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Background Patients presenting with advanced ovarian cancer can be managed in a variety of ways. No clear selection algorithms exist to guide decision-making and there is significant geographical variation in practice. Decision-making takes place in specialist multidisciplinary team meetings (MDTs). We wanted to examine whether format and behaviour within these meetings could explain the geographical variation in treatment patterns seen in England Methods Observational study of five cancer centre MDTs over a six-week period. Data were recorded for overall MDT performance. The GO-MDT-MODe tool was used to provide a measure of participation and quality of case discussion for all cases of advanced ovarian cancer. MDT scores were correlated with surgical and survival data extracted from national audit data. Results A total of 870 case discussions, including 145 cases of advanced ovarian cancer, were observed. MDTs varied in structure, format and time allocation between centres. Cluster analysis showed significant variation in quality and participation of discussion between centres (p

Objective The role of surgery for patients with advanced ovarian cancer is well established but wide variations in the percentage of patients undergoing surgery exist. Design Observational Study Setting The 19 cancer alliances in England, UK Methods We undertook a comparative analysis to examine the effects of rates of surgery on one and five year disease specific survival across the 19 English Cancer Alliances. We also undertook a propensity score analysis of a single cohort of patients with advanced ovarian cancer to compare patients who did, and did not, undergo surgery as part of their primary management. Main outcome measures Correlations between rates of surgery, demographics, and cancer alliance performance against one and five year survival Results In multivariable analysis, rates of surgery undertaken by a Cancer Alliance remained an independent predictor of both one and five year survival (correlation coefficient 0.81, and 0.70, respectively) although this relationship is likely non linear with optimal rates of surgery still to be defined. Using propensity score analysis a subgroup of patients who gain no benefit from surgery can be identified. Conclusions Overall survival of patients with advanced ovarian cancer is likely to be improved with an increase in overall rates of surgery however there are likely to be optimal maximal rates, above which patients will be disadvantaged. Mechanisms for identifying individual outcome predictions are required.

Our study aimed to establish 'real-world' performance and cost-effectiveness of ovarian cancer (OC) surveillance in women with pathogenic germlineOur study recruited 875 femaleOur study identified 8 OCs during 1277 women screen years: 2 occult OCs at RRSO (both stage 1a), and 6 screen-detected; 3 of 6 (50%) were ≤stage 3a and 5 of 6 (83%) were completely surgically cytoreduced. Modelled sensitivity, specificity, Positive Predictive Value (PPV) and Negative Predictive Value (NPV) for OC were 87.5% (95% CI, 47.3 to 99.7), 99.9% (99.9-100), 75% (34.9-96.8) and 99.9% (99.9-100), respectively. The predicted number of quality-adjusted life years (QALY) gained by surveillance was 0.179 with an ICER costOC surveillance for women deferring RRSO in a 'real-world' setting is feasible and demonstrates similar performance to research trials; it down-stages OC, leading to a high complete cytoreduction rate and is cost-saving in the UK National Health Service (NHS) setting. While RRSO remains recommended management, ROCA-based surveillance may be considered for female

Background Neoadjuvant therapy is increasingly used in the first-line setting in people with advanced endometrial cancer despite a paucity of evidence for this approach. Objective To systematically evaluate the literature in this area. Search Strategy Electronic searches of Ovid MEDLINE, Ovid Embase, Clinical trials.gov and the International clinical trials registry platform were performed for studies published between 1990 and 2021 comparing neoadjuvant therapy with upfront debulking surgery in Stage 3 or 4 endometrial cancers. Selection Criteria Studies reporting overall survival, progression free survival, adverse events and/or quality of life in those undergoing neoadjuvant therapy or upfront debulking. Data Collection and Analysis Odds ratios (OR) and log hazard ratios (HR) along with 95% confidence intervals (CI) were calculated and pooled for analysis. Risk of bias was assessed using the ROBINS-I tool. Main Results Eight non-randomised studies with a total of 50,510 patients were identified. These showed that patients undergoing primary chemotherapy had similar survival outcomes to those undergoing primary surgery (HR 1.26 (95% CI 0.95-1.69)). Fewer patients in the neoadjuvant group had surgery but those that did were less likely to be suboptimally cytoreduced (OR 0.24; 95% CI 0.21-0.28). Surgical morbidity was no different between the two approaches (OR 0.51, 95% confidence interval 0.08-3.25). However, the potential for bias in these studies is very high. Conclusion There is significant uncertainty as to whether the outcomes for those undergoing primary cytoreductive surgery or neoadjuvant chemotherapy in the presence of unresectable disease are better. Prospective reporting of outcomes is needed.

PURPOSE: Epithelial ovarian cancer (EOC) is associated with pathogenic variants (PVs) in homologous recombination and/or mismatch repair genes. We aimed to review the testing of women with familial EOC at our center.METHODS: Women with familial EOC (≥2 EOC in family, including index case) referred to our center between 1993 and 2021 were included. Genetic testing (BRCA/Lynch syndrome screening, exome sequencing, panel testing, 100,000 Genome Project, and NIHR BioResource genome sequencing) and clinical demographic, diagnosis, and survival data were reviewed.RESULTS: Of 277, 128 (46.2%) women were BRCA heterozygotes (BRCA1: 89, BRCA2: 39). The detection rate in BRCA-negative women was 21.8%; the most commonly affected gene was BRIP1 (5.9%). The non-BRCA detection rate was significantly higher in families with 2 affected members with EOC only (22.4%) than the families with ≥3 (11.1%) affected members (odds ratio = 9.9, 95% CI = 1.6-105.2, P = .0075). Overall, 112 different PVs in 12 homologous recombination/mismatch repair genes were detected in 150 of 277 (54.2%) unrelated women.CONCLUSION: This is the largest report of women with familial EOC undergoing wider testing to date. One-fifth of BRCA-negative women were heterozygous for a PV in a potentially actionable gene. Wider genetic testing of women with familial EOC is essential to optimize their treatment and prevention of disease in family members.

Objective\ud \ud To investigate quality of life (QoL) and association with surgical complexity and disease burden after surgical resection for advanced ovarian cancer in centres with variation in surgical approach.\ud \ud \ud \ud Design\ud \ud Prospective multicentre observational study.\ud \ud \ud \ud Setting\ud \ud Gynaecological cancer surgery centres in the UK, Kolkata, India, and Melbourne, Australia.\ud \ud \ud \ud Sample\ud \ud Patients undergoing surgical resection (with low, intermediate or high surgical complexity score, SCS) for late-stage ovarian cancer.\ud \ud \ud \ud Main Outcome Measures\ud \ud Primary: change in global score on the European Organisation for Research and Treatment of Cancer (EORTC) core quality-of-life questionnaire (QLQ-C30). Secondary: EORTC ovarian cancer module (OV28), progression-free survival.\ud \ud \ud \ud Results\ud \ud Patients’ preoperative disease burden and SCS varied between centres, confirming differences in surgical ethos. QoL response rates were 90% up to 18 months. Mean change from the pre-surgical baseline in the EORTC QLQ-C30 was 3.4 (SD 1.8, n = 88) in the low, 4.0 (SD 2.1, n = 55) in the intermediate and 4.3 (SD 2.1, n = 52) in the high-SCS group after 6 weeks (p = 0.048), and 4.3 (SD 2.1, n = 51), 5.1 (SD 2.2, n = 41) and 5.1 (SD 2.2, n = 35), respectively, after 12 months (p = 0.133). In a repeated-measures model, there were no clinically or statistically meaningful differences in EORTC QLQ-C30 global scores between the three SCS groups (p = 0.840), but there was a small statistically significant improvement in all groups over time (p < 0.001). The high-SCS group experienced small to moderate decreases in physical (p = 0.004), role (p = 0.016) and emotional (p = 0.001) function at 6 weeks post-surgery, which resolved by 6–12 months.\ud \ud \ud \ud Conclusions\ud \ud The global QoL of patients undergoing low-, intermediate- and high-SCS surgery improved at 12 months after surgery and was no worse in patients undergoing extensive surgery.\ud \ud \ud \ud Tweetable Abstract\ud \ud Compared with surgery of lower complexity, extensive surgery does not result in poorer quality of life in patients with advanced ovarian cancer.

Ovarian cancer survival in the UK lags behind comparable countries. Results from the ongoing National Ovarian Cancer Audit feasibility pilot (OCAFP) show that approximately 1 in 4 women with advanced ovarian cancer (Stage 2, 3, 4 and unstaged cancer) do not receive any anticancer treatment and only 51% in England receive international standard of care treatment, i.e., the combination of surgery and chemotherapy. The audit has also demonstrated wide variation in the percentage of women receiving anticancer treatment for advanced ovarian cancer, be it surgery or chemotherapy across the 19 geographical regions for organisation of cancer delivery (Cancer Alliances). Receipt of treatment also correlates with survival: 5 year Cancer survival varies from 28.6% to 49.6% across England. Here, we take a systems wide approach encompassing both diagnostic pathways and cancer treatment, derived from the whole cohort of women with ovarian cancer to set out recommendations and quality performance indicators (QPI). A multidisciplinary panel established by the British Gynaecological Cancer Society carefully identified QPI against criteria: metrics selected were those easily evaluable nationally using routinely available data and where there was a clear evidence base to support interventions. These QPI will be valuable to other taxpayer funded systems with national data collection mechanisms and are to our knowledge the only population level data derived standards in ovarian cancer. We also identify interventions for Best practice and Research recommendations.

Women testing positive forWomen were prospectively followed up from positive genetic test (GT) result to censor date. χ² testing compared categorical variables; Cox regression model estimated HRs and 95% CI for BC/ovarian cancer cases associated with RRS, and impact on all-cause mortality; Kaplan-Meier curves estimated cumulative RRS uptake. The annual cancer incidence was estimated by women-years at risk.In total, 887 women were included in this analysis. Mean follow-up was 6.26 years (range=0.01-24.3; total=4685.4 women-years). RRS was performed in 512 women, 73 before GT. Overall RRM uptake was 57.9% and RRSO uptake was 78.6%. The median time from GT to RRM was 18.4 months, and from GT to RRSO-10.0 months. Annual BC incidence in the study population was 1.28%. Relative BC risk reduction (RRM versus non-RRM) was 94%. Risk reduction of ovarian cancer (RRSO versus non-RRSO) was 100%.Over a 24-year period, we observed an increasing number of women opting for RRS. We showed that the timing of RRS remains suboptimal, especially in women undergoing RRSO. Both RRM and RRSO showed a significant effect on relevant cancer risk reduction. However, there was no statistically significant RRSO protective effect on BC.

The British Gynecological Cancer Society and the British Association of Gynecological Pathologists established a multidisciplinary consensus group comprising experts in surgical gynecological oncology, medical oncology, genetics, and laboratory science, and clinical nurse specialists to identify the optimal pathways to

Polaris Sensor Technologies reports on the development of Pedestrian Automated System for Enforcement and Safety (PASES), a radar and video based system used to monitor vehicle and pedestrian traffic with the intent of improving pedestrian safety. Data is fused from a system of multiple sensors and multiple sensor modalities to identify vehicular violations of pedestrian right of way. A focus was placed on the selection of low cost COTS sensors to make the system more widely available to state and local DOTs with limited budgets. Applications include automated enforcement, adaptive traffic control, and improved intersection and crosswalk design based on high quality data available for traffic engineering. We discuss early results with high fidelity sensors, and the performance trades made in order to make the system affordable. A discussion of the system processing architecture is included which highlights the treatment of each sensor data type, and the means of combining the processed data products into state information related to traffic incidents involving vehicles and pedestrians.

Polaris Sensor Technologies (PST) has developed a stereo vision upgrade kit for TALON® robot systems comprised of a replacement gripper camera and a replacement mast zoom camera on the robot, and a replacement display in the Operator Control Unit (OCU). Harris Corporation has developed a haptic manipulation upgrade for TALON® robot systems comprised of a replacement arm and gripper and an OCU that provides haptic (force) feedback. PST and Harris have recently collaborated to integrate the 3D vision system with the haptic manipulation system. In multiple studies done at Fort Leonard Wood, Missouri it has been shown that 3D vision and haptics provide more intuitive perception of complicated scenery and improved robot arm control, allowing for improved mission performance and the potential for reduced time on target. This paper discusses the potential benefits of these enhancements to robotic systems used for the domestic homeland security mission.

Polaris Sensor Technologies has developed numerous 3D display systems using a US Army patented approach. These displays have been developed as prototypes for handheld controllers for robotic systems and closed hatch driving, and as part of a TALON robot upgrade for 3D vision, providing depth perception for the operator for improved manipulation and hazard avoidance. In this paper we discuss the prototype rugged 3D laptop computer and its applications to defense missions. The prototype 3D laptop combines full temporal and spatial resolution display with the rugged Amrel laptop computer. The display is viewed through protective passive polarized eyewear, and allows combined 2D and 3D content. Uses include robot tele-operation with live 3D video or synthetically rendered scenery, mission planning and rehearsal, enhanced 3D data interpretation, and simulation.

Improved situational awareness results not only from improved performance of imaging hardware, but also when the operator and human factors are considered. Situational awareness for IR imaging systems frequently depends on the contrast available. A significant improvement in effective contrast for the operator can result when depth perception is added to the display of IR scenes. Depth perception through flat panel 3D displays are now possible due to the number of 3D displays entering the consumer market. Such displays require appropriate and human friendly stereo IR video input in order to be effective in the dynamic military environment. We report on a stereo IR camera that has been developed for integration on to an unmanned ground vehicle (UGV). The camera has auto-convergence capability that significantly reduces ill effects due to image doubling, minimizes focus-convergence mismatch, and eliminates the need for the operator to manually adjust camera properties. Discussion of the size, weight, and power requirements as well as integration onto the robot platform will be given along with description of the stand alone operation.

Polaris Sensor Technologies has developed a Stereovision Upgrade Kit for TALON robot to provide enhanced depth perception to the operator. This kit previously required the TALON Operator Control Unit to be equipped with the optional touchscreen interface to allow for operator control of the camera convergence angle adjustment. This adjustment allowed for optimal camera convergence independent of the distance from the camera to the object being viewed. Polaris has recently improved the performance of the stereo camera by implementing an Automatic Convergence algorithm in a field programmable gate array in the camera assembly. This algorithm uses scene content to automatically adjust the camera convergence angle, freeing the operator to focus on the task rather than adjustment of the vision system. The autoconvergence capability has been demonstrated on both visible zoom cameras and longwave infrared microbolometer stereo pairs.

In this paper, we report on the development of a high definition stereoscopic liquid crystal display for use in a variety of applications. The display technology provides full spatial and temporal resolution on a liquid crystal display panel consisting of 1920×1200 pixels at 60 frames per second. Applications include training, mission rehearsal and planning, and enhanced visualization. Display content can include mixed 2D and 3D data. Source data can be 3D video from cameras, computer generated imagery, or fused data from a variety of sensor modalities. Recent work involving generation of 3D terrain from aerial imagery will be demonstrated. Discussion of the use of this display technology in military and medical industries will be included.

Uncalibrated stereo imagery experimental and analytical results are presented for path planning and navigation. An Army Research and Development Engineering Command micro-size UAV was outfitted with two commercial cameras and flown over varied landscapes. Polaris Sensor Technologies processed the data post flight with an image correspondence algorithm of their own design. Stereo disparity (depth) was computed despite a quick assembly, image blur, intensity saturation, noise and barrel distortion. No camera calibration occurred. Disparity maps were computed at a processing rate of approximately 5 seconds per frame to improve perception. Disparity edges (treeline to ground, voids and plateaus) were successfully observed and confirmed to be properly identified. Despite the success of localizing this disparity edges sensitivity to saturated pixels, lens distortion and defocus were strong enough to overwhelm more subtle features such as the contours of the trees, which should be possible to extract using this algorithm. These factors are being addressed. The stereo data is displayed on a flat panel 3D display well suited for a human machine interface in field applications. Future work will entail extraction of intelligence from acquired data and the overlay of such data on the 3D image as displayed.

In this paper, we report on the use of a 3D vision field upgrade kit for TALON robot consisting of a replacement flat panel stereoscopic display, and multiple stereo camera systems. An assessment of the system's use for robotic driving, manipulation, and surveillance operations was conducted. A replacement display, replacement mast camera with zoom, auto-focus, and variable convergence, and a replacement gripper camera with fixed focus and zoom comprise the upgrade kit. The stereo mast camera allows for improved driving and situational awareness as well as scene survey. The stereo gripper camera allows for improved manipulation in typical TALON missions.

In this paper, we report on the development of a 3D vision field upgrade kit for TALON robot consisting of a replacement flat panel stereoscopic display, and multiple stereo camera systems. An assessment of the system's use for robotic driving, manipulation, and surveillance operations was conducted. The 3D vision system was integrated onto a TALON IV Robot and Operator Control Unit (OCU) such that stock components could be electrically disconnected and removed, and upgrade components coupled directly to the mounting and electrical connections. A replacement display, replacement mast camera with zoom, auto-focus, and variable convergence, and a replacement gripper camera with fixed focus and zoom comprise the upgrade kit. The stereo mast camera allows for improved driving and situational awareness as well as scene survey. The stereo gripper camera allows for improved manipulation in typical TALON missions.

In this paper, we report on the development of a high definition stereoscopic liquid crystal display for use in training applications. The display technology provides full spatial and temporal resolution on a liquid crystal display panel consisting of 1920×1200 pixels at 60 frames per second. Display content can include mixed 2D and 3D data. Source data can be 3D video from cameras, computer generated imagery, or fused data from a variety of sensor modalities. Discussion of the use of this display technology in military and medical industries will be included. Examples of use in simulation and training for robot tele-operation, helicopter landing, surgical procedures, and vehicle repair, as well as for DoD mission rehearsal will be presented.

In September 2009 the Fort Leonard Wood Field Element of the US Army Research Laboratory - Human Research and Engineering Directorate, in conjunction with Polaris Sensor Technologies and Concurrent Technologies Corporation, evaluated the objective performance benefits of Polaris' 3D vision upgrade kit for the TALON small unmanned ground vehicle (SUGV). This upgrade kit is a field-upgradable set of two stereo-cameras and a flat panel display, using only standard hardware, data and electrical connections existing on the TALON robot. Using both the 3D vision system and a standard 2D camera and display, ten active-duty Army Soldiers completed seven scenarios designed to be representative of missions performed by military SUGV operators. Mission time savings (6.5% to 32%) were found for six of the seven scenarios when using the 3D vision system. Operators were not only able to complete tasks quicker but, for six of seven scenarios, made fewer mistakes in their task execution. Subjective Soldier feedback was overwhelmingly in support of pursuing 3D vision systems, such as the one evaluated, for fielding to combat units.

The use of tele-operated Unmanned Ground Vehicles (UGVs) for military uses has grown significantly in recent years with operations in both Iraq and Afghanistan. In both cases the safety of the Soldier or technician performing the mission is improved by the large standoff distances afforded by the use of the UGV, but the full performance capability of the robotic system is not utilized due to insufficient depth perception provided by the standard two dimensional video system, causing the operator to slow the mission to ensure the safety of the UGV given the uncertainty of the perceived scene using 2D. To address this Polaris Sensor Technologies has developed, in a series of developments funded by the Leonard Wood Institute at Ft. Leonard Wood, MO, a prototype Stereo Vision Upgrade (SVU) Kit for the Foster-Miller TALON IV robot which provides the operator with improved depth perception and situational awareness, allowing for shorter mission times and higher success rates. Because there are multiple 2D cameras being replaced by stereo camera systems in the SVU Kit, and because the needs of the camera systems for each phase of a mission vary, there are a number of tradeoffs and design choices that must be made in developing such a system for robotic tele-operation. Additionally, human factors design criteria drive optical parameters of the camera systems which must be matched to the display system being used. The problem space for such an upgrade kit will be defined, and the choices made in the development of this particular SVU Kit will be discussed.

Ante-mortem diagnosed metastatic pericardial effusion in cervical cancer patients is very rare. Symptomatic relief and treatment of the underlying disease offered by a multidisciplinary team, may prolong survival with a good quality of life to these often young patients.

The flow of information among our armed forces is greater than ever and the workload on the warfighter is increasing. A novel, stereo-based 3D display has been developed to aid the warfighter by displaying information in a more intuitive fashion by exploiting depth perception. The flat panel display has a footprint consistent with current and future vehicles, unmanned systems, and aircraft and is capable of displaying analog 3D video and OpenGL 3D imagery. A description of the display will be given along with discussion of the applications evaluated to date.

In this paper, we report on the development of a 3D vision system consisting of a flat panel stereoscopic display and auto-converging stereo camera and an assessment of the system's use for robotic driving, manipulation, and surveillance operations. The 3D vision system was integrated onto a Talon Robot and Operator Control Unit (OCU) such that direct comparisons of the performance of a number of test subjects using 2D and 3D vision systems were possible. A number of representative scenarios were developed to determine which tasks benefited most from the added depth perception and to understand when the 3D vision system hindered understanding of the scene. Two tests were conducted at Fort Leonard Wood, MO with noncommissioned officers ranked Staff Sergeant and Sergeant First Class. The scenarios; the test planning, approach and protocols; the data analysis; and the resulting performance assessment of the 3D vision system are reported.

Pulse Coupled Neural Networks (PCNNs) have been shown to be of value in image processing applications, especially at identifying features of small spatial extent at low signal to noise ratio. In our use of the PCNN, every pixel in a scene feeds a neuron in a fully connected lateral neural network. Nearest neighbor neurons contribute to the output of any given neuron using weights that link the neuron and its neighborhood in both a linear and a non-linear fashion. The network is pulsed, and the output of the network at each pulse is a binary mask of neurons that are active. Pulsing drives the network to evaluate its state. The multi-dimensionality and the non-linear nature of the network make selecting weights using trial and error a non-trivial problem. It is important that the desired features of the input are identified on a predictable pulse, a problem that has yet to be sufficiently addressed by proponents of the PCNN. Our method to overcome these problems is to use a Genetic Algorithm to select the set of PCNN coefficients which will identify the pixels of interest on a predetermined pulse. This method enables PCNNs to be trained, which is a novel capability and renders the method of use for applications.

Using matched filters to find targets in cluttered images is an old idea. Human operators can interactively find threshold values to be applied to the correlation surface that will do a good job of binarizing it into signal/non-signal pixel regions. Automating the thresholding process with nine measured image statistics is the goal of this paper. The nine values are the mean, maximum, and standard deviation of three images: the input image presumed to have some signal, an NxN matched filter kernel in the shape of the signal, and the correlation surface generated by convolving the input image with the matched filter kernel. Several thousand input images with known target locations and reference images were run through a correlator with kernels that resembled the targets. The nine numbers referred to above were calculated in addition to a threshold found with a time consuming brutal algorithm. Multidimensional radial basis functions were associated with each nine number set. The bump height corresponded to the threshold value. The bump location was within a nine dimensional hypercube corresponding to the nine numbers scaled so that all the data fell within the interval 0 to 1 on each axis. The sigma (sharpness of the radial basis function) was calculated as a fraction of the squared distance to the closest neighboring bump. A new threshold is calculated as a weighted sum of all the Gaussian bumps in the vicinity of the input 9D vector. The paper will conclude with a table of results using this method compared to other methods.

This paper details a coiled tubing perforating campaign conducted in New Zealand, during the first half of 2006. Coiled tubing has been used for deploying perforating guns for many years now. The challenge with this type of work, is first designing a coiled tubing string which can push and pull long gun lengths along highly deviated wells. Secondly, a method of retrieving (and deploying) the guns against a high wellhead pressure is required. In this instance, down hole isolation valves could not be used. Instead, a pressure deployment system was utilized. The length of perforating guns run during this campaign is extreme, but not unique1. The use of pressure deployment systems is also not unique2. The combination of these factors along with the facts that this is a very remote operation (with regard to oilfield manufacturing and supply), that much specialized equipment needed to be fabricated (in a market cycle where all equipment deliveries see long lead times), and that the operation must take place in a seismically active area, all combine to make this a very challenging project. This paper lists observations taken from the project that may prove useful to future campaigns of a similar nature.

Abstract 2045 The novel syndrome of Dendritic Cell, Monocyte, B and NK lymphocyte (DCML) deficiency has recently been characterised and found to be caused by mutations in the transcription factor GATA-2 (Dickinson et al. Blood 2011, Hsu et al. Blood 2011). In this report we describe a series of 10 patients and show that there is a high risk of death unless curative haematopoietic stem cell transplantation (HSCT) is performed. Our case series includes 10 patients; 4 sporadic cases and 6 cases from 2 pedigrees showing autosomal dominant inheritance. 7 patients died; of the 3 survivors, 2 were transplanted and show marked resolution of their disease with a follow up of 36 and 11 months, respectively. Causes of death in the patients who died include disseminated mycobacterium avium infection (2) H1N1 influenza (1) candidiasis (1) pulmonary alveolar proteinosis (2) and CMV pneumonitis (1). At least 8 patients had chronic HPV infection and 2 patients had autoimmune phenomena including inflammatory arthritis and erythema nodosum. One patient also developed lymphoma. All patients had monocytopenia and decreased B and NK cells with normal T cells at presentation. Haemoglobin, platelets and neutrophil counts were within normal ranges or mildly reduced. In 4 patients, near absolute dendritic cell (DC) deficiency and elevated Flt-3 ligand was also confirmed. BM aspirate showed dysplastic megakaryocytes and increased fibrosis in some instances. Macrophages were present in dermis, bone marrow and lung, epidermal Langerhans cells were largely preserved and plasma cells were found in inflammatory lesions by immunohistochemistry. GATA-2 mutation was confirmed by Sanger sequencing in all cases. Of the 2 patients treated with transplantation, the first patient presented aged 12 with disseminated BCG sepsis 6 months following BCG vaccination. Skin biopsy showed acid fast bacilli but no well-formed granulomata, and ongoing sepsis failed to respond to antitubercular therapy including addition of IFN gamma. The second patient presented to gynaecology services at the age of 20 with vulval intraepithelial neoplasia stage 3 (VIN3), and then to respiratory physicians a year later with breathlessness, productive cough, weight loss and night sweats; pulmonary alveolar proteinosis was diagnosed on biopsy. She continued to deteriorate despite whole lung lavage, and developed respiratory failure requiring 35% oxygen and nocturnal non-invasive ventilation (NIV). The patient's family history included two generations affected by haematological malignancy or respiratory illness in early adulthood, inherited in a fashion suggesting an autosomal dominant trait. Both patients underwent reduced intensity allogeneic haematopoietic stem cell transplantation with PBMC from unrelated adult donors (patient 1 – 10/12 HLA match; patient 2 – 9/12); transplant conditioning was with Fludarabine 150mg/m2, Melphalan 140mg/m2 and Alemtuzumab 60mg (patient 1), and Fludarabine 150mg/m2, Busulphan 6.4mg/kg, Alemtuzumab 60mg (patient 2). GVHD prophylaxis was with Ciclosporin and Mycophenolate Mofetil. Both transplants were uneventful. 32 months post-transplantation, patient 1 is well and off all medication. The monocyte count, lymphocyte subsets and immunoglobulins are normal and there were good responses to tetanus and HIB vaccinations. Chimerism shows 100% donor myeloid and B cells and 85% donor T cells. 9 months post-transplantation patient 2 has improved significantly and no longer requires oxygen or NIV. Lung function tests and radiology have significantly improved. Monocytes are in the normal range and chimerism shows 100% donor CD15 and 85% donor CD3. Follow-up continues with gynaecology for VIN3 associated with HPV16/18 infection. Neither patient has developed GVHD. Here we show that DCML deficiency caused by GATA-2 mutation incurs a high risk of mortality due to infection or respiratory failure unless treated by HSCT. Rapid correction of both immunodeficiency and pulmonary alveolar proteinosis is possible, even in the context of severe infection or respiratory failure, using reduced intensity conditioning. The absence of GVHD in both these cases, despite significant HLA-mismatching, may reflect the absence of recipient DC at transplantation. Disclosures: No relevant conflicts of interest to declare.

To investigate whether the introduction of liquid-based cytology (LBC) in an urban setting decreases the diagnosis of glandular neoplasia (grade 6) and improves the positive predictive value (PPV) of cervical cytological screening.A retrospective database review was conducted identifying women with cervical cytological abnormalities including glandular neoplasia (grade 6) before and after the introduction of LBC.Following the introduction of LBC the rate of glandular neoplasia (grade 6) referrals fell from 1.08% to 0.69% of all cervical cytological abnormalities. There was a significant reduction in 'abnormal' cytological samples subsequently found to be associated with no invasive or preinvasive disease but no decrease in the number showing preinvasive or invasive disease. A significant decrease in number of patients having a final diagnosis of normal/inflammatory or wart changes was seen in those patients referred during the LBC period (p0.01).The introduction of LBC in an urban setting decreased cytological glandular neoplasia referrals but not at the expense of missing preinvasive and invasive cancers. It has also increased the PPV of cervical sampling to detect preinvasive and invasive cancer from 59.6% to 76.0%.