller in the α-loop group. To predict the α-loop shape, a combined IVC-IVST angle < 50° and IVC-RVA angle < 55° revealed higher sensitivity (81.8%). The delivery sheath positioned right of the vertical line was more frequent in the α-loop group (90.9% vs. 23.1%, p < 0.01). CONCLUSIONS: When the preoperatively calculated angles of IVC to IVST and RVA on CT were narrow, the right side of the sheath in the IVC from the vertical line drawn from the deflection point in the LAO view indicated the need to shape the delivery sheath head into an α-loop during LP implantation.

Proper use of fentanyl facilitates anesthesia during pulmonary vein isolation.

Although intraoperative anesthetic management of extensive encircling pulmonary vein isolation (PVI) is essential for the safe performance of this procedure, there is no standardized approach for the use of sedation and analgesia. Therefore, the present study aimed to clarify the optimal fentanyl dosage and timing of administration for the anesthetic management during PVI. A total of 364 patients with atrial fibrillation (AF) who underwent PVI at our institution between June 2017 and October 2020 were recruited. All patients were anesthetized with propofol for induction and maintenance under controlled ventilation via the supraglottic airway without neuromuscular blocking drugs. Among them, 234 patients received less frequent injections (Group 1) and 130 received a scheduled injection of 50 mg of fentanyl (Group 2) in addition to propofol during PVI. We compared the total and additional propofol doses, frequency of additional propofol, and procedure time between the two groups. The mean patient age was 67.2 years, and 69% were male. The total propofol dose was significantly lower in Group 2 than in Group 1 (17.0 ± 5.2 mg/kg vs. 19.0 ± 5.5 mg/kg, p < 0.01). The loading dose and frequency of additional propofol were also significantly lower in Group 2 than in Group 1. The procedure time was significantly shorter in Group 2 than in Group 1 (119 ± 36 min vs. 132 ± 31 min, p < 0.01). During PVI, proper use of fentanyl decreased the propofol dose, additional propofol frequency, and procedure time.

Effects of Radiofrequency Catheter Ablation on Cardiac Reserve Using Preload Stress Echocardiography in Paroxysmal and Persistent Atrial Fibrillation.

The effects of catheter ablation on exercise tolerance and quality of life in patients with atrial fibrillation (AF) have been reported. We assessed cardiac function in more detail using the leg positive pressure (LPP) technique and found that contractile reserve is particularly important in relation to exercise tolerance and prognosis. In this study, we used the LPP technique to examine changes in contractile reserve immediately after ablation and 6 months later. We prospectively enrolled patients who underwent catheter ablation for AF at 2 institutes. We performed LPP stress echocardiography 2 to 3 days after (FU-1) and 6 months after (FU-2) ablation to examine changes in cardiac function indexes. The primary end point was improvement in contractile reserve. Ultimately, 109 patients (mean age 67.4 ± 9.6 years; 70% men) underwent 2 sessions of LPP stress echocardiography. The median CHA2DS2-VASC score was 2 (interquartile range 13). From FU-1 to FU-2, the change in the stroke volume index after the LPP maneuver increased in patients with paroxysmal and persistent AF with low CHA2DS2-VASC scores (both p <0.05). Regardless of AF subtype, contractile reserve at FU-2 improved in patients with low CHA2DS2-VASC scores compared with that at FU-1. In contrast, patients with high CHA2DS2-VASC scores had no change. In conclusion, patients with AF with a low CHA2DS2-VASC score had improved contractile reserve after ablation, whereas patients with high scores did not show any improvement. Aggressive interventions in patients with high scores may lead to better management after catheter ablation.

Complete resolution of severe secondary mitral regurgitation accompanying eosinophilic myocarditis due to immunosuppressive treatment.

Eosinophilic myocarditis (EM) is an under-diagnosed inflammatory heart disease that often leads to severe left ventricular (LV) dysfunction. Meanwhile, severe secondary mitral regurgitation (MR) with valve disruption, possibly requiring mitral valve repair, is rarely concomitant with EM. We present the case of a 64-year-old female diagnosed with heart failure with severe LV dysfunction and localized asynergy. Echocardiography revealed severe secondary MR with mitral valve disruption. Cardiac magnetic resonance imaging (CMR) showed transmural late-gadolinium enhancement localized in the anterior wall and diffuse high-signal areas on T2-weighted images, suggesting non-ischemic and inflammatory heart disease. Although the peripheral eosinophil count was not elevated on admission, it gradually increased during hospitalization. These findings encouraged us to perform endomyocardial biopsy, which confirmed myocardial eosinophilic infiltration with mild fibrosis and necrosis, leading to the diagnosis of EM. Immunosuppressive treatment with oral corticosteroids improved LV dysfunction and completely resolved severe secondary MR. The current case highlighted that comprehensive assessment of laboratory, imaging, and pathological examinations including CMR is crucial to develop the appropriate therapeutic strategy for refractory heart failure. Immunosuppressive treatment should be considered as the first therapeutic option even in EM cases with severe secondary MR, possibly requiring mitral valve repair. .