Characteristics and outcomes of diffuse large B-cell lymphoma presenting in leukaemic phase

Diffuse large B‐cell lymphoma (DLBCL) occasionally presents with circulating malignant cells. The clinical characteristics and long‐term outcomes of these patients have not been described. Twenty‐nine newly diagnosed DLBCL presenting in leukaemic phase were identified between 1996 and 2010, at two institutions. Median age was 48 years, and patients presented with leucocytosis, high lactate dehydrogenase levels, B symptoms, and high International Prognostic Index score. Extra nodal site involvement was observed in all patients and affected the bone marrow (100%), spleen (62%), pleura/lung (41%), liver (21%), bone (17%), bowels (7%) and cerebrospinal fluid (14%). Blood lymphomatous cells co‐expressed CD19, CD20, CD22, CD38, CD45, HLA‐DR and FMC7 in >90%, and kappa or lambda light chain restriction in >50%. Ninety per cent received rituximab and anthracycline‐based chemotherapy. Overall, remission was complete in 54% and partial in 31%; 15% had resistant disease. Median follow‐up was 47 months; 13 (45%) patients remain alive in complete remission. Median progression‐free and overall survivals were 11·5 and 46·7 months, respectively. In summary, patients with DLBCL in leukaemic phase present with high tumour burden and frequent involvement of extra nodal sites. In this uncommon DLBCL subgroup, anthracycline‐based regimens with rituximab are associated with early morbidity and mortality, but yield approximately 50% 4‐year survival.     

A young-onset frontal dementia with dramatic calcifications due to a novel CSF1R mutation

ABSTRACT

Neuroimaging and genomic analysis greatly aid in the identification of young-onset dementia antemortem. We present the case of a 33-year-old female with a 2-year rapid decline to dementia and immobility marked by personality change, executive deficits including compulsions, attention deficit, apraxia, Parkinsonism, and pyramidal signs. She had unique and dramatic calcifications and confluent white matter changes on imaging and was found to have a novel mutation in the colony stimulating factor 1 receptor gene causing adult-onset leukoencephalopathy with axonal spheroids and pigmented glia (ALSP). Here, we review ALSP and briefly discuss differential diagnoses.     

Lower gastrointestinal emergencies and appendicitis

There is a general trend towards increasing numbers of emergency surgical admissions and a quarter of these are colonic. There are common themes in early management, resuscitation and investigation. The best outcomes are achieved with active conservative management, aggressive resuscitation and repeated assessment to measure response. If surgical intervention is required, preoperative investigation, to establish an accurate diagnosis, will ensure that appropriately skilled clinicians are involved in an expedient fashion to achieve the best outcomes.     

Streptococcus gordonii Peritonitis in a Patient on CAPD

We report the first case of Streptococcus gordonii-related continuous ambulatory peritoneal dialysis (CAPD) peritonitis. He is a 69-year-old man with end-stage renal failure due to chron ic glomerulonephritis who had been put on CAPD for 1 year. He was successfully treated with a 2-week course of cefazolin. This case highlights the emerging threat that S. gordonii can be the source of infection in patients on CAPD.     

Innovation and Change in Medical Technology: Interactions Between Physicians and Engineers

Intraperitoneal adhesions following surgical procedures cause considerable morbidity. Hyaluronic acid/carboxymethylcellulose (HA/CMC) films have been shown to be effective agents in decreasing adhesion formation. However, when there is an inadvertent leak of bowel contents into the peritoneum due to incomplete anastomosis, adhesion formation about a defect in order to prevent further leakage and to promote healing of the wound is important for the prevention of morbidity and mortality. The purpose of this study was to determine if an antiadhesion film (HA/CMC) impairs these potentially beneficial adhesions to bowel anastomoses, thus predisposing them to enteric leaks with subsequent peritonitis. Sixty-four rabbits were divided in two groups, each undergoing a complete or partial (90% anastomosis to simulate anastomotic leak) large bowel anastomosis. Half of each of the above groups were treated by wrapping a HNCMC film over the anastomosis and the other half were untreated controls. These two subgroups were then further divided equally and sacrificed at either 7 or 14 days for evaluation of anastomosis integrity and strength. The average anastomtic bursting pressures did not change significantly between those groups treated with HMCMC when compared to untreated controls at 7 or 14 days or in the complete or partial anastomosis group (Student's t test). Adhesion formation to the anastomosis was not impaired in either group independent of HAKMC film application. This study suggests that while HA/CMC film has been shown to decrease adhesions in other models, healing of u rabbit colonic unastomosis even in the presence of an anastomotic defect takes place, further suggesting that the stimulus for adhesion formation can overcome the antiadhesion properties of HA/CMC. Therefore, HA/CMC is a potentially safe adjuvant for preventing postoperative intraabdominal adhesions.     

Postsynaptic muscarinic m2 receptors at cholinergic and glutamatergic synapses of mouse brainstem motoneurons

In many brain areas, few cholinergic synapses are identified. Acetylcholine is released into the extracellular space and acts through diffuse transmission. Motoneurons, however, are contacted by numerous cholinergic terminals, indicating synaptic cholinergic transmission on them. The muscarinic m2 receptor is the major acetylcholine receptor subtype of motoneurons; therefore, we analyzed the localization of the m2 receptor in correlation with synapses by electron microscopic immunohistochemistry in the mouse trigeminal, facial, and hypoglossal motor nuclei. In all nuclei, m2 receptors were localized at the membrane of motoneuronal perikarya and dendrites. The m2 receptors were concentrated at cholinergic synapses located on the perikarya and most proximal dendrites. However, m2 receptors at cholinergic synapses represented only a minority (<10%) of surface m2 receptors. The m2 receptors were also enriched at glutamatergic synapses in both motoneuronal perikarya and dendrites. A relatively large proportion (20–30%) of plasma membrane–associated m2 receptors were located at glutamatergic synapses. In conclusion, the effect of acetylcholine on motoneuron populations might be mediated through a synaptic as well as diffuse type of transmission. J. Comp. Neurol. 521:2008–2024, 2013. © 2012 Wiley Periodicals, Inc.     

Peritonitis in children with automated peritoneal dialysis: a single-center study of a 10-year experience

Peritoneal dialysis (PD) constitutes the preferred dialysis modality for children requiring renal replacement therapy with peritonitis being one of the most common complications of PD. This study was performed to evaluate the epidemiology, microbiology, and outcomes of PD-associated peritonitis in Greek children for a 10-year period. A total of 27 patients (16 males) with a mean age 121.8?±?57.2 months were retrospective analyzed. Patients were on PD therapy for a mean duration of 45.2?±?26.1 months. We found 23 episodes of PD-associated peritonitis occurred in 9 out of 27 patients (0.23 episodes/patient-year), with four patients experienced two or more peritonitis episodes. Gram-positive bacteria were responsible for 15 (65.2%) peritonitis episodes, with Staphylococcus aureus being the predominant specie isolated in 30.4% of cases. A total of seven episodes of exit-site infections (ESIs) were identified in five patients (0.069 episodes/patient-year) with the most common bacteria isolated being S. aureus (57.4%). Initial antibiotic treatment included intraperitoneal vancomycin plus ceftazidime in the majority of cases (82.6%). At the end of study, 12 (44.4%) patients remained on PD, 11 (41.8%) underwent renal transplantation, 2 (7.4%) shifted to hemodialysis and unfortunately, two patients (7.4%) died. Conclusively, our study revealed a noticeable low peritonitis and ESIs rate as compared to international data and represents the first evaluation of the characteristics and outcomes of peritonitis in the Greek pediatric PD population.     

277例弥漫性实质性肺疾病住院患者死亡危险因素分析

目的 探讨277例弥漫性实质性肺疾病住院患者死亡的影响因素.方法 本院呼吸内科弥漫性实质性肺疾病(DPLD)的病例依据病因分为结缔组织疾病相关DPLD组(CTD-IP)、其他已知病因DPLD组和特发性间质性肺炎(IIPS)组,每组按预后情况分又为非死亡组和死亡组两个亚组,采用病例对照的研究方法进行风险因素回顾性分析.结果 IIPS组死亡亚组年龄平均、基础疾病患病率及CRP、ALT、AST指标高于非死亡组,CTD-IP组死亡亚组的CRP、WBC、AST指标高于非死亡组,其他病因组两亚组的病因构成提示有统计学差异,其死亡亚组CRP、PT、APTT、ALT、AST高于非死亡组.将上述各组指标进行曲线分析,AUC大于0.7指标作为死亡风险预测指标,并计算出其截断点作为最佳诊断阈值纳入多因素logistics回归分析.结果显示IIPS组中CRP ＞40(mg/L)和年龄＞70(岁)、CTD-IP中CRP ＞40(mg/L)、其他组中CRP＞ 50(mg/L)为独立死亡危险因素.结论 CRP升高、高龄及药物继发性DPLD是弥漫性实质性肺疾病患者住院死亡的危险因素,其中前者是DPLD患者的共同危险因素,而后两者分别是IIPS组和其他病因组的独立危险因素.上述因素在用于预测不同类型DPLD患者死亡风险中需区别分析.     

自发性细菌性腹膜炎易感因素分析

目的分析自发性细菌性腹膜炎 (SBP)的易感因素 ,选择预防性抗菌治疗对象。方法将 30 7例肝硬变腹水患者按有无胃镜检查、嗜酒、Child Pugh分级、消化道出血、SBP病史等因素 5次分组 ,比较各组SBP发生率。 结果嗜酒者SBP发生率 2 5 .0 % ,非嗜酒者为 1 4 .6 % ;Child PughC级SBP发生率为 2 4 .0 % ,Child PughB级为 1 2 .4% ;消化道出血者SBP发生率为 2 7.5 % ,无消化道出血者为7.9% ;有SBP病史者发生率 66 .7% ,无SBP病史者为 1 0 .9%。其发生率均具有显著性差异。而有或无胃镜检查 ,其SBP发生率无显著差异。结论嗜酒、消化道出血、SBP病史以及Child Pugh分级作为SBP易感因素可以预测SBP的发生 ,采用选择性肠道去污染等办法可以降低SBP的发生率 ,改善预后。