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Supplemental Digital Content is available in the text., Background and importance Bystander cardiopulmonary resuscitation (CPR) and initial shockable rhythm are crucial predictors of survival after out-of-hospital cardiac arrest (OHCA). However, the relationship between dispatcher-assisted CPR (DA-CPR) and initial shockable rhythm is not completely elucidated. Objective To examine the association of DA-CPR with initial shockable rhythm and outcomes. Design, setting and participants This nationwide population-based observational study conducted in Japan included 59 688 patients with witnessed OHCA of cardiac origin after excluding those without bystander CPR. Patients were divided into DA-CPR (n = 42 709) and CPR without dispatcher assistance (unassisted CPR, n = 16 979) groups. Outcome measures and analysis The primary outcome measure was initial shockable rhythm, and secondary outcome measures were 1-month survival and neurologically intact survival. A Cox proportional hazards model adjusted for collapse-to-first-rhythm-analysis time and multivariable logistic regression models were used after propensity score (PS) matching to compare the incidence of initial shockable rhythm and outcomes, respectively. Main results Among all patients (mean age 76.7 years), the rates of initial shockable rhythm, 1-month survival and neurologically intact survival were 20.8, 10.7 and 7.0%, respectively. The incidence of initial shockable rhythm in the DA-CPR group (20.4%, 3462/16 979) was significantly higher than that in the unassisted CPR group (18.5%, 3133/16 979) after PS matching (P < 0.0001). However, no significant differences were found between the two groups with respect to the incidence of initial shockable rhythm in the Cox proportional hazards model [adjusted hazard ratio of DA-CPR for initial shockable rhythm compared with unassisted CPR, 0.99; 95% confidence interval (CI), 0.97–1.02, P = 0.56]. No significant differences were observed in the survival rates in the two groups after PS matching [10.8% (1833/16 979) vs. 10.3% (1752/16 979), P = 0.16] and neurologically intact survival rates [7.3% (1233/16 979) vs. 6.8% (1161/16 979), P = 0.13]. The multivariable logistic regression model showed no significant differences between the groups with regard to survival (adjusted odds ratio of DA-CPR compared with unassisted CPR: 1.00; 95% CI, 0.89–1.13, P = 0.97) and neurologically intact survival (adjusted odds ratio: 1.12; 95% CI, 0.98–1.29, P = 0.14). Conclusion DA-CPR after OHCA had the same independent association with the likelihood of initial shockable rhythm and 1-month meaningful outcome as unassisted CPR.

The relapse of acute lymphoblastic leukemia (ALL) usually involves the bone marrow, with the central nervous system being the most frequent extramedullary site. The relapse of ALL in the female genital organs, particularly the uterus, is markedly rare. We report such a patient who developed relapse in the bone marrow and uterus. The uterine lesion, which presented as abnormal uterine bleeding, consisted of a mass on MRI and proliferation of ALL cells on histology. MRI revealed a heterogeneous high-intensity mass (T2-WI/D-WI) with a diameter of 6.8 cm, a notable decrease in the apparent diffusion coefficient (ADC), and mild enhancement by contrast enhancement study. Histological findings of the uterine cervix demonstrated the infiltration of ALL. The patient achieved remission by allogeneic haplo-identical hematopoietic stem-cell transplantation, but died of complications of the transplantation. This case suggested that attention should be paid to the uterus as a site of extramedullary relapse. In addition, abnormal uterine bleeding, which is a common sign of hormonal imbalance and hormone replacement therapy after chemotherapy, may be an initial sign of extramedullary recurrence. To confirm uterine relapse as an intractable disease, the accumulation of more cases is required.

Mixed chimerism (MC) and/or secondary graft failure (SGF) with recipient- or donor-type chimerism is a major obstacle in allogeneic transplantation for aplastic anemia (AA). From a registry database in Japan, patients with AA age >15 years who underwent a first allogeneic bone marrow or peripheral blood stem cell transplantation between 2000 and 2014 and achieved engraftment were included in this study. MC that did not require either granulocyte-colony stimulating factor (G-CSF) or transfusion support (group 1), MC (not SGF) that required G-CSF and/or transfusion support (group 2), SGF with MC or complete recipient-type chimerism (group 3), and SGF with complete donor-type chimerism (group 4) developed in 26, 16, 19, and 17 patients, respectively. The overall median duration of follow-up for survivors was 1727 days. The overall survival (OS) was 90.4% at 1 year and 83.5% at 5 years in patients without MC or SGF (n = 340), which was not different from the OS in groups 1 and 2. However, inferior OS was observed in group 3 (1 year, 52.1%; 5 years, 52.1%) and group 4 (1 year, 82.4%; 5 years, 56.3%). In multivariate analyses, the use of fludarabine (Flu) and the absence of irradiation in conditioning were associated with the development of SGF with MC or complete recipient-type chimerism, and the use of Flu in conditioning was associated with SGF with complete donor-type chimerism. In conclusion, the use of Flu may affect the occurrence of SGF with both recipient-type and donor-type chimerism.

Background The International Liaison Committee on Resuscitation recommends that dispatchers provide instructions to perform compression-only cardiopulmonary resuscitation (CPR) to callers responding to adults with out-of-hospital cardiac arrest (OHCA). This study aimed to determine the optimal dispatcher-assisted CPR (DA-CPR) instructions for OHCA. Methods We analysed the records of 24,947 adult patients (aged ≥ 18 years) who received bystander DA-CPR after bystander-witnessed OHCA. Data were obtained from a prospectively recorded Japanese nationwide Utstein-style database for a 2-year period (2016–2017). Patients were divided into compression-only DA-CPR (n = 22,778) and conventional DA-CPR (with a compression-to-ventilation ratio of 30:2, n = 2169) groups. The primary outcome measure was 1-month neurological intact survival, defined as a cerebral performance category score of 1–2 (CPC 1–2). Results The 1-month CPC 1–2 rate was significantly higher in the conventional DA-CPR group than in the compression-only DA-CPR group (before propensity score (PS) matching, 7.5% [162/2169] versus 5.8% [1309/22778], p p p p Conclusion Within the limitations of this retrospective observational study, conventional DA-CPR with a compression-to-ventilation ratio of 30:2 was preferable to compression-only DA-CPR as an optimal DA-CPR instruction for coaching callers to perform bystander CPR for adult patients with bystander-witnessed OHCAs.

As asphyxial cardiac arrest is more common than cardiac arrest from a primary cardiac event in paediatric cardiac arrest, effective ventilation is important during paediatric cardiopulmonary resuscitation (CPR). We aimed to determine optimal dispatcher-assisted CPR instructions for bystanders after paediatric out-of-hospital cardiac arrest (OHCA).We analysed the records of 8172 children who received bystander dispatcher-assisted CPR. Data were obtained from an All-Japan Utstein-style registry from 2005 to 2017. Patients were divided into conventional CPR and compression-only CPR groups. The primary study endpoint was 1-month neurologically intact survival, defined as a Cerebral Performance Category score of 1 or 2 (CPC 1-2).The 1-month CPC 1-2 rate was significantly higher in the dispatcher-assisted conventional CPR group than in the dispatcher-assisted compression-only CPR group (before propensity score matching, 5.7% [175/3077] vs. 3.1% [160/5095], p 0.0001, adjusted odds ratio 2.48, 95% confidence interval 1.19-3.22; after propensity score matching, 6.0% [156/2618] vs. 2.6% [69/2618], p 0.0001, adjusted odds ratio 2.42, 95% confidence interval 1.76-3.32). In most subgroup analyses after matching, dispatcher-assisted conventional CPR had a higher CPC 1-2 rate than dispatcher-assisted compression-only CPR; however, CPC 1-2 rates were similar between the two groups for patients with an initial shockable rhythm, those with total prehospital CPR time ≥ 20 min, those receiving public access defibrillation, advanced airway management, or adrenaline administration.Within the limitations of this retrospective observational study, dispatcher-assisted conventional CPR was preferable to dispatcher-assisted compression-only CPR as optimal CPR instructions for coaching callers to perform bystander CPR.

Aim: Ventricular fibrillation (VF) cardiac arrest may consist of three time-sensitive phases: electrical, circulatory, and metabolic. However, the time boundaries of these phases are unclear. We aimed to determine the time boundaries of the three-phase model for VF cardiac arrest. Methods: We reviewed 20,741 out-of-hospital cardiac arrest cases with initial VF and presumed cardiac origin from the All-Japan Utstein-style registry between 2013 and 2017. The study endpoint was 1-month neurologically intact survival. The collapse-to-shock interval was defined as the time from collapse to the first shock delivery by emergency medical service personnel. The patients were divided into the bystander cardiopulmonary resuscitation (CPR, n = 11,606) and non-bystander CPR (n = 9135) groups. Results: In the bystander CPR group, the collapse-to-shock times that were associated with increased adjusted 1-month neurologically intact survival, compared with those in the non-bystander CPR group, ranged from 7 min (42.9% [244/4999] vs. 26.0% [119/458], adjusted odds ratio [aOR], 1.95; 95% confidence interval [CI], 1.44–2.63; P 17 min onward.

Background It remains unclear whether men have more favorable survival outcomes after out-of-hospital cardiac arrest (OHCA) than women. Methods We reviewed a total of 386,535 patients aged ≥ 18 years with OHCA who were included in the Japanese registry from 2013 to 2016. The study endpoints were the rates of 1-month survival and neurologically intact survival (Cerebral Performance Category Scale score = 1 or 2). Based on age, the reviewed patients were categorized into the following eight groups: 0.02). Moreover, after hierarchical propensity score matching, the survival outcomes did not significantly differ between both sexes (all P > 0.05). Conclusions No significant sex-specific differences were found in the rates of 1-month survival and neurologically intact survival after OHCA. Electronic supplementary material The online version of this article (10.1186/s13054-019-2547-x) contains supplementary material, which is available to authorized users.

Exophiala dermatitidis is a dematiaceous fungus that is increasingly becoming the cause of fungal infection in immunocompromised patients. However, the risk factors and optimal treatment modality for E. dermatitidis infection are unknown to date. Herein, we present a fatal case of E. dermatitidis infection in an adult patient that developed after allogeneic hematopoietic stem cell transplantation for chronic active Epstein-Barr virus infection. The dematiaceous fungus caused a breakthrough fungemia despite prophylactic administration of micafungin. Although the patient was intensively treated with liposomal-amphotericin B and voriconazole, serum level of beta-D-glucan continuously increased, and the patient eventually died because of cerebral hemorrhage. An autopsy found multiple involvements of the fungal infection at the bilateral lungs, thoracic cavities, diaphragm, and thyroid. To the best of our knowledge, this is the first reported case of E. dermatitidis infection involving these tissues as determined via autopsy. This case highlights the importance of attention for Exophiala infection in immunocompromised individuals in those given antifungal therapy with echinocandins.

Background Guidelines for cardiopulmonary resuscitation (CPR) recommend using the universal termination-of-resuscitation (TOR) rule to identify out-of-hospital cardiac arrest (OHCA) patients eligible for field termination of resuscitation, thus avoiding medically futile transportation to the hospital. However, in Japan, emergency medical services (EMS) personnel are forbidden from terminating CPR in the field and transport almost all patients with OHCA to hospitals. We aimed to develop and validate a novel TOR rule to identify patients eligible for field termination of CPR. Methods We analyzed 540,478 patients with OHCA from 2011 to 2015 using a Japanese registry. Main outcome measures were specificity and positive predictive value (PPV) of the newly developed TOR rule in predicting 1-month mortality after OHCA. Results Recursive partitioning analysis in the development group (n = 434,208) showed that EMS personnel could consider TOR if patients with OHCA met all of the following five criteria: (1) initial asystole, (2) arrest unwitnessed by a bystander, (3) age ≥81 years, (4) no bystander-administered CPR or automated external defibrillator use before EMS arrival, and (5) no return of spontaneous circulation after EMS-initiated CPR for 14 min. For patients meeting these criteria, specificity and PPV for predicting 1-month mortality were 99.2% [95% confidence interval (CI), 99.0–99.3%] and 99.7% (95% CI, 99.6–99.7%), respectively, for the development group and were 99.5% (95% CI, 99.3–99.7%) and 99.8% (95% CI, 99.7–99.9%), respectively, for the validation group. Implementation of this novel rule would reduce patient transports to hospitals by 10.6% in the development group and 10.4% in the validation group. Conclusions Having both high specificity and PPV of >99% for predicting 1-month mortality, our developed TOR rule may be applied in the field for Japanese patients with OHCA who meet all five criteria. Prospective validation studies and establishment of prehospital EMS protocol are required before implementing this rule.

Background/Introduction Shockable rhythm after cardiac arrest is highly expected after early initiation of bystander cardiopulmonary resuscitation (CPR) owing to increased coronary perfusion. However, the relationship between bystander CPR and initial shockable rhythm in patients with out-of-hospital cardiac arrest (OHCA) remains unclear. We hypothesized that chest-compression-only CPR (CC-CPR) before emergency medical service (EMS) arrival has an equivalent effect on the likelihood of initial shockable rhythm to the standard CPR (chest compression plus rescue breathing [S-CPR]). Purpose We aimed to examine the rate of initial shockable rhythm and 1-month outcomes in patients who received bystander CPR after OHCA. Methods The study included 59,688 patients (age, ≥18 years) who received bystander CPR after an OHCA with a presumed cardiac origin witnessed by a layperson in a prospectively recorded Japanese nationwide Utstein-style database from 2013 to 2017. Patients who received public-access defibrillation before arrival of the EMS personnel were excluded. The patients were divided into CC-CPR (n=51,520) and S-CPR (n=8168) groups according to the type of bystander CPR received. The primary end point was initial shockable rhythm recorded by the EMS personnel just after arrival at the site. The secondary end point was the 1-month outcomes (survival and neurologically intact survival) after OHCA. In the statistical analyses, a Cox proportional hazards model was applied to reflect the different bystander CPR durations before/after propensity score (PS) matching. Results The crude rate of the initial shockable rhythm in the CC-CPR group (21.3%, 10,946/51,520) was significantly higher than that in the S-CPR group (17.6%, 1441/8168, p Conclusions Compared with S-CPR, the CC-CPR before EMS arrival had an equivalent multivariable-adjusted association with the likelihood of initial shockable rhythm in the patients with OHCA due to presumed cardiac causes that was witnessed by a layperson. Funding Acknowledgement Type of funding source: None

Introduction: Placenta previa (PP) is one of the most significant risk factors for adherent placenta (AP). The aim of this study was to evaluate the diagnostic efficacy of a novel scoring system for predicting AP in pregnant women with PP. Methods: This prospective cohort study enrolled 175 women with PP. The placenta previa with adherent placenta score (PPAP score) is composed of 2 categories: (1) past history of cesarean section (CS), surgical abortion, and/or uterine surgery; and (2) ultrasonography and magnetic resonance imaging findings. Each category is graded as 0, 1, 2, or 4 points, yielding a total score between 0 and 24. When women with PP had PPAP score ≥8, they were considered to be at a high risk for AP and received placement of preoperative internal iliac artery occlusion balloon catheters. If they were found to have AP during CS, they underwent hysterectomy or placenta removal using advanced bipolar with balloon catheter occlusion. The predictive accuracy of PPAP score was evaluated. Results: In total, 23 of the 175 women with PP were diagnosed as having AP, histopathologically or clinically. Twenty-one of 24 women with PPAP score ≥8 had AP, whereas two of 151 women with PPAP score

The purpose of this study was to clarify whether a gait analysis using an accelerometer could estimate gait independence. Eighty-six stroke patients and 21 healthy control subjects participated in this study. Stroke patients were identified as dependent or independent based on their gait ability. The acceleration of the trunk and bilateral thigh was measured using three wireless sensors during walking. The root mean square, gait regularity, and symmetry were calculated from the acceleration to estimate gait quality. ANCOVA showed that gait regularity of the trunk and bilateral thigh were significantly lowest in the dependent group, regardless of gait velocity. A logistic regression analysis showed that the regularity and root mean square of the anteroposterior acceleration of the unaffected thigh were the key factors for estimating gait independence. This study suggests that an acceleration-based gait analysis facilities gait independence estimation, and is a useful tool during the rehabilitation of stroke patients.

Background The Glasgow prognostic score (GPS) has been reported as a sensitive prognostic marker for gastric cancer. This study aimed to investigate whether the GPS is equally applicable to patients with early-stage and advanced-stage gastric cancers. Methods Patients (n = 544) who underwent elective gastrectomy for gastric cancer between 2007 and 2015 were retrospectively studied. GPSs of 2, 1, and 0 were allocated to patients with both an elevated C-reactive protein level (> 1.0 mg/dL) and hypoalbuminemia (< 3.5 mg/dL), patients with only one of these abnormalities, and patients with neither abnormality, respectively. The prognostic factors relevant to patients with early-stage (pStage I, n = 304) and advanced-stage (pStage II, III, and IV, n = 240) gastric cancer were analyzed through univariate and multivariate analyses. Results In the early-stage group, only the serum carbohydrate antigen (CA) 19-9 level (P = 0.037) was a significant prognostic factor in the multivariate analysis; the GPS was not significant (P = 0.095). In the advanced-stage group, an American Society of Anesthesiologists physical status of 3 or 4 (P = 0.032), elevated carcinoembryonic antigen (CEA) (P = 0.043) and CA19-9 (P = 0.045) levels, a GPS 1 - 2 (P = 0.017), and type 4 tumor (P = 0.020) correlated significantly with worse overall survival. Conclusions GPS is a simple and useful prognostic score for patients with advanced-stage, but is not applicable to early-stage patients.

Aim: Among patients with paediatric out-of-hospital cardiac arrests (OHCAs), most have an initial non-shockable rhythm with poor outcomes. There is a subset who developed shockable rhythms. This study aimed to investigate the association between subsequent shock delivery and outcomes after paediatric OHCAs. Methods: We analysed records of 19,095 children (aged

Objectives: This study aimed to investigate the validity of gastric cancer surgery in elderly patients. Methods: A total of 544 patients who underwent elective gastrectomy for gastric cancer were divided into an elderly group (age ≥75 years, n = 171) and a control group (age n = 373). The clinicopathological data of the patients were reviewed. Results: The overall morbidity rate (26.3 vs. 16.1%, p = 0.005) and the incidence rate of anastomotic leakage (6.4 vs. 1.6%, p = 0.003) were significantly higher in the elderly group. The proportion of patients who had severe complications (≥grade IIIa) was relatively higher in the elderly group (10.5 vs. 5.7%); however, the difference was not significant (p = 0.074). A stage-matched survival analysis revealed no significant differences between the groups (stage I: p = 0.978; stage II: p = 0.964; stage III: p = 0.199). For the pathological stages II and III, the overall survival of the patients in the elderly group who received adjuvant chemotherapy for >3 months was significantly better than that of the patients who received it for ≤3 months or did not receive it (p = 0.023). Conclusions: An aggressive treatment strategy should be adopted in selected elderly patients with gastric cancer.

We consecutively examined the utility of measurements of percentage of immature platelet fraction (IPF%) and absolute IPF number (A-IPF) in predicting thrombopoietic recovery in 15 adult patients who underwent allogeneic hematopoietic stem cell transplantation (allo-SCT). Four patients were excluded from the evaluation due to insufficient data. Platelet count and IPF were measured by Sysmex XN-1000 (XN), a newer generation analyzer. First, we confirmed that platelet count measured by XN was more accurate than by XE-2100 (XE). IPF measurement was effective to predict the recovery in 7 of the 11 patients examined. Moreover, IPF measurement, especially IPF% measurement, suggested accelerated platelet turnover in two patients who failed to achieve platelet recovery by day 60. In addition to IPF%, A-IPF showed a complementary role on the prediction of thrombopoietic recovery. The increase in IPF% was only transient, while A-IPF values showed lasting increase during platelet recovery. In two patients (cases 6 and 7) an increase in A-IPF, but not in IPF%, was observed during platelet recovery. Our data suggest that IPF% and A-IPF measured by XN are useful for the prediction of thrombopoietic recovery and the assessment of pathogenesis of thrombocytopenia in patients after allo-SCT.

A 57-year-old female patient on hemodialysis with chronic renal failure due to chronic glomerular nephritis received deceased donor kidney transplantation. Induction immunosuppressive therapy was combination of tacrolimus, mycophenolate mofetil, everolimus, prednisolone, and basiliximab. She was diagnosed with secondary thrombotic microangiopathy (TMA) by clinical findings such as hemolytic anemia, thrombocytopenia and acute kidney injury not by pathological findings on the 4th post-operative date. Plasma exchange was performed with suspension of tacrolimus. General conditions recovered, and the graft function was preserved.

Background The effects of prehospital epinephrine administration in combination with the quality of cardiopulmonary resuscitation (CPR) on neurological outcomes in out-of-hospital cardiac arrest (OHCA) patients with non-shockable rhythm remains unclear. Purpose This study aimed to elucidate the effects of prehospital epinephrine administration in combination with the quality of CPR on neurologically intact survival in OHCA patients with non-shockable rhythm. Methods We analysed 118,732 adult OHCA patients with non-shockable rhythm from the All-Japan OHCA registry between 2011 and 2016 (29,989 emergency medical service [EMS]-witnessed arrests with EMS-initiated CPR [high-quality CPR] and 88,743 bystander-witnessed arrests with bystander-initiated CPR continued by EMS providers [low-quality CPR]). Patients who achieved prehospital return of spontaneous circulation without prehospital epinephrine administration were excluded. The primary outcome measure was 1-month neurologically intact survival (cerebral performance category 1 or 2; CPC 1–2). Time from collapse to prehospital epinephrine administration for patients with prehospital epinephrine administration, or to hospital arrival for patients without prehospital epinephrine administration was calculated and adjusted collectively in multivariate logistic regression analysis for 1-month CPC 1–2. Results Multivariate logistic regression analysis revealed that the time from collapse to prehospital epinephrine administration or to hospital arrival was negatively associated with 1-month CPC 1–2 (adjusted odds ratio [OR] 0.95 per 1-minute increment, 95% confidence interval [CI] 0.94–0.96). Compared with bystander-witnessed arrests without prehospital epinephrine administration, EMS-witnessed arrests with or without prehospital epinephrine administration were significantly associated with increased chances of 1-month CPC 1–2 (adjusted OR 2.04, 95% CI 1.50–2.75 and adjusted OR 1.97, 95% CI 1.57–2.48, respectively). Prehospital epinephrine administration was significantly associated with an increased chance of 1-month CPC 1–2 among bystander-witnessed arrests (adjusted OR 1.57, 95% CI 1.24–1.98), but not among EMS-witnessed arrests. EMS-witnessed arrests without prehospital epinephrine administration were significantly associated with an increased chance of 1-month CPC 1–2 compared with bystander-witnessed arrests with prehospital epinephrine administration (adjusted OR 1.26, 95% CI 1.01–1.56). Conclusions High-quality CPR is crucial for increasing neurologically intact survival in OHCA patients with non-shockable rhythm. The additional beneficial effects of prehospital epinephrine administration were observed only among OHCA patients with low-quality CPR.

Background Data on the effects of witness status and time from an emergency call to initiation of cardiopulmonary resuscitation (CPR) by emergency medical service (EMS) providers on neurological outcome in out-of-hospital cardiac arrest (OHCA) patients with non-shockable rhythm according to the first documented rhythm are limited. Purpose We aimed to determine the effects of witness status and time from an emergency call to CPR initiation by EMS providers on neurologically intact survival in OHCA patients according to the type of non-shockable rhythm (pulseless electrical activity [PEA] and asystole). Methods We analysed the records of 583,431 adult OHCA patients with non-shockable rhythm (191,905 bystander-witnessed arrest and 391,526 unwitnessed arrest). Data were derived from the prospectively recorded All-Japan OHCA registry between 2011 and 2016. Call to EMS-CPR interval was defined as the time from an emergency call to CPR initiation by EMS providers. The primary outcome was 1-month neurologically intact survival (cerebral performance category 1 or 2; CPC 1–2) and secondary outcome was presence of PEA. Results The rates of 1-month CPC 1–2 were 1.21% (2,326/191,905) for bystander-witnessed arrest and 0.24% (959/391,526) for unwitnessed arrest. When divided into 4 groups based on witness status and initial documented rhythm, these rates were 2.42% (1,869/77,190) for bystander-witnessed arrest with PEA (group A), 0.40% (457/114,715) for bystander-witnessed arrest with asystole (group B), 1.51% (679/44,926) for unwitnessed arrest with PEA (group C) and 0.08% (280/346,600) for unwitnessed arrest with asystole (group D). Multivariate logistic regression analysis revealed each 1-min delay of Call to EMS-CPR interval to be significantly associated with decreased chances of 1-month CPC 1–2 for groups A, B and D (adjusted odds ratio [OR]: 0.95, 0.91 and 0.96, respectively; 95% confidence interval [CI]: 0.93–0.96, 0.88–0.94 and 0.93–0.99, respectively). However, for group C, there was no significant relationship between these variables (adjusted OR: 1.00; 95% CI: 0.98–1.02). The proportion of PEA was 40.2% (77,190/191,905) for bystander-witnessed arrest and 11.5% (44,926/391,526) for unwitnessed arrest. Multivariate logistic regression analysis revealed that, as Call to EMS-CPR interval lengthened (per 1-min delay), the number of OHCA patients with PEA decreased for bystander-witnessed arrest (adjusted OR: 0.94; 95% CI: 0.93–0.94) and for unwitnessed arrest (adjusted OR: 0.96; 95% CI: 0.96–0.97). Conclusions The 1-month CPC 1–2 rate differed by witness status and initial documented rhythm in OHCA patients with non-shockable rhythm. Shortening of Call to EMS-CPR interval is crucial for improving 1-month CPC 1–2 rate and sustaining PEA, particularly in bystander-witnessed arrest.

Background Bronchiolitis obliterans syndrome (BOS) is the term used for the progressive obliteration of small airways before the patient has had a confirmatory lung biopsy. It is also recognized as a transplant-related complication. There have been no reports of BOS during initial standard chemotherapy. Case presentation A 50-year-old woman with newly diagnosed follicular lymphoma grade 2, stage 3A, presented with hypoxia and progressive dyspnoea after the fifth cycle of R-CHOP. High-resolution computed tomography showed air trapping enhanced at the end-expiratory phase. Pulmonary function testing revealed severe obstructive and restrictive failure without bronchodilator response. We diagnosed BOS based on current criteria and treated the patient with glucocorticoids and cyclosporin. She was discharged home on oxygen therapy. However, soon after discharge, her respiratory symptoms deteriorated and she was hospitalized in a palliative care unit. She died of respiratory failure within a year of symptom onset. Conclusions This is the first case report to describe rapidly progressive BOS in a patient undergoing R-CHOP treatment, which strongly suggests the condition was caused by the chemotherapy. Although a pathological diagnosis was not obtained, the clinical diagnosis of BOS was important so that the patient could receive appropriate treatment and palliative care based on the prognosis of this incurable condition. Learning points R-CHOP chemotherapy may cause rapidly progressive bronchiolitis obliterans syndrome (BOS).When it is difficult to obtain a pathological diagnosis, a clinical diagnosis of BOS is important for early intervention and appropriate palliative care.

Various central nervous system (CNS) complications may occur after allogeneic hematopoietic stem cell transplantation (allo-HSCT), which can result in severe clinical problems. Diagnosis is often difficult, as distinctive clinical symptoms may be absent and different neurological disorders may exhibit similar symptoms. Despite the fact that antibodies responding to brain cell surface antigens have become well recognized in several CNS disorders, cases of autoimmune CNS disorders after allo-HSCT have rarely been reported. Here, we report on a patient who developed encephalitis associated with antibodies against N-methyl-D-aspartate (NMDA)-type glutamate receptor (GluR) after allo-HSCT. To the best of our knowledge, this is the first report of the involvement of antibodies against NMDA-type GluR in post-transplantation encephalitis. Autoimmunity to NMDA-type GluR may have contributed to neurological complications after transplantation in unresolved cases.

Purpose To investigate the impacts of emergency calls made using mobile phones on the quality of dispatcher-assisted cardiopulmonary resuscitation (DA-CPR) and survival from out-of-hospital cardiac arrests (OHCAs) that were not witnessed by emergency medical service (EMS). Methods In this prospective study, we collected data for 2530 DA-CPR-attempted medical emergency cases (517 using mobile phones and 2013 using landline phones) and 2980 non-EMS-witnessed OHCAs (600 using mobile phones and 2380 using landline phones). Time factors and quality of DA-CPR, backgrounds of callers and outcomes of OHCAs were compared between mobile and landline phone groups. Results Emergency calls are much more frequently placed beside the arrest victim in mobile phone group (52.7% vs. 17.2%). The positive predictive value and acceptance rate of DA-CPR in mobile phone group (84.7% and 80.6%, respectively) were significantly higher than those in landline group (79.2% and 70.9%). The proportion of good-quality bystander CPR in mobile phone group was significantly higher than that in landline group (53.5% vs. 45.0%). When analysed for all non-EMS-witnessed OHCAs, rates of 1-month survival and 1-year neurologically favourable survival in mobile phone group (7.8% and 3.5%, respectively) were higher than those in landline phone group (4.6% and 1.9%; p, Embargo Period 12 months

To compare the factors associated with survival after out-of-hospital cardiac arrests (OHCAs) among three time-distance areas (defined as interquartile range of time for emergency medical services response to patient's side).From a nationwide, prospectively collected data on 716,608 OHCAs between 2007 and 2012, this study analyzed 193,914 bystander-witnessed OHCAs without pre-hospital physician involvement.Overall neurologically favourable 1-month survival rates were 7.4%, 4.1% and 1.7% for close, intermediate and remote areas, respectively. We classified BCPR by type (compression-only vs. conventional) and by dispatcher-assisted CPR (DA-CPR) (with vs. without); the effects on time-distance area survival were analyzed by BCPR classification. Association of each BCPR classification with survival was affected by time-distance area and arrest aetiology (p0.05). The survival rates in the remote area were much higher with conventional BCPR than with compression-only BCPR (odds ratio; 95% confidence interval, 1.26; 1.05-1.51) and with BCPR without DA-CPR than with BCPR with DA-CPR (1.54; 1.29-1.82). Accordingly, we classified BCPR into five groups (no BCPR, compression-only with DA-CPR, conventional with DA-CPR, compression-only without DA-CPR, and conventional without DA-CPR) and analyzed for associations with survival, both cardiac and non-cardiac related, in each time-distance area by multivariate logistic regression analysis. In the remote area, conventional BCPR without DA-CPR significantly improved survival after OHCAs of cardiac aetiology, compared with all the other BCPR groups. Other correctable factors associated with survival were short collapse-to-call and call-to-first CPR intervals.Every effort to recruit trained citizens initiating conventional BCPR should be made in remote time-distance areas.

Late-onset noninfectious pulmonary complication after allogenic hematopoietic stem cell transplantation is an important contributing factor associated with high rate morbidity and mortality. We report a case with pleuroparenchymal fibroelastosis (PPFE) occurred after allogenic bone marrow transplantation. The onset was infiltrative shadows in upper lobes, and the haziness spread gradually throughout the lungs with recurrent episodes of pneumothorax in both lungs. Progressive respiratory failure in course of adrenocortical steroid administration eventually caused death. Histological examination in general autopsy showed patchy subpleural fibrosis predominantly distributed in the upper lobes with substantial density rise of elastic fibers in the subpleural area, consistent with the diagnosis of PPFE. PPFE after allogenic transplantation has been seldom reported to date, but it is one of the most important histological components of late-onset noninfectious pulmonary complication after allogenic transplantation characterized by recurrent pneumothorax. Retrospective analysis in our case indicates early diagnosis may be possible by histological evaluation of elastic fibers in lung specimen when pneumothorax is treated surgically. This case suspects that it is important for hematologist and pathologist to aware this progressive disease along with information of histological characteristics, therefore, leading to the establishment of therapeutic strategies and the improvement of poor prognosis.

BACKGROUND There is sparse data regarding the survival and neurological outcome of elderly patients with out-of-hospital cardiac arrest (OHCA). METHODS AND RESULTS OHCA patients (334,730) aged ≥75 years were analyzed using a nationwide, prospective, population-based Japanese OHCA database from 2008 to 2012. The overall 1-month survival with favorable neurological outcome (Cerebral Performance Category Scale, category 1 or 2; CPC 1-2) rate was 0.88%. During the study period, the annual 1-month CPC 1-2 rate in whole OHCA significantly improved (0.73% to 0.96%, P for trend

We analyzed data from 64,539 consecutive patients in the Japanese national transplant registry, including 40,195 after allogeneic hematopoietic stem cell transplantation (HSCT), 24,215 after autologous HSCT and 129 after syngeneic HSCT, of whom 299 developed Epstein-Barr virus-positive post-transplant lymphoproliferative disorder (PTLD). The probability of developing PTLD at 2 years post-HSCT was .79% after allogeneic transplantation, .78% after syngeneic transplantation, and .11% after autologous transplantation. The following variables were identified as risk factors after allogeneic HSCT in multivariate analysis: antithymocyte globulin (ATG) use in a conditioning regimen, ATG use for acute graft-versus-host disease (GVHD) treatment, donor other than an HLA-matched related donor, aplastic anemia, second or subsequent allogeneic HSCT, the most recent year of transplantation, and acute GVHD. The probability at 2 years increased particularly after 2009 (1.24%) than before 2009 (.45%). To stratify the risk of PTLD before allogeneic HSCT, we developed a novel 5-point scoring system based on 3 pretransplant risk factors: ATG use in a conditioning regimen (high dose, 2 points; low dose, 1 point), donor type (HLA-mismatched related donor, 1 point; unrelated donor, 1 point; cord blood, 2 points), and aplastic anemia (1 point). Patients were classified into 4 risk groups according to the summed points: low risk (0 or 1 point), intermediate risk (2 points), high risk (3 points), and very high risk (4 or 5 points) groups, with probabilities at 2 years of .3%, 1.3%, 4.6%, and 11.5%, respectively. Our scoring system is useful for predicting patients at high risk for PTLD. Careful observation and close monitoring of Epstein-Barr virus reactivation are warranted for these high-risk patients.

Compared with 4-times-daily infusion of intravenous busulfan (ivBU4), the safety and efficacy of once-daily infusion of ivBU (ivBU1) has not been fully clarified. We have been routinely using ivBU1 in a conditioning regimen in adult patients with myeloid malignancy who undergo allogeneic hematopoietic stem cell transplantation. In this study, a total of 91 patients who received ivBU1 for 2 days (n = 18) or 4 days (n = 73) in our institutions were compared with 273 control patients who received ivBU4, who were matched for age, sex, performance status, disease risk, conditioning regimen, and donor type, selected from the database of the Japanese Society for Hematopoietic Cell Transplantation using optimal matching algorithms. One-year overall survival (56.8% versus 57.1%, P = .94), disease-free survival (51.6% versus 50.8%, P = .73), relapse rate (28.5% versus 26.2%, P = .94), nonrelapse mortality (19.9% versus 23.0%, P = .71), and the incidence of graft-versus-host disease were not significantly different between the ivBU1 and ivBU4 groups. In patients who received ivBU1, neutrophil recovery was slower (median days: 22 versus 17, P = .001), and the incidence of veno-occlusive disease was lower (2.6% versus 17.4%, P = .04). In conclusion, ivBU1 can be safely administered with clinical outcomes similar to those with ivBU4.

Background Elderly patients have a high risk of adverse outcomes after surgery. Therefore, it is essential to determine the predictive factors for postoperative morbidity in elderly patients undergoing gastric cancer surgery. Methods A total of 544 patients who underwent elective gastrectomy for gastric cancer at Yodogawa Christian Hospital between January 2007 and December 2015 were divided into the elderly group (age ≥70 years, n=282) and a control group (age

Purpose To investigate temporal variations in dispatcher-assisted and bystander-initiated resuscitation efforts and their association with survival after bystander-witnessed out-of-hospital cardiac arrests (OHCAs). Methods We retrospectively analyzed the neurologically favorable 1-month survival and the parameters related to dispatcher assisted cardiopulmonary resuscitation (DA-CPR) and bystander CPR (BCPR) for 227,524 OHCA patients between 2007 and 2013 in Japan. DA-CPR sensitivity for OHCAs, bystander's compliance to DA-CPR assessed by the proportion of bystanders who follow DA-CPR, and performance of BCPR measured by the rate of bystander-initiated CPR in patients without DA-CPR were calculated as indices of resuscitation efforts. Results Performance of BCPR was only similar to temporal variations in the survival (correlation between hourly paired values, R2 = 0.263, P = 0.01): a lower survival rate (3.4% vs 4.2%) and performance of BCPR (23.1% vs 30.8%) during night-time (22:00–5:59) than during non-night-time. In subgroup analyses based on interaction tests, all three indices deteriorated during night-time when OHCAs were witnessed by non-family (adjusted odds ratio, 0.73–0.82), particularly in non-elderly patients. The rate of public access defibrillation for these OHCAs markedly decreased during night-time (adjusted odds ratio, 0.49) with delayed emergency calls and BCPR initiation. Multivariable logistic regression analyses revealed that the survival rate of non-family-witnessed OHCAs was 1.83-fold lower during night-time than during non-night-time. Conclusions Dispatcher-assisted and bystander-initiated resuscitation efforts are low during night-time in OHCAs witnessed by non-family. A divisional alert system to recruit well-trained individuals is needed in order to improve the outcomes of night-time OHCAs witnessed by non-family bystanders.

Aim To determine the effectiveness of ventilations in bystander cardiopulmonary resuscitation (BCPR) and to identify the factors associated with ventilation-only BCPR. Methods From out-of-hospital cardiac arrest (OHCA) data prospectively collected from 2005 to 2011 in Japan, we extracted data for 210,134 bystander-witnessed OHCAs with complete datasets but no prehospital involvement of physician [no BCPR, 115,733; ventilation-only, 2093; compression-only, 61,075; and conventional (compressions+ventilations) BCPR, 31,233] and determined the factors associated with 1-month neurologically favourable survival using simple and multivariable logistic regression analyses. In 91,885 patients with known BCPR durations, we determined the factors associated with ventilation-only BCPR. Results The rate of survival in the no BCPR, ventilation-only, compression-only and conventional group was 2.8%, 3.9%, 4.5% and 5.0%, respectively. After adjustment for other factors associated with outcomes, the survival rate in the ventilation-only group was higher than that in the no BCPR group (adjusted OR; 95% CI, 1.29; 1.01–1.63), but lower than that in the compression-only (0.76; 0.59–0.96) or conventional groups (0.70; 0.55–0.89). Conventional CPR had the highest OR for survival in almost all OHCA subgroups. The adjusted OR (95% CI) for survival after dividing BCPR into ventilation and compression components was 1.19 (1.11–1.27) and 1.60 (1.51–1.69), respectively. Older guidelines, female sex, younger patient age, bystander-initiated CPR without instruction, early BCPR and short BCPR duration were associated with ventilation-only BCPR. Conclusions Ventilation is a significant component of BCPR, but alone is less effective than compression in improving neurologically favourable survival after OHCAs.

Allogeneic hematopoietic stem cell transplantation (HSCT) has largely improved the prognosis of leukemia patients. However, relapse is still a major concern. One promising option for the prevention of relapse is vaccination therapy. The post allogeneic HSCT period provides a unique platform for vaccination, because tumor burden is minimal, lymphopenic condition allows for rapid expansion of cytotoxic T cells (CTLs), donor-derived CTLs are not exhausted and inflammatory condition is caused by allo reactions. Tumor cells, dendritic cells and peptides have been used as vaccines targeting leukemia-associated antigens or minor histocompatibility antigens. Clinical trials with several types of vaccines for post-HSCT patients showed that the vaccination induced immunological response and might benefit patients with minimal residual disease, while their effect in patients with advanced disease were limited. To enhance the effect, vaccination in combination with other immune-modulatory drugs such as checkpoint antibodies is now being considered.

Although the prognosis of leukemia patients after allogeneic hematopoietic stem cell transplantation (HSCT) has greatly improved, relapse is still a major cause of death after HSCT. Cancer vaccines may have the potential to enhance the graft-versus-leukemia (GVL) effect. The post-allogeneic HSCT period provides a unique platform for vaccination, because (I) tumor burden is minimal, (II) lymphopenia allows for rapid expansion of cytotoxic T cells (CTLs), (III) donor-derived CTLs are not exhausted, (IV) inflammation is caused by alloreactions, and (V) the abundance of regulatory T cells is low due to their late recovery. Tumor cell lysates, dendritic cells (DCs), and peptides derived from leukemia-associated antigens (LAAs) have been used as vaccines. Clinical trials with several types of vaccines for post-HSCT patients revealed that the vaccination induced an immunological response and might benefit patients with minimal residual disease; however, the efficacy of this approach must be examined in randomized studies. In addition, it is important to consider the combination of cancer vaccine with checkpoint antibodies, recently shown to be useful in treating leukemia relapse after HSCT.

We report the case of a 44-year-old woman with Masaoka stage IV, World Health Organization type B1 thymoma associated with pure red cell aplasia, thrombocytopenia, and myasthenia gravis, which occurred during preoperative chemotherapy with high-dose methylprednisolone. Noninvasive positive-pressure ventilation, intravenous immunoglobulin infusion, and methylprednisolone pulse therapy were performed for the myasthenic crisis. Disseminated thymoma was markedly reduced after these treatments, and macroscopic complete resection was performed after achieving control of pure red cell aplasia, myasthenia gravis, and thrombocytopenia using cyclosporine A. Chemotherapy, including high-dose methylprednisolone, may carry a risk of myasthenic crisis, although the regimen is effective against lymphocyte-rich thymoma.

This study evaluated the effect of foot progression angle on the reduction in knee adduction moment caused by a lateral wedged insole during walking. Twenty healthy, young volunteers walked 10 m at their comfortable velocity wearing a lateral wedged insole or control flat insole in 3 foot progression angle conditions: natural, toe-out, and toe-in. A 3-dimensional rigid link model was used to calculate the external knee adduction moment, the moment arm of ground reaction force to knee joint center, and the reduction ratio of knee adduction moment and moment arm. The result indicated that the toe-out condition and lateral wedged insole decreased the knee adduction moment in the whole stance phase. The reduction ratio of the knee adduction moment and the moment arm exhibited a close relationship. Lateral wedged insoles decreased the knee adduction moment in various foot progression angle conditions due to decrease of the moment arm of the ground reaction force. Moreover, the knee adduction moment during the toe-out gait with lateral wedged insole was the smallest due to the synergistic effect of the lateral wedged insole and foot progression angle. Lateral wedged insoles may be a valid intervention for patients with knee osteoarthritis regardless of the foot progression angle.

We have recently reported that the outcome of acute myeloid leukemia (AML) patients with t(6;9)(p23;q34) who underwent allogeneic hematopoietic stem cell transplantation (allo-HSCT) was comparable to that of patients with a normal karyotype. We performed a further analysis regarding the prognostic factors for t(6;9)(p23;q34) AML patients who underwent a HSCT. Seven pediatric patients and 57 adult patients, transplanted between 1996 and 2007, were assessed in this study. The overall survival (OS) of the pediatric patients tended to be better than the OS of the adults, although there were no statistically significant differences. The present study focused on the adult patients revealed that the disease status at HSCT was the sole prognostic factor affecting the OS identified in the univariate analysis. A multivariate analysis showed that the disease status at HSCT and M2 in the FAB classification were extracted as the significant variables affecting the OS. The patients who were not in remission at HSCT and had non-FAB-M2 showed a poorer outcome; 6 deaths in the 9 patients were due to a relapse of the AML. These findings suggest that novel therapeutic approaches might be needed for patients with these poor prognostic factors., 発行後6か月より全文公開

Acute myeloid leukemia (AML) with t(6;9)(p23;q34) is well known to have a poor prognosis treated with chemotherapy and autotransplantation. The presence of this karyotype is an indicator for allogeneic hematopoietic stem cell transplantation (HSCT); however, the impact of t(6;9)(p23;q34) on the HSCT outcome remains unclear. We conducted a matched-pair analysis of de novo AML patients with and without t(6;9)(p23;q34) using data obtained from the Japanese HSCT data registry. A total of 57 patients with t(6;9)(p23;q34) received transplants between 1996 and 2007, and 171 of 2056 normal karyotype patients matched for age, disease status at HSCT and graft source were selected. The overall survival, disease-free survival, cumulative incidence of relapse and the non-relapse mortality in t(6;9)(p23;q34) patients were comparable to those for normal karyotype patients. A univariate analysis showed that t(6;9)(p23;q34) had no significant impact on the overall survival. These findings suggest that allogeneic HSCT may overcome the unfavorable impact of t(6;9)(p23;q34) as an independent prognostic factor. © 2012 Macmillan Publishers Limited All rights reserved.

The purpose of this article is to review fluorodeoxyglucose-positron emission tomography/computed tomography (FDG-PET/CT) and magnetic resonance imaging (MRI) findings in a variety of benign, malignant, and borderline malignant ovarian tumors. It is advantageous to become familiar with the wide variety of FDG-PET/CT findings of this entity. Benign ovarian tumors generally have faint uptake, whereas endometriomas, fibromas, and teratomas show mild to moderate uptake. Malignant ovarian tumors generally have intense uptake, whereas tumors with a small solid component often show minimal uptake.

A 45-year-old man with chronic active Epstein-Barr virus (EBV) infection (CAEBV) with natural killer cell type developed pulmonary arterial hypertension (PAH). After chemotherapy, he showed marked depression of the EBV DNA genome in the peripheral blood, but PAH sustained. He died of heart failure due to PAH, and the histo-pathological examination revealed pulmonary vascular abnormalities without lung disease on autopsy. Although the EBV DNA genome and the infiltrating lymphocytes were not detected in the lung, his clinical course suggested that his PAH might be caused by CAEBV. This is the first reported case of PAH associated CAEBV in an adult.

Post-transplant lymphoproliferative disorder (PTLD) following hematopoietic stem cell transplantation (HSCT) is a rare, but life-threatening complication. PTLD typically develops within 6-12 months of HSCT, which is before the reconstitution of EBV-specific cytotoxic T-cell immunity. Several risk factors for developing PTLD have been reported in the literature, including the use of antithymocyte globulin (ATG) and ex vivo T-cell depletion (TCD). However, only a few large-scale retrospective studies have been conducted and risk scores have not yet been well defined. Therefore, to further evaluate the probability of and risk factors for PTLD and establish a risk scoring system, we conducted a retrospective multicenter analysis using data from a Japanese nationwide transplant outcome registry database. A total of 71,904 patients aged 16 years or older who received HSCT between January 1990 and December 2016 were included in this study. We excluded patients for whom information regarding the transplantation type or PTLD development was unavailable, resulting in 64,539 patients ultimately being analyzed. The probability of developing PTLD was estimated using the Kaplan-Meier method, and differences between groups were evaluated using the log-rank test. To identify risk factors for PTLD, univariate and multivariate analyses were performed using the Cox's proportional hazard regression model for patients who received allogeneic HSCT. Among the 64,539 patients analyzed, 40,195 received allogeneic HSCT, 24,215 received autologous HSCT, and 129 received syngeneic HSCT. A total of 299 patients developed PTLD, including 267 who had received allogeneic HSCT, 31 who had received autologous HSCT, and one who had received syngeneic HSCT. The median age of patients with PTLD after allogeneic HSCT was 44 years (range, 16-76), and males accounted for approximately 60%. The underlying disease was acute myeloid leukemia and myelodysplastic syndrome in 20,474 patients (51%), acute lymphoblastic leukemia in 6,808 (17%), chronic myeloid leukemia and myeloproliferative disorder in 2,827 (7%), malignant lymphoma in 7,824 (19%), aplastic anemia (AA) in 1,327 (3%) and others in 932 (2%). ATG was used in 4,026 patients (10%) who underwent allogeneic HSCT. In contrast, ex vivo TCD was used only in 296 (0.7%). The donor type was human leukocyte antigen (HLA)-matched-related donor (MRD) in 13,058 patients (33%), HLA-mismatched-related donor (MMRD) in 4,095 (10%), HLA-matched-unrelated donor (MURD) in 10,216 (25%), HLA-mismatched-unrelated donor (MMURD) in 1,993 (5%) and unrelated cord blood (CB) in 10,118 (25%), but was unknown for the remaining 715 patients (2%).The probability of PTLD at 2 years after HSCT was 0.79% with a median onset of 131 (range, 15-6,356) days in patients after allogeneic HSCT. In contrast, the probability of PTLD was 0.11% with a median onset of 198 (range, 21-4,759) days in those after autologous HSCT. Only one patient developed PTLD at 29 days in those after syngeneic HSCT (0.78%). In multivariate analyses performed for patients after allogeneic HSCT, the following variables were identified as risk factors for PTLD: the use of ATG, donor type (MMRD, MURD, MMURD and CB), disease (AA), the number of HSCT, year of HSCT, and acute graft-versus-host disease (grade II-IV). To predict the risk of developing PTLD before HSCT, we established a novel scoring system using three pre-transplant risk factors that correlated with the development of PTLD. We assigned point(s) for the three risk factors by referring to each hazard ratio of the multivariate analysis as shown in the Table. Risk scores were calculated by adding each point(s), and patients were then successfully classified into 4 risk groups: low (score 0-1), intermediate (score 2), high (score 3), and very high risk (score 4-5), with probabilities of PTLD at 2 years after HSCT of 0.3, 1.1, 3.0, and 8.6%, respectively (Figure). Although ex vivo TCD is described as a risk factor for PTLD as well as ATG, it was not identified as a significant risk factor for PTLD in our cohort, probably due to the small number of the affected patients. This scoring system enables us to predict higher risk patients before HSCT. The close monitoring of EBV-DNA may be beneficial for the early diagnosis of these patients and more rapid initiation of treatment interventions, such as rituximab and EBV-specific cytotoxic T cells. Disclosures Mori: Janssen: Honoraria; Novartis Pharma: Research Funding; Taisho Toyama Pharmaceutical Co: Honoraria; CHUGAI: Honoraria; Kyowa Hakko Kirin: Honoraria; MSD: Honoraria; Novartis Pharma: Honoraria; Asahi Kasei: Research Funding; SHIONOGI: Honoraria; Pfizer: Honoraria; Japan Blood Products Organization: Honoraria; Shire Japan: Honoraria; Astella Pharma: Honoraria; Celgene: Honoraria; MSD: Research Funding; Eisai: Honoraria; Ono: Honoraria. Kanda:Nippon-Shinyaku: Research Funding; Chugai: Consultancy, Honoraria, Research Funding; Otsuka: Research Funding; Astellas: Consultancy, Honoraria, Research Funding; Eisai: Consultancy, Honoraria, Research Funding; Dainippon-Sumitomo: Consultancy, Honoraria, Research Funding; Kyowa-Hakko Kirin: Consultancy, Honoraria, Research Funding; Taiho: Research Funding; Pfizer: Research Funding; MSD: Research Funding; Takeda: Consultancy, Honoraria, Research Funding; Asahi-Kasei: Research Funding; Ono: Consultancy, Honoraria, Research Funding; Sanofi: Research Funding; Novartis: Research Funding; Shionogi: Consultancy, Honoraria, Research Funding; Taisho-Toyama: Research Funding; CSL Behring: Research Funding; Tanabe-Mitsubishi: Research Funding; Bristol-Myers Squibb: Consultancy, Honoraria; Celgene: Consultancy, Honoraria; Mochida: Consultancy, Honoraria; Alexion: Consultancy, Honoraria; Takara-bio: Consultancy, Honoraria. Tanaka:Pfizer: Honoraria; Otsuka: Honoraria; Bristol-Myers Squibb: Honoraria, Research Funding; Novartis Pharma: Honoraria. Ichinohe:Celgene: Honoraria; Takeda Pharmaceutical Co.: Research Funding; Taiho Pharmaceutical Co.: Research Funding; Zenyaku Kogyo Co.: Research Funding; Mundipharma: Honoraria; Bristol-Myers Squibb: Honoraria; Alexion Pharmaceuticals: Honoraria; MSD: Research Funding; Otsuka Pharmaceutical Co.: Research Funding; Sumitomo Dainippon Pharma Co.: Research Funding; Repertoire Genesis Inc.: Research Funding; JCR Pharmaceuticals: Honoraria; Janssen Pharmaceutical K.K.: Honoraria; Novartis.: Honoraria; Nippon Shinyaku Co.: Research Funding; Pfizer: Research Funding; Ono Pharmaceutical Co.: Research Funding; Kyowa Hakko Kirin Co.: Research Funding; Eisai Co.: Research Funding; CSL Behring: Research Funding; Chugai Pharmaceutical Co.: Research Funding; Astellas Pharma: Research Funding. Suzuki:Celgene: Honoraria; Sawai Pharmaceutical: Honoraria; Ohtsuka: Honoraria; MSD: Research Funding; Meiji Seika Pharma: Honoraria; Takeda Pharmaceuticals: Honoraria; Shionogi: Honoraria; Novartis: Honoraria; Mochida Pharmaceutical: Honoraria; Chugai Pharmaceutical: Honoraria; Kyowa-Hakko Kirin: Honoraria; Bristol-Myers Squibb: Honoraria; Sumitomo Dainippon Pharma: Honoraria; Gilead Sciences: Consultancy; MundiPharma: Consultancy; Jazz Pharmaceuticals: Consultancy.

A combination regimen of fludarabine and myeloablative busulfan (FLU/BU4) has been widely used as a myeloablative conditioning regimen with reduced intensity for myeloid malignancies. However, FLU/BU4 has two major disadvantages. A disadvantage is the insufficient anti-leukemic effect in advanced cases. Although the overall survival (OS) after FLU/BU4 treatment is approximately 50-80% for myeloid malignancies in the 1st complete remission (CR), the regimen could result in unsatisfactory outcomes with 19-32% OS in patients with advanced disease (Shimoni 2010; Alatrash 2011). Another disadvantage is the slow achievement of complete donor-type chimerism, especially T-cell chimerism (Rambaldi 2015), which also affects the outcome of allogeneic hematopoietic stem cell transplantation (allo-HCT), particularly relapse (Peterlin 2015). To overcome these issues, we designed a combination regimen with FLU, intravenous (IV) BU, and melphalan (FLU/BU4/MEL) and conducted retrospective analyses in this study. The FLU/BU4/MEL regimen consisted of 30 mg/m2 FLU for 5 days (150 mg/m2), 3.2 mg/kg BU for 4 days (12.8 mg/kg), and 50 mg/m2 MEL for 2 days (100 mg/m2) administered by IV infusion. A total body irradiation of 3 Gy was given in cases of unrelated transplantation, and 5 mg/kg anti-thymocyte globulin (Thymoglobuline®) was given for human leukocyte antigen-mismatched transplantation. Between January 2009 and March 2016, 42 patients, including 33 with acute myeloid leukemia and 9 with myelodysplastic syndromes, received allogeneic bone marrow transplantation or peripheral blood stem cell transplantation (allo-BMT/PBSCT) with the FLU/BU4/MEL regimen in our institute. The median patient age at transplantation was 46.5 years (range: 20-63 years), and the median follow-up time was 1513.5 days (214-2975 days). At transplantation, 29 (69%) patients were in hematological CR, and 13 (31%) patients were not in CR. Most of the patients received standard prophylaxis for graft-versus-host disease (GVHD) with IV tacrolimus or cyclosporin A and methotrexate. The rate of neutrophil and platelet engraftment was 100% and 95.2% (95% CI: 81.6-98.8%), respectively, and the median time for their engraftment was 19 days (13-30 days) and 25 days (14-108 days), respectively. Among the patients who were examined for chimerism in the whole blood or T-cell fraction at day 28 after transplantation (n=32 in whole blood; n=16 in T-cell fraction), complete donor chimerism was achieved. Febrile neutropenia and severe (≧grade 3) oral/pharyngeal mucositis occurred in more than 80% of the patients. Although sinusoidal obstruction syndrome did not occur, thrombotic microangiopathy (TMA) occurred in 4 (10%) patients. The cumulative incidence of grade II-IVacute GVHD at day +100 was 42.2% (25.0-55.6%), and the cumulative incidence of moderate-to-severe chronic GVHD was 33.0% (14.4-53.0%). At the last follow-up, 28 of 42 patients were alive, and 14 patients died because of infection (n=9; bacterial: 8, viral: 1), relapse (n=2), interstitial pneumonia (n=2), and TMA (n=1). The 1-year OS, disease-free survival (DFS), non-relapse mortality (NRM), and relapse rate (RR) of all patients were 73.7% (57.5-84.5%), 66.7% (50.3-78.7%), 19.0% (8.8-32.2%), and 14.3% (5.7-26.7%), respectively, and the 4-year OS, DFS, NRM, and RR were 66.0% (49.4-78.3%), 59.5% (43.2-72.6%), 19.0% (10.2-32.1%), and 21.4% (10.5-34.9%), respectively. Among patients who were not in CR at allo-HCT, the 4-year OS and DFS were 49.4% (19.7-73.6%) and 38.5% (14.1-62.8%), respectively. These results indicated an improvement in the OS of patients with uncontrolled disease after the addition of MEL to FLU/BU4. In contrast, although the RR among patients who were in CR at allo-HCT was exceedingly low (1-year: 0.0% (0.0-0.0%); 4-year: 3.4% (0.2-15.4%)), the NRM after HCT in this group was relatively high (day +100: 13.8% (4.2-28.9%); 1-year: 24.1% (10.4-40.9%); 4-year: 27.6% (12.8-44.6%)). The most frequent cause of NRM in this group was bacterial infection, which was closely related to the high incidence of mucositis. Given the extremely low RR, the FLU/BU4/MEL regimen can be modified to reduce NRM in such cases. In conclusion, FLU/BU4/MEL had curative potential even for patients with advanced myeloid malignancies, accompanied by the rapid complete chimerism achievement after allo-BMT/PBSCT. The NRM should be minimized to further improve the outcomes. Disclosures Yokota: Bristol-Myers Squibb: Research Funding; Pfizer Inc.: Research Funding; MSD K.K.: Research Funding; CHUGAI PHARMACEUTICAL CO., LTD.: Research Funding; Celgene: Research Funding. Shibayama:Bristol-Meyer Squibb K.K: Honoraria, Research Funding; Novartis Pharma K.K.: Honoraria, Research Funding; Fujimoto Pharmaceutical: Honoraria, Research Funding; Ono Pharmaceutical Co.,LTD: Honoraria, Research Funding; Jansen Pharmaceutical K.K: Honoraria; Mundipharma K.K.: Honoraria, Research Funding; Takeda Pharmaceutical Co.,LTD.: Honoraria, Research Funding; Celgene K.K.: Honoraria, Research Funding. Tomiyama:Sysmex Corporation: Consultancy; Chugai Pharmaceutical Co., Ltd.: Honoraria; Novartis Pharma Co., Ltd.: Honoraria, Membership on an entity's Board of Directors or advisory committees; Kyowa Hakko Kirin Co., Ltd.: Honoraria. Kanakura:Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria, Research Funding.

The human gut harbors various microorganisms, which are important for maintenance of systemic health, including metabolism and immune system homeostasis. Recent studies demonstrated the association of intestinal flora with various diseases such as inflammatory bowel disease and rheumatoid arthritis. In addition, studies on patients with hematopoietic stem cell transplantation (HSCT) showed that the variation in intestinal bacterial flora after transplantation is related to prognosis, frequency of infection, sensitivity to antimicrobial agents, and severity of graft versus host disease. Nonetheless, most studies showing less diversity of intestinal flora evaluated patients' feces a certain period after HSCT. In order to determine the relationship between the alterations in intestinal flora composition and the clinical course post-HSCT, we used metagenomic analysis to evaluate the time-dependent changes in intestinal bacterial flora in patients undergoing allogeneic (allo) HSCT, and compared them with those of healthy controls (HC) or recipients of autologous (auto) peripheral blood HSCT. Patients who received autologous or allogeneic HSCT at the Osaka University Hospital were enrolled after obtaining informed consent. We collected 613 feces samples from 24 patients from August 2016 to May 2017 before and after transplantation. Samples were collected in sterile centrifuge tubes and 16S rRNA deep sequencing was performed using MiSeq (Illumina). In total, 543 specimens were available for analysis. First, we evaluated the diversity of the microbiota in pre-transplantation specimens using Simpson's and Shannon-Wiener index. However, neither method revealed any significant difference between auto/allo-patients and HC despite intensive treatment history of the patients, but showed that the major phyla of gut microbiota contained Bacteroides, Firmicutes, and Fusobacteria in all three groups. Next, we utilized 'uniFrac distance' to evaluate both the diversity in community structure and members. Indeed, this method was useful for evaluating both weighted (quantitative) and unweighted (qualitative) variations of numerous organisms. Using this method, we were able to accurately estimate the abundance of gut microbiota. Interestingly, weighted uniFrac distance, which describes distances in community structure, was significantly wider between allo vs HC and HC vs HC (p < 0.001) and auto vs HC and HC vs HC (p < 0.001). Furthermore, unweighted uniFrac, which describes the distance between community members, was significantly wider between allo vs HC and HC vs HC (p < 0.001), but not between auto vs HC and HC vs HC (p = 0.69). Next, we analyzed the structural components of microbiota using pre-treatment samples. Results showed that the population of beneficial bacteria such as butyric acid-producing bacteria, Coprococcus, Faecalibacterium, and Lachnospiraceae, were significantly reduced in the microflora of pre-transplantation patient samples compared to HC. In addition, sequential chronological analyses revealed that dynamic fluctuation in the intestinal flora was induced in patients by use of antibiotics. We also observed inter-patient variation in the extent of fluctuation; > 50% patients lost flora diversity after transplantation, whereas some patients retained it despite administration of broad spectrum antibiotics. Next, we analyzed whether alteration in flora composition and/or the loss of diversity affected patient survival. Among 24 patients, 16 showed altered flora composition, whereas 8 retained the flora characteristics. Among these 16 cases, the 2-year mortality of patients who had lost flora diversity was 63.6 %, whereas it was 25.0 % for patients who retained the diversity (p < 0.001). In contrast, all 8 patients who retained original flora composition were alive. Hence, we concluded that both alteration in flora composition and loss of diversity are strongly associated with patient survival. In conclusion, commonly used diversity indexes do not completely describe the differences within genera of gut microbiota, and uniFrac analysis is a powerful tool for evaluating gut microbiota in patients with HSCT, as it analyzes community composition/structure in detail. This method can also be used to simultaneously evaluate alterations in flora composition and loss of diversity, both of which affect survival post-HSCT. Disclosures Yokota: Pfizer Inc.: Research Funding; Bristol-Myers Squibb: Research Funding; MSD K.K.: Research Funding; CHUGAI PHARMACEUTICAL CO., LTD.: Research Funding; Celgene: Research Funding. Shibayama:Ono Pharmaceutical Co.,LTD: Honoraria, Research Funding; Celgene K.K.: Honoraria, Research Funding; Bristol-Meyer Squibb K.K: Honoraria, Research Funding; Mundipharma K.K.: Honoraria, Research Funding; Fujimoto Pharmaceutical: Honoraria, Research Funding; Jansen Pharmaceutical K.K: Honoraria; Novartis Pharma K.K.: Honoraria, Research Funding; Takeda Pharmaceutical Co.,LTD.: Honoraria, Research Funding. Kanakura:Alexion Pharmaceuticals, Inc.: Consultancy, Honoraria, Research Funding.

[Background] Mixed chimerism (MC) and/or secondary graft failure (SGF) with recipient or donor-type chimerism is a major obstacle in allogeneic hematopoietic stem cell transplantation for patients with aplastic anemia (AA). Recently, various kinds of conditioning regimen and donor are used in transplantation for AA, and their influences on MC and SGF remain to be elucidated. [Methods] In the Transplant Registry Unified Management Program (TRUMP), which is the registry database of the Japanese Society for Hematopoietic Cell Transplantation, patients with AA aged more than 15 years, who underwent a first allogeneic bone marrow or peripheral blood transplantation between 2000 and 2014 and achieved engraftment were eligible subjects of this study. A survey was conducted to collect the additional data regarding MC and SGF in each patient. [Results] Additional data were collected in 418 patients, and their median age was 30 years (range 16 - 65 years). A median follow-up period for survivors was 1727 days in all patients. MC requiring neither granulocyte-colony stimulating factor (G-CSF) nor transfusion support (Group 1), MC (not SGF) requiring G-CSF or transfusion support (Group 2), SGF with MC or complete recipient-type chimerism (Group 3), and SGF with complete donor-type chimerism (Group 4) developed in 26 (6.2 %), 16 (3.8 %), 19 (4.5 %), 17 (4.1 %) patients, respectively. Overall survival rates (OS) were 90.4 % at 1 year and 83.5 % at 5 years in patients without MC and SGF (Group 0, n = 340), which was not different from those in Group 1 (1yOS: 100.0 %, 5yOS: 94.1 %, P = 1.0) or Group 2 (1yOS: 87.5 %, 5yOS: 60.9 %, P = 1.0). In contrast, OS rates were significantly lower in Group 3 (1yOS: 52.1 %, 5yOS: 52.1 %, P < 0.001) and Group 4 (1yOS: 82.4 %, 5yOS 56.3 %, P = 0.003) as compared to Group 0 (Figure 1). SGF developed later in Group 4 than Group 3 (median days from transplantation: 99 days vs. 55 days, P = 0.04). In multivariate analyses, the use of fludarabine (Flu) (odds ratio: 4.26, P = 0.03) and no use of irradiation (odds ratio: 5.88, P < 0.001) in conditioning regimens were associated with the development of SGF with MC or complete recipient-type chimerism, and the use of Flu in conditioning regimens (odds ratio: 8.68, P = 0.04) was associated with SGF with complete donor-type chimerism. The dose of cyclophosphamide (CY), use of in vivo T-cell depletion in conditioning regimens, prophylaxis of graft-versus-host disease (GVHD), donor type, and stem cell source did not affect the development of SGF with both MC/recipient-type and donor-type chimerism. We further investigated the clinical course of patients with SGF, including the usage of post-transplant immunosuppressants (IS). SGF developed before starting the tapering of IS in 14 out of 19 patients in Group 3, and in 9 out of 17 patients in Group 4 (P = 0.299). After the onset of SGF, increase of IS was effective for SGF in only one patient in Group 3, but on the other hand, in 5 patients in Group 4. Decrease/discontinuation of IS was effective in one patient in Group 3, but none in Group 4. Second transplantation was successfully performed in 11 and 6 patients in Group 3 and 4, respectively. [Conclusion] The occurrence of SGF with both MC/recipient-type and donor-type chimerism after allogeneic transplantation for AA was associated with inferior OS, and conditioning regimens had an impact on the occurrence of SGF. The use of Flu may increase the occurrence of SGF with both MC/recipient-type and donor-type chimerism. When SGF occurs, efficacious intervention may differ according to the type of SGF. Disclosures Kako: Takeda Pharmaceutical Company Limited.: Honoraria; Takeda Pharmaceutical Company Limited.: Honoraria; Celgene K.K.: Honoraria; Bristol-Myers Squibb: Honoraria; Sumitomo Dainippon Pharma Co., Ltd.: Honoraria; Chugai Pharmaceutical Co., Ltd.: Honoraria; Otsuka Pharmaceutical Co., Ltd.: Honoraria; Ono Pharmaceutical Co., Ltd.: Honoraria; Janssen Pharmaceutical K.K.: Honoraria. Kanda:Novartis: Research Funding; Sanofi: Research Funding; Tanabe-Mitsubishi: Research Funding; CSL Behring: Research Funding; Chugai: Consultancy, Honoraria, Research Funding; Taiho: Research Funding; Astellas: Consultancy, Honoraria, Research Funding; Taisho-Toyama: Research Funding; Otsuka: Research Funding; Takeda: Consultancy, Honoraria, Research Funding; Shionogi: Consultancy, Honoraria, Research Funding; Pfizer: Research Funding; Kyowa-Hakko Kirin: Consultancy, Honoraria, Research Funding; Nippon-Shinyaku: Research Funding; MSD: Research Funding; Asahi-Kasei: Research Funding; Ono: Consultancy, Honoraria, Research Funding; Eisai: Consultancy, Honoraria, Research Funding; Dainippon-Sumitomo: Consultancy, Honoraria, Research Funding; Bristol-Myers Squibb: Consultancy, Honoraria; Celgene: Consultancy, Honoraria; Mochida: Consultancy, Honoraria; Alexion: Consultancy, Honoraria; Takara-bio: Consultancy, Honoraria. Ishiyama:Alexion Pharmaceuticals, Inc.: Honoraria. Ichinohe:Zenyaku Kogyo Co.: Research Funding; Alexion Pharmaceuticals: Honoraria; Takeda Pharmaceutical Co.: Research Funding; Sumitomo Dainippon Pharma Co.: Research Funding; Taiho Pharmaceutical Co.: Research Funding; Ono Pharmaceutical Co.: Research Funding; Novartis.: Honoraria; Mundipharma: Honoraria; Janssen Pharmaceutical K.K.: Honoraria; JCR Pharmaceuticals: Honoraria; Otsuka Pharmaceutical Co.: Research Funding; Nippon Shinyaku Co.: Research Funding; MSD: Research Funding; Eisai Co.: Research Funding; CSL Behring: Research Funding; Kyowa Hakko Kirin Co.: Research Funding; Pfizer: Research Funding; Repertoire Genesis Inc.: Research Funding; Bristol-Myers Squibb: Honoraria; Celgene: Honoraria; Chugai Pharmaceutical Co.: Research Funding; Astellas Pharma: Research Funding. Mori:SHIONOGI: Honoraria; Janssen: Honoraria; Pfizer: Honoraria; Celgene: Honoraria; Taisho Toyama Pharmaceutical Co: Honoraria; Asahi Kasei: Research Funding; Astella Pharma: Honoraria; Shire Japan: Honoraria; Ono: Honoraria; MSD: Honoraria; Novartis Pharma: Research Funding; MSD: Research Funding; Novartis Pharma: Honoraria; CHUGAI: Honoraria; Japan Blood Products Organization: Honoraria; Kyowa Hakko Kirin: Honoraria; Eisai: Honoraria.

Summary Bone marrow transplantation (BMT) is one of the promising methods of treatment of hematologic malignancy. However, it has a variety of complications since it involves fatal doses of anti-cancer drugs and radiation during the conditioning period. Among the various complications of BMT, pulmonary hypertension is rare and its pathogenesis is poorly understood. A 35-year-old female with acute myeloid leukemia (AML) presented with pulmonary hypertension after BMT. Although she exhibited severe dyspnea on admission, her general condition markedly improved after oxygen therapy and treatment with warfarin and beraprost sodium. Her pulmonary hypertension was diagnosed as pulmonary arterial hypertension (PAH) related to BMT. Although PAH has only rarely been reported as a complication of BMT, we present here for the first time an adult patient with PAH associated with BMT who exhibited marked improvement with medical treatment. This case indicates that attention needs to be paid to the clinical symptoms and physical findings of PAH as a complication of BMT.

脾臓の炎症性偽腫瘍（inflammatory pseudotumor，以下IPT）はまれな疾患である．特徴的な画像所見がなく，術前診断は困難である．診断的治療として脾臓摘出術が行われることが多い．今回，偶然発見された脾腫瘍に対して腹腔鏡下脾臓摘出術を行い．脾原発IPTと診断した1例を経験したので報告する．症例は48歳，男性．左腎盂尿管移行部狭窄症，尿路結石の診断で当院の泌尿器科に通院中であった．今回，左腎盂尿管移行部狭窄症の精査目的で撮影された造影CTで，脾臓に2cm大の腫瘍を指摘され当科を紹介された．腹腔鏡下脾臓摘出術の適応と考え，手術を行った．悪性疾患も否定できないため，脾臓の皮膜を破らぬよう注意しつつ脾臓摘出術を行った．術後の病理組織診断で脾のIPTと診断した．術中術後経過は極めて良好で，術後6日目に通院した．本邦で腹腔鏡下脾臓摘出術を行ったIPTの報告は本例を含めて19例であった．文献的考察を加えて報告する．

To report the efficacy of intravitreal chemotherapy for primary intraocular lymphoma (PIOL).Retrospective, noncomparative interventional case reports are presented for six patients (ten eyes; mean age, 58.8 years) with pathologically confirmed PIOL who participated in this study and were treated at our hospital with intravitreal injections of methotrexate (MTX) between January 2004 and February 2007. Intravitreal injections of MTX (400 microg MTX/50 microl Opeguard-MA) were administered once or twice weekly for 1 month followed by ten monthly injections. Interleukin-10 (IL-10) and IL-6 were measured in the vitreous before and after injections to determine tumor activity.All eyes were clinically cleared of malignant cells. One eye lost vision. After intravitreal chemotherapy, the vitreous IL-10 concentration reached barely detectable levels.Intravitreal chemotherapy achieves clinical remission and preserves vision in patients with PIOL.

The standard conditioning regimen in allogeneic hematopoietic stem cell transplantation (HSCT) for aplastic anemia from a human leukocyte antigen (HLA)-identical sibling has been high-dose cyclophosphamide (CY 200 mg/kg). In the present study, results for 203 patients with aplastic anemia aged 16 years or older who underwent allogeneic HSCT from HLA-identical siblings were retrospectively analyzed using the registry database of Japan Society for Hematopoietic Cell Transplantation. Conditioning regimens were defined as a (1) high-dose CY (200 mg/kg or greater)-based (n = 117); (2) reduced-dose CY (100 mg/kg or greater, but less than 200 mg/kg)-based (n = 38); and (3) low-dose CY (less than 100 mg/kg)-based (n = 48) regimen. Patient age and the proportion of patients receiving fludarabine were significantly higher in the reduced- and low-dose CY groups than the high-dose CY group. Engraftment was comparable among the groups. Five-year overall survival (OS) tended to be higher in the low-dose CY group [93.0 % (95 % CI 85.1-100.0 %)] than the high-dose CY [84.2 % (95 % CI 77.1-91.3 %)] or reduced-dose CY groups [83.8 % (95 % CI 71.8-95.8 %); P = 0.214]. Age-adjusted OS was higher in the low-dose CY group than the high- and reduced-dose CY groups with borderline significance (P = 0.067). These results suggest that CY dose can safely be reduced without increasing graft rejection by adding fludarabine in allogeneic HSCT for aplastic anemia from an HLA-identical sibling.

Malignant transformation of endometriosis has been extensively described in the literature. However, extragonadal endometrioid adenocarcinoma, either de novo or arising from malignant transformation of endometriosis, is rare. The present case report describes a patient with endometrioid adenocarcinoma on the serous surface of the small intestine. A 25-year-old female with no history of endometriosis was referred to our hospital with an intrapelvic tumor. An internal examination, ultrasound, and magnetic resonance imaging revealed a round mass approximately 80 mm in diameter; however, identification of the affected organ was difficult. Because we could not rule out malignancy based on the non-specific radiologic findings, laparotomy was performed. A mass with ileal adhesions was detected intraoperatively. In addition, the uterus and bilateral adnexa appeared normal. The tumor was resected with part of the ileum. Histopathology confirmed a diagnosis of endometrioid adenocarcinoma originating from the serous surface of the small intestine.

We report the computed tomography (CT) and magnetic resonance imaging (MRI) features of a 10-year-old boy with low-grade fibromyxoid sarcoma (LGFMS) in the shoulder and correlate them with the clinicopathological features. LGFMS, a rare soft tissue sarcoma with bland histological features and paradoxically aggressive behavior, tends to occur in superficial regions and in children more than previously recognized. To date, few imaging studies have been reported, particularly in pediatric patients. In this case, precontrast CT showed a low- to isodensity mass and punctate calcification. On contrast-enhanced CT, heterogeneous enhancement was seen. MRI findings correlated well with the pathological features of the tumor. The myxoid area showed high signal intensity on T2-weighted MR images and heterogeneous enhancement after administration of contrast material; the hypercellular area demonstrated lower signal intensity on T2-weighted MR images and slight enhancement.