BACKGROUND & AIMS: BACKGROUND:We proposed diagnostic criteria for immune thrombocytopenic purpura (ITP) by modifying the existing guidelines for diagnosis of ITP and by incorporating laboratory tests found useful for predicting its diagnosis, for example erythrocyte count, leukocyte count, anti-GPIIb/IIIa antibody-producing B cells, platelet-associated anti-GPIIb/IIIa antibodies, percentage of reticulated platelets, and plasma thrombopoietin. OBJECTIVE AND METHODS:To validate our criteria, we conducted a multi-center prospective study involving 112 patients with thrombocytopenia and a morphologically normal peripheral blood film at the first visit. Each patient underwent a physical examination, routine laboratory tests, and specialized tests for the anti-GPIIb/IIIa antibody response and platelet turnover. RESULTS:Ninety-one patients (81%) satisfied the proposed criteria at first visit. Clinical diagnosis was made by skilled hematologists > 6 months after the first visit; ITP was diagnosed in 88 patients and non-ITP disorders in 24. The proposed criteria had 98% sensitivity, 79% specificity, a 95% positive predictive value, and a 90% negative predictive value. A relatively low specificity appears to be attributed to a few patients who had both ITP and aplastic anemia or myelodysplastic syndrome. CONCLUSIONS:Our preliminary diagnostic criteria based on ITP-associated laboratory findings were useful for the differential diagnosis of ITP, but additional evaluations and modifications will be necessary to develop criteria that can be used routinely.

背景与目标：

BACKGROUND & AIMS: OBJECTIVE:Presentation of a case illustrating the benefits of traditional Chinese herbal granules for treatment of immune thrombocytopenic purpura in children. CLINICAL FEATURES:A 4-year-old girl presented with persistent immune thrombocytopenic purpura refractory to the first-line conventional treatment of steroids and intravenous immunoglobulin over 7 months. She was brought to the traditional Chinese medical clinic at the Chang Gung Memorial Hospital in 2011 for alternative therapy. She received a modified Chinese herbal formula, Zi-Ying-Jiang-Huo-Tang (Phellodendri Combination), for 6 months and was followed clinically by both a pediatrician and a traditional Chinese medical doctor. The patient had a dramatic improvement in platelet count and entered complete remission after treatment with the traditional Chinese medicine. There was no recurrence of disease or side effects of treatment noted during the 12-month follow-up period. CONCLUSIONS:Our case report suggests that collaborative monitoring of treatments with traditional Chinese medicine may prove beneficial in the management of childhood persistent immune thrombocytopenic purpura. A larger clinical study is warranted for further evaluation of the role of Zi-Ying-Jiang-Huo-Tang in treating immune thrombocytopenic purpura.

背景与目标：

BACKGROUND & AIMS: :Henoch-Schönlein purpura (HSP) is generally a self-limited vasculitis disease and has a good prognosis. We report a 4-year-old Thai boy who presented with palpable purpura, abdominal colicky pain, seizure, and eventually developed intestinal ischemia and perforation despite adequate treatment, including corticosteroid and intravenous immunoglobulin therapy. Imaging modalities, including ultrasonography and contrast-enhanced computed tomography, could not detect intestinal ischemia prior to perforation. In this patient, we also postulated that vasculitis-induced mucosal ischemia was a cause of the ulcer, leading to intestinal perforation, and high-dose corticosteroid could have been a contributing factor since the histopathology revealed depletion of lymphoid follicles. Intestinal perforation in HSP is rare, but life-threatening. Close monitoring and thorough clinical evaluation are essential to detect bowel ischemia before perforation, particularly in HSP patients who have hematochezia, persistent localized abdominal tenderness and guarding. In highly suspicious cases, exploratory laparotomy may be needed for the definite diagnosis and prevention of further complications.

背景与目标： : 过敏性紫癜 (HSP) 通常是一种自限性血管炎疾病，预后良好。我们报告了一名4岁的泰国男孩，尽管接受了适当的治疗 (包括皮质类固醇和静脉内免疫球蛋白治疗)，但仍出现明显的紫癜，腹部绞痛，癫痫发作，并最终发展为肠缺血和穿孔。成像方式，包括超声检查和对比增强计算机断层扫描，无法在穿孔前检测到肠缺血。在该患者中，我们还推测血管炎诱导的粘膜缺血是溃疡的原因，导致肠穿孔，并且由于组织病理学显示淋巴滤泡耗竭，大剂量皮质类固醇可能是促成因素。HSP的肠穿孔很少见，但会危及生命。密切监测和全面的临床评估对于穿孔前发现肠缺血至关重要，尤其是在患有便血，持续性局部腹部压痛和监护的HSP患者中。在高度可疑的情况下，可能需要进行探查性剖腹手术以明确诊断并预防进一步的并发症。

BACKGROUND & AIMS: :Thrombotic thrombocytopenic purpura (TTP) is a rare but potentially fatal disease in childhood. The association of microangiopathic hemolytic anemia, schistocytes, and thrombocytopenia without fever, neurologic, and renal involvement is sufficient to suspect TTP at an early stage for prompt plasma infusion or exchange therapy. TTP has been increasingly described especially in association with systemic lupus erythematosus (SLE). We report the youngest Chinese boy who presented his SLE with TTP and subsequently experienced 9 relapses of TTP in a 2-year period. SLE disease activity index was low during his TTP relapses and therefore alertness of TTP relapse is required even in a relatively inactive period of SLE. TTP should be recognized even without renal or neurologic features and can respond to plasma therapy.

背景与目标： 血栓性血小板减少性紫癜 (TTP) 是儿童时期罕见但可能致命的疾病。微血管性溶血性贫血，血吸细胞和血小板减少症而没有发热，神经和肾脏受累的关联足以在早期怀疑TTP，以迅速进行血浆输注或交换治疗。TTP已被越来越多地描述，特别是与系统性红斑狼疮 (SLE) 有关。我们报告了最年轻的中国男孩，他的SLE患有TTP，随后在2年内经历了9次TTP复发。在他的TTP复发期间，SLE疾病活动指数较低，因此即使在SLE相对不活跃的时期，也需要对TTP复发保持警惕。即使没有肾脏或神经系统特征，TTP也应被识别，并且可以对血浆治疗产生反应。

BACKGROUND & AIMS: :Idiopathic thrombocytopenic purpura (ITP) is a bleeding disorder in patients who are otherwise healthy and present with thrombocytopenia with normal red cells and leukocytes. ITP is a diagnosis of exclusion and is in origin heterogeneous. The unknown etiology and the lack of clinical data from controlled prospective studies are reasons for controversies in diagnosis and management. Study endpoints traditionally include the velocity of platelet increase after drug intervention or observation, although a rapid elevation of the platelet count is of questionable clinical value. Evaluation of other endpoints is needed.

背景与目标： : 特发性血小板减少性紫癜 (ITP) 是健康且伴有正常红细胞和白细胞的血小板减少症患者的出血性疾病。ITP是排除的诊断，并且起源于异质性。未知的病因和缺乏对照前瞻性研究的临床数据是诊断和治疗争议的原因。传统上，研究终点包括药物干预或观察后血小板增加的速度，尽管血小板计数的快速升高具有可疑的临床价值。需要评估其他端点。

BACKGROUND & AIMS: :Bleeding and isolated thrombocytopenia in idiopathic thrombocytopenic purpura (ITP) are phenomena of a transient or long-term disturbed immune response. Since 1980, ITP has become a model for targeted therapeutic immunomodulation with still unclear mechanisms of action. The systematic analysis of ITP aims to determine more clearly defined patient subgroups for targeted therapy and to explore the complex immune disturbances in this autoimmune disorder.

背景与目标： : 特发性血小板减少性紫癜 (ITP) 中的出血和孤立的血小板减少症是短暂或长期受干扰的免疫反应的现象。自1980以来，ITP已成为靶向治疗性免疫调节的模型，其作用机制仍不清楚。ITP的系统分析旨在确定更明确定义的靶向治疗患者亚组，并探索这种自身免疫性疾病中的复杂免疫紊乱。

BACKGROUND & AIMS: :The role of pulsed high-dose dexamethasone (DXM) in the treatment of patients with chronic idiopathic thrombocytopenic purpura (ITP) is still uncertain. Following an early report in which it was described as an effective and well-tolerated treatment with a sustained platelet response in 100% of cases, a number of subsequent studies have failed to confirm such favorable results. As all these studies were conducted on small numbers of patients, we investigated further the effectiveness and side effects of this therapeutic modality in a larger cohort. Thirty-two patients with chronic ITP were scheduled to receive six monthly courses of intravenous DXM at the dose of 40 mg/day for 4 consecutive days. All patients had ITP that had been resistant to between two and five different therapeutic regimens, including 9 patients who had already failed splenectomy. All patients had to be seen 2 weeks after each cycle to asses their response as well as secondary effects. Three patients failed to respond and clinically required other therapy. Thirteen patients (41%) had a partial (platelet count between 50 and 100 x 10(9)/liter) or complete (platelet count >100 x 10(9)/liter) response to treatment, responses being mostly transient. Responses were observed early during the course of treatment, usually right after the first cycle of DXM. There were no late responses. Side effects were mild and did not require discontinuation of treatment. No clinical or laboratory parameter was found to predict treatment outcome. We conclude that high-dose DXM has a limited effect in patients with chronic ITP. Novel approaches and controlled multicenter trials may help identify new therapeutic strategies for this disease.

背景与目标： : 脉冲大剂量地塞米松 (DXM) 在慢性特发性血小板减少性紫癜 (ITP) 患者治疗中的作用仍不确定。在早期报告中，它被描述为一种有效且耐受性良好的治疗方法，在100% 情况下具有持续的血小板反应，随后的许多研究未能证实这种有利的结果。由于所有这些研究都是针对少量患者进行的，因此我们在更大的队列中进一步调查了这种治疗方式的有效性和副作用。计划将32例慢性ITP患者以40 mg/天的剂量连续4天每月接受六个疗程的静脉注射DXM。所有患者的ITP均对两种至五种不同的治疗方案耐药，其中包括9例脾切除术失败的患者。所有患者必须在每个周期后2周观察，以评估他们的反应以及继发作用。三名患者无反应，临床上需要其他治疗。13名患者 (41%) 对治疗有部分 (血小板计数在50和100 × 10(9)/升之间) 或完全 (血小板计数> 100 × 10(9)/升) 反应，反应大多是短暂的。在治疗过程的早期观察到反应，通常是在DXM的第一个周期之后。没有较晚的回应。副作用轻微，不需要停止治疗。未发现临床或实验室参数可预测治疗结果。我们得出的结论是，大剂量DXM对慢性ITP患者的作用有限。新的方法和对照的多中心试验可能有助于确定该疾病的新治疗策略。

BACKGROUND & AIMS: :Etanercept is a recombinant dimeric fusion protein consisting of a tumor necrosis factor-alpha receptor ligand-binding region linked to the Fc portion of human IgG. It is approved for use in the treatment of rheumatoid arthritis, ankylosing spondylitis, juvenile rheumatoid arthritis, psoriasis, and psoriatic arthritis. Since 1998, there have been reports of vasculitic adverse events, including necrotizing vasculitis and leukocytoclastic vasculitis. In addition, the adverse events reporting system of the U.S. Food and Drug Administration has recorded 35 cases of leukocytoclastic vasculitis, 20 after etanercept therapy and 15 after infliximab. Most cases of cutaneous vasculitis describe development of symptoms within 3 months of etanercept use. In only one case report was direct immunofluorescence performed on tissue and no specific immunoreactivity found. We describe the first case of Henoch-Schönlein purpura with acute renal failure associated with increase in etanercept dose after 11 months of use for treatment of psoriasis. Discontinuation of the drug and treatment with a course of systemic steroids led to the complete resolution of the vasculitis and improvement of renal function. Vasculitis occurring even during chronic use of antitumor necrosis factor agents must be considered as possibly related to the therapy.

背景与目标： : 依那西普是一种重组二聚体融合蛋白，由与人IgG的Fc部分相连的肿瘤坏死因子-α 受体配体结合区组成。它被批准用于治疗类风湿关节炎，强直性脊柱炎，幼年类风湿性关节炎，牛皮癣和牛皮癣关节炎。自1998年以来，已有血管不良事件的报道，包括坏死性血管炎和白细胞碎屑血管炎。此外，美国食品和药物管理局的不良事件报告系统记录了35例白细胞碎屑病血管炎，依那西普治疗后20例，英夫利昔单抗后15例。大多数皮肤血管炎病例描述使用依那西普3个月内出现症状。仅在一种情况下，报告是对组织进行直接免疫荧光，未发现特异性免疫反应性。我们描述了第一例过敏性紫癜伴急性肾功能衰竭的病例，该病例与使用11个月治疗牛皮癣后依那西普剂量增加有关。停药并使用全身性类固醇治疗可完全解决血管炎并改善肾功能。即使在长期使用抗肿瘤坏死因子药物期间发生的血管炎也必须被认为可能与治疗有关。

BACKGROUND & AIMS: :There is currently no consensus on how best to manage refractory immune thrombocytopenic purpura (ITP). In part, this reflects the need for individualized treatment due to the wide spectrum of patients' requirements and responsiveness to therapies. The objective of this review is to provide a clinically useful guide to current management strategies. This article suggests investigations to identify factors that may exacerbate thrombocytopenia and underlie poor therapeutic responses, and highlights emerging therapies, including the thrombopoietic agents, which are anticipated to dramatically alter the natural history of "refractory" ITP. Morbidity, mortality and heath-related quality of life are also discussed.

背景与目标： : 目前关于如何最好地治疗难治性免疫性血小板减少性紫癜 (ITP) 尚无共识。在某种程度上，这反映了由于患者的广泛需求和对治疗的反应性而需要个性化治疗。本综述的目的是为当前的管理策略提供临床上有用的指南。本文建议进行研究，以确定可能加剧血小板减少症并导致不良治疗反应的因素，并重点介绍了新兴疗法，包括血小板生成剂，这些疗法有望显着改变 “难治性” ITP的自然史。还讨论了发病率，死亡率和与健康相关的生活质量。

BACKGROUND & AIMS: Cutaneous manifestations of petechiae, purpura, and ecchymosis can lead the physician to discover an underlying platelet abnormality. Autoimmune idiopathic thrombocytopenic purpura (AITP) is a diagnosis of exclusion, mediated by a destructive IgG antibody response to the platelets' membrane components. In addition to showing evidence of cutaneous and mucosal bleeding (ie, epistaxis, hematuria), patients with AITP are at an increased risk for systemic lupus erythematosus (SLE). Therefore, it is suggested that patients with AITP be closely monitored for SLE.

背景与目标： 瘀点，紫癜和瘀斑的皮肤表现可导致医生发现潜在的血小板异常。自身免疫性特发性血小板减少性紫癜 (AITP) 是一种排除的诊断，由对血小板膜成分的破坏性IgG抗体反应介导。除了显示皮肤和粘膜出血 (即鼻出血，血尿) 的证据外，AITP患者患系统性红斑狼疮 (SLE) 的风险增加。因此，建议密切监测AITP患者的SLE。

BACKGROUND & AIMS: :Thrombotic thrombocytopaenic purpura (TTP) is a rare but potentially devastating complication of pregnancy. We report the first documented case of a successful treatment of recurrent TTP complicating pregnancy in a renal transplant patient.

背景与目标： : 血栓性血小板减少性紫癜 (TTP) 是一种罕见但具有潜在破坏性的妊娠并发症。我们报告了首例成功治疗肾移植患者复发性TTP并发妊娠的病例。

BACKGROUND & AIMS: OBJECTIVES:To describe a group of patients treated at our service for Henoch-Schönlein purpura, with emphasis on recurrent and chronic cases, and to compare clinical and demographic characteristics of patients with monocyclic and recurrent disease. METHODS:Data on 67 patients who had been treated since disease onset were analyzed. Twelve patients were excluded because they failed to return for follow-up consultations after less than 3 months, leaving a total of 55 children in the study sample. Recurrence was defined as the presence of a fresh episode after a period of at least 3 months without symptoms, and cases were defined as chronic when cutaneous, abdominal and renal manifestations persisted for a period of 12 months or more. RESULTS:Recurrence was observed in 8/55 patients (14.4%) and four cases were chronic (7.2%). In 29/55 patients (52.7%), infection was identified as the trigger factor. A monocyclic clinical course was observed in 43 patients (26 of whom were girls, with a mean age of 5.4 years). Gastrointestinal and renal involvement was observed in 55.8 and 20.9% of patients, respectively. Among the 12 patients with recurrent or chronic Henoch-Schönlein purpura, three had arthritis, four exhibited signs and symptoms of abdominal involvement and seven of kidney disease: microscopic hematuria in five, macroscopic hematuria in one and hematuria with proteinuria in one other. Late onset was the only variable related to recurrence (p < 0.05). CONCLUSIONS:As is observed in medical literature, monocyclic cases are more common among children with early onset disease. Patients with Henoch-Schönlein purpura should be followed over the long term, since recurrent and chronic cases account for more than 20% of the total.

背景与目标：

BACKGROUND & AIMS: :Thrombotic thrombocytopenic purpura (TTP) is a classic, but not a common disorder of hematology. Plasma exchange (PE) was shown to nearly reverse its 90% mortality rate. However, there are still some fatal outcomes in this dramatic disease. We present our experience of plasma exchange plus corticosteroids for the treatment of TTP in our hospital. Patients with TTP diagnosed between January 1996 and January 2005 were identified by a retrospective review of records of the Uludag University Hospital, Bursa (the largest referral center for adults with this disorder in this region with an estimated 2.2 million residents), which performs all therapeutic PE in the southern Marmara region in Turkey. A total of 11 (6 male, 5 female) patients were treated for TTP during this period. The median age was 39 years (range 18-49). One plasma volume exchange daily plus steroid was the principle treatment in all patients. A total of 295 PE sessions were performed. We have obtained six complete responses (CR) and three partial responses (PR) with daily PE and steroid (response rate 9/11). One of our primary refractory patients was saved with pulse steroid+cyclosporine+vincristine. Now, he is disease free for over one year. The other refractory patient did not develop any response to salvage therapy and expired on day 15 with status epilepticus and ventilator related pneumonia (mortality rate 1/11). A CR was obtained with adjuvant treatments in all three PR patients. Only one CR patient developed an early relapse (early relapse rate in CR patients 1/6). She was treated successfully with daily PE plus vincristine. Our median follow up period was 25 months (range 9-108). Considering our local population, our annual incidence is only about 0.63 new cases per one million people. This figure is considerably less than the data from US, which indicated an incidence of 3.7 cases per 1,000,000. To our knowledge, there is no high variability in the incidence of TTP in the different geographical regions of the world. It suggests that considerable number of patients escaped notice. We hope that, demonstrating the successful outcome, this article serves to urge primary physicians to keep in mind the diagnosis of TTP and refer suspected cases quickly.

背景与目标： : 血栓性血小板减少性紫癜 (TTP) 是一种经典但不是血液学的常见疾病。等离子体交换 (PE) 显示几乎逆转其90% 死亡率。然而，这种戏剧性的疾病仍然有一些致命的结果。我们介绍了我们医院血浆置换加皮质类固醇治疗TTP的经验。通过对布尔萨乌鲁达格大学医院 (该地区最大的成人转诊中心，估计有220万名居民) 的记录进行回顾性审查，确定了1996年1月至2005年1月之间诊断出的TTP患者，该医院在土耳其南部马尔马拉地区进行所有治疗性PE。在此期间，共有11名 (6名男性，5名女性) 患者接受了TTP治疗。中位年龄为39岁 (18-49岁)。在所有患者中，每天一次血浆量交换加类固醇是主要治疗方法。总共进行了295次PE会话。我们已经获得了六个完全反应 (CR) 和三个部分反应 (PR) 与每日PE和类固醇 (反应率9/11)。我们的一名主要难治性患者被脉冲类固醇环孢素长春新碱挽救。现在，他一年多没有疾病。另一难治性患者对挽救治疗没有任何反应，并在第15天因癫痫持续状态和呼吸机相关性肺炎而死亡 (死亡率1/11)。所有三名PR患者均接受辅助治疗，获得CR。只有一名CR患者出现早期复发 (1/6 CR患者的早期复发率)。她接受了每日PE加长春新碱的治疗。我们的中位随访期为25个月 (范围9-108)。考虑到我们的当地人口，我们的年发病率仅为每100万人中0.63例新病例。这一数字大大低于美国的数据，后者表明每1,000,000有3.7例病例。据我们所知，在世界不同的地理区域，TTP的发病率没有很高的变异性。这表明相当多的患者逃脱了通知。我们希望，通过证明成功的结果，本文可以敦促初级医生牢记TTP的诊断并迅速转诊疑似病例。

BACKGROUND & AIMS: :Treatment of relapsed or refractory autoimmune mediated haemolytic syndromes, such as autoimmune haemolytic anaemia (AIHA) and thrombotic thrombocytopenic purpura (TTP), represents a therapeutic challenge. Here we report on our experience with the monoclonal anti-CD20 antibody rituximab (R) compared to standard treatment in these diseases. Patients with non-familial TTP or AIHA and no underlying malignancy were included in our analysis. Safety and efficacy of R-treatment were compared to results obtained in standard treatment approaches. Altogether, 27 patients were analyzed, comprising 15 patients with TTP and 12 patients with AIHA. The patients' average age at the time of diagnosis was 54 years. Eleven patients received antibody treatment (8 TTP, 3 AIHA). No acute or late WHO grade III/IV toxicity associated with rituximab was noted. With standard therapy, the overall response rate (ORR) was 66.7% for AIHA and 65.8% for TTP, respectively. For the R-containing regimens the ORR was 100%. In patients with TTP, median progression free survival (PFS) with R-treatment was 3.8 years, as compared to 0.1 years in the standard-treatment group. In patients with AIHA median PFS was not reached for R-containing treatment; all patients are in sustained remissions with a median follow up of 12.5 months. In the absence of prospective trials, our data underline the safety and efficacy of rituximab in relapsed and refractory autoimmune anaemias with favourable response rates and promising long-term progression-free survival. Therefore, prospective clinical trials evaluating rituximab as salvage- and first-line-therapy are clearly warranted.

背景与目标： : 治疗复发或难治性自身免疫介导的溶血综合征，如自身免疫溶血性贫血 (AIHA) 和血栓性血小板减少性紫癜 (TTP)，是一种治疗挑战。在这里，我们报告了与这些疾病的标准治疗相比，单克隆anti-CD20抗体利妥昔单抗 (R) 的经验。我们的分析包括非家族性TTP或AIHA且无潜在恶性肿瘤的患者。将R治疗的安全性和有效性与标准治疗方法中获得的结果进行了比较。总共分析了27例患者，其中15例TTP患者和12例AIHA患者。诊断时患者的平均年龄为54岁。11例患者接受了抗体治疗 (8 TTP，3 AIHA)。未发现与利妥昔单抗相关的急性或晚期WHO III/IV级毒性。在标准治疗中，AIHA的总缓解率 (ORR) 66.7%，TTP的总缓解率 (ORR) 65.8%。对于含R的方案，100% ORR。在TTP患者中，R治疗的中位无进展生存期 (PFS) 为3.8年，而标准治疗组为0.1年。在AIHA患者中，含R治疗未达到中位PFS; 所有患者均持续缓解，中位随访12.5个月。在缺乏前瞻性试验的情况下，我们的数据强调了利妥昔单抗在复发和难治性自身免疫性贫血中的安全性和有效性，具有良好的缓解率和有希望的长期无进展生存期。因此，评估利妥昔单抗作为挽救治疗和一线治疗的前瞻性临床试验显然是必要的。

BACKGROUND & AIMS: OBJECTIVE:To describe the hypotheses that may explain a diminished hemostatic response in a patient receiving multiple doses of recombinant coagulation factor VIIa (rFVIIa) for off-label treatment of bleeding events. CASE SUMMARY:A 70-year-old female with a significant history of idiopathic thrombocytopenic purpura (ITP) was admitted for coronary artery bypass grafting surgery. The patient developed thrombocytopenia and persistent hemorrhage postoperatively that was refractory to conventional therapy for ITP. She experienced an initial hemostatic response to rFVIIa after receiving 3 doses. During her second trial of rFVIIa a few days later, the duration of hemostatic effect was approximately half that of the first. The patient then received rFVIIa almost daily over the following 9 days to which she remained unresponsive, ultimately resulting in death. All doses in this patient were 9.6 mg (101 microg/kg), except the last, which was 4.8 mg (50.5 microg/kg). DISCUSSION:Several hypotheses may explain this patient's resistance to rFVIIa therapy. Two involve depletion of platelets or coagulation factors essential for rFVIIa efficacy. Another involves development of an antibody to rFVIIa. The last involves acidemia, which may interfere with the pharmacologic effect of rFVIIa. CONCLUSIONS:The combination of persistent thrombocytopenia and exhaustion of coagulation factors is the likely cause leading to resistance to rFVIIa therapy in this patient.

背景与目标：