Sézary综合征1例报告及文献复习

目的归纳、分析S啨zary综合征的临床表现特征,提高对该病的诊疗水平。方法报告1例S啨zary综合征患者,并复习相关文献。结果患者男59岁,以全身皮肤潮红伴瘙痒为主要特征,外周血淋巴细胞达171.9×109/L,骨髓中异常淋巴细胞达33.5%,经大剂量糖皮质激素等治疗后缓解。1994年至今国内共报道11例S啨zary综合征患者,男性10例占90.9%,平均年龄56.9±19.1岁,以红皮病、剧痒为显著皮肤表现。结论S啨zary综合征是一种较罕见的T细胞淋巴瘤,多见于老年男性,早期皮肤损害显著但不典型,易被误诊为神经性皮炎、湿疹等。     

Impact of Adapted Physical Activity on Joint Pain Induced by Hormonotherapy in Adjuvant Breast Cancer Treatment: APAISE Study Protocol<br/>Impact de l’Activité Physique Adaptée sur les Douleurs Articulaires Induites sous Hormonothérapie en Traitement Adjuvant du Cancer : Protocole de l’Etude APAISE

Breast cancer is the most common cancer in women and approximately 80% of patients will receive hormone therapy. If survival rate after breast cancer patients is the most important, their treatment, induces strong side effects on quality of life, including joint pain which is encountered by one woman in two. These joint pains are likely to reduce compliance with the treatment and consequently impact survival. In this context, this work aims to evaluate the potential benefit of adapted physical activity to relieve pain and its impact on daily functions. The APAISE protocol, described in this article, is a prospective observational study carried out in women operated on for breast cancer and suffering from pain induced by hormone therapy (approximately 50 women followed up in 2 living areas). The main objective is to evaluate the evolution of pain in the context of an adapted physical activity supervised by a sports educator over a period of 3 months. In addition to pain and its interference, the other parameters studied are quality of life, therapeutic compliance and the level of physical activity. All these indicators will be measured at inclusion, at 3 months and at 6 months. In addition to an expected benefit on the well-being of patients, the results should shed light on the links between physical exercise and pain and in the long term reinforce recommendations in terms of physical activity in breast cancer patients.

Résumé
Le cancer du sein est le cancer le plus fréquent chez la femme, l’un de ceux qui présente le meilleur taux de survie, et pour lequel environ 80% des patientes recevront une hormonothérapie. Si les patientes présentent un pronostic de survie favorable, leur traitement hormonal induit des effets indésirables impactant sur la qualité de vie, parmi lesquels les douleurs articulaires rencontrées chez une femme sur deux. Ces douleurs articulaires sont susceptibles de diminuer l’observance au traitement et par conséquent d’impacter la survie. Dans ce contexte, ce travail vise à évaluer le bénéfice que pourrait présenter l’activité physique adaptée pour soulager ces douleurs et leur retentissement. Le protocole APAISE, décrit dans cet article, est une étude observationnelle prospective menée chez des femmes opérées d’un cancer du sein présentant des douleurs induites par hormonothérapie (environ 50 patientes suivies dans 2 bassins de vie). L’objectif principal est d’évaluer l’évolution des douleurs dans le cadre d’une activité physique adaptée encadrée par un éducateur sportif sur une durée de 3 mois. Outre la douleur et son retentissement, les autres paramètres étudiés sont la qualité de vie, l’observance thérapeutique et le niveau d’activité physique. Tous ces indicateurs seront mesurés, à l’inclusion, à 3 mois et à 6 mois. Outre un bénéfice attendu sur le bien-être des patientes, l’étude vise à éclairer les liens entre l’exercice physique et la douleur et à long terme de renforcer les recommandations et préconisations en matière d’activité physique chez les femmes traitées pour un cancer du sein.     

An ovarian mucinous borderline tumour with mixed mural nodules

The occurrence of mural nodules in serous or mucinous ovarian tumours is not frequent. Mural nodule can be developed in benign, borderline or malignant tumours. They can be benign, malignant or mixed type. Thus the prognosis of the ovarian tumour can be dramatically modified by the presence if these nodules. Eighty-two cases of mural nodules were reported in the literature, among which we account four cases of mixed nodules type. We report an additional case of mixed type mural nodules of anaplastic carcinoma and sarcoma-like developed in an ovarian mucinous borderline tumour at a 60-year-old woman.We give details about the classification, the differential diagnosis and prognosis of theses nodules.     

Conservative treatment of upper urinary tract tumours

The conservative management of kidney cancer is widely accepted as a therapeutic option for tumours measuring less than 4 cm or in case of underlying renal disease and solitary kidney. The functional and carcinologic success of this conservative treatment results from a radical resection of the tumour and a careful repair of the collecting system and selective ligature of the vessels. Kidney artery clamping is a key to reach these objectives. The cooling of the kidney preserves from warm ischemia and reperfusion lesions. In this review, we explain the physiological basis of warm ischemia induced kidney lesions due to the kidney artery clamping and the advantage of hypothermia. The surgical technique as described by Novick is detailed. This well standardized technique has the advantage of being reproducible and adaptable to all situations.     

Prise en charge d’un paragangliome rétropéritonéal

Paragangliomas are rare tumors arising from extraadrenal chromaffin cells. We report a 43-year-old man who presented with abdominal pain. An abdominal computed tomography scan revealed a large retroperitoneal mass. During an endoscopic biopsy of this tumor, the patient experienced marked hemodynamic fluctuations with tachycardia and high blood pressure, and an extraadrenal pheochromocytoma was suspected. Measurements of plasma and urinary catecholamines and urinary total metanephrines ruled in the diagnosis. Echocardiography disclosed acute myocardial dysfunction that returned to normal after surgical resection of the paraganglioma. This report also underlines the importance of the anesthetic preparation and monitoring around the surgical procedure and the need of a long-term follow-up to detect malignant paraganglioma in the absence of histological criteria of benign tumor.     

Late mediastinal metastasis of Ewing's sarcoma of tibia

Peripheral neuroectodermal tumors constitute a large spectrum of small round cell tumors among which Ewing's sarcoma is the most undifferentiated type. They are rare tumors which often concern young people and generally occur in bone tissue. Their metastatic potential is high, generally early, and rarely after five years. We report the case of a 45-year-old woman who presented a mediastinal metastasis eight years after the primitive tumor of tibia. The diagnosis was made on small core biopsies obtained by CT ponction, and was based on morphologic analysis, immunohistochemistry, and confirmed by molecular biology. The presence of metastasis is the main prognostic factor. Despite therapeutic progress, the global survival rate of metastatic patients is still poor.     

卡介苗中PE和PPE家族蛋白B细胞抗原表位多态性研究

目的 了解13株卡介苗(bacille calmette-gurin, BCG)基因组中由PE/PPE基因家族编码的B细胞抗原表位分布情况, 分析其对BCG免疫保护力的潜在影响。方法 从国际免疫表位数据库(Immune Epitope Data base, IEDB)中检索结核分枝杆菌B细胞抗原表位, 与结核分枝杆菌参考菌株H37Rv的蛋白质组进行序列比对, 确定PE/PPE基因家族编码B细胞抗原表位序列;并与13株BCG基因组进行序列比对, 提取表位编码序列, 分析其在BCG中的分布与变异。结果 6个PE/PPE基因家族的基因编码28个B细胞抗原表位, 21个B细胞抗原表位在13株BCG中高度保守, 7个B细胞抗原表位在BCG中发生基因变异, 变异表位分别产生于BCG的不同形成期。结论 PE/PPE基因家族所表达的蛋白大多为细菌表面蛋白, PE/PPE蛋白家族中B细胞抗原表位的变化可能对BCG的保护力产生影响, 应继续开展基于B细胞表位的BCG遗传特征研究。     

Performance of the 4-mg intravenous dexamethasone suppression test in differentiating Cushing disease from pseudo-Cushing syndrome

ContextDiscriminating Cushing disease (CD) from pseudo-Cushing syndrome (PCS) is a challenging task that may be overcome with the 4-mg intravenous (IV) dexamethasone suppression test (DST).ObjectiveAssess the performance of the 4-mg IV DST in the differential diagnosis between CD and PCS in well-characterized patients.DesignRetrospective comparative study of subjects seen in a tertiary care unit (November 2008 to July 2011).MethodsThirty-six patients with PCS and 32 patients with CD underwent 4-mg IV dexamethasone infusions from 11 am to 3 pm. Areas Under ROC Curves (AUCs) were estimated and compared for ACTH and cortisol measured at 4 pm the same day (day 1) and 8 am the next day (day 2). The ROC curve of the marker with the highest AUC was used to determine the threshold with the highest specificity for 100% sensitivity.ResultsThe AUC of ACTH at 8 am on day 2 was estimated at 98.4% (95% CI: [92.1–100]), which is significantly greater than that of ACTH at 4 pm on day 1 (P = 0.04) and that of cortisol at 8 am on day 2 (P = 0.05). For ACTH at 8 am on day 2, the threshold with the highest specificity for 100% sensitivity was estimated at 14.8 ng/L. At this threshold, the sensitivity was estimated at 100% [89–100] and the specificity at 83.3% [67–94].ConclusionThe 4-mg IV DST is an easy and accurate tool in distinguishing CD from PCS. It deserves thus a better place in establishing the diagnosis of CD.     

Glomus tumor of the finger pulp: an unusual pediatric case

Glomus tumor is a rare benign tumor. Diagnosis is often delayed because of the absence of specific symptoms and confirmation can only be made by histological study. Treatment is always surgical. We report a clinical case of glomus tumor of the thumb pulp in a 6-year-old girl, and we discuss clinical, radiological and histological aspects of this tumor.