纵隔灰区淋巴瘤的研究进展

纵隔灰区淋巴瘤(mediastinal gray zone lymphoma)作为一个疾病实体,常不能依据现有的诊断标准进行分类.这类淋巴瘤同时具有纵隔弥漫大B细胞淋巴瘤(PMBL)和经典霍奇金淋巴瘤(cHL)的特征.在2008年WHO造血与淋巴组织肿瘤分类中将其命名为"B细胞淋巴瘤,不能分类,具有介于弥漫大B细胞淋巴瘤和经典霍奇金淋巴瘤之间的特征(BCLu)".BCLu具有独特的临床特点、免疫表型和分子遗传学特征,临床过程更具侵袭性,预后较差.目前尚无达成共识的治疗方案,可参照侵袭性B细胞淋巴瘤的方案化疗.     

常见惰性B细胞淋巴瘤治疗进展:第35届欧洲肿瘤内科学会大会报道

2010年10月8日至12日第35届欧洲肿瘤内科学会(ESMO)大会在意大利米兰召开.米兰是霍奇金淋巴瘤(HL)首选治疗方案(ABVD)的发源地,本届大会就淋巴瘤治疗进展进行了多场精彩的学术报告,现简要介绍3种常见惰性B细胞淋巴瘤的治疗进展.     

Cyclin D1和bcl-2在B细胞淋巴瘤中的表达及其在鉴别诊断中的意义

B细胞淋巴瘤根据免疫表型可分为不同亚型,且不同亚型侵袭度不同,预后也有很大差异.Cyclin D1是已被证实与肿瘤有最直接关系的细胞周期蛋白,在大多B细胞淋巴瘤[套细胞淋巴瘤(MCL)、慢性淋巴细胞白血病(CLL)、边缘区淋巴瘤(MZL)、弥漫大B细胞淋巴瘤(DLBCL)等」中均有表达.多数B细胞淋巴瘤[滤泡性淋巴瘤(FL)、DLBCL等]都能可见易位活化的bcl-2表达增强.Cyclin D1及bcl-2作为B细胞淋巴瘤重要的细胞周期蛋白及抗凋亡基因,在淋巴瘤的鉴别诊断中起重要作用,其检测及检测手段的灵敏度和特异度具有重要的临床价值.     

非霍奇金淋巴瘤合并纯红细胞再生障碍性贫血一例

患者男,58岁.2004年3月出现体温升高、乏力、盗汗等症状,体温最高37.5℃.骨髓涂片及活检:小B细胞型非霍奇金淋巴瘤,诊断:非霍奇金淋巴瘤(小B细胞型)ⅣB期,骨髓浸润.先后给予9个疗程CHOP方案、3个疗程BECHOP方案、2个疗程FC方案化疗后,低热、盗汗等症状消失.     

第33届国际血液病学大会热点聚焦

2010年10月10日至13日,国际血液病学会(ISH)主办的第33届国际血液病学会大会在以色列耶路撒冷召开.大会在白血病和淋巴瘤的治疗的最新进展方面有很多精彩的内容,现就慢性淋巴细胞白血病(CLL)、B细胞非霍奇金淋巴瘤和滤泡性淋巴瘤(FL)方面的热点内容作简要介绍,与读者共享.     

非霍奇金淋巴瘤中枢神经系统累及的诊断和预防

非霍奇金淋巴瘤(NHL)患者中枢神经系统(CNS)累及预后不良,其中位生存期2～6个月.与NHLCNS累及相关参数是年轻、进展期、累及结外部位数、乳酸脱氢酶(LDH)增高和国际预后指标(IPI)积分.最有希望的治疗为自体造血于细胞移植,可延长中数生存期10～26个月.处于CNS侵袭高危状态的某些NHL亚型患者需要早期进行CNS预防,如伯基特淋巴瘤(BL)和淋巴母细胞淋巴瘤(LBL).弥漫性大B细胞淋巴瘤(DLBCL)初期治疗时是否需应用CNS预防久有争议,因为它属于CNS累及(≈5%)的低危群体.危险模式的确定有助于预示NHL的CNS复发.     

弥漫大B细胞淋巴瘤细胞耐药的研究进展

弥漫大B细胞淋巴瘤(DLBCL)化疗耐药的主要原因是细胞耐药.研究表明DLBCL细胞耐药与耐药基因及其耐药相关蛋白、细胞因子、黏附分子有关,并在造血微环境中通过信号转导介导细胞耐药.     

系统型间变性大细胞淋巴瘤30例临床分析

目的 探讨系统型间变性大细胞淋巴瘤(S-ALCL)的临床特征和预后相关因素.方法 回顾性分析30例S-ALCL患者的临床资料.30例患者均以联合化疗为主,配合局部病灶野放疗8例.化疗方案主要为CHOP、EPOCH、Hyper-CVAD,以CHOP方案为主.结果 30例S-ALCL患者中位年龄36岁,男女比例为1.5∶1,有B症状、Ⅲ～Ⅳ期和结外侵犯者分别占60.0%(18/30)、73.3%(22/30)和60.O%(18/30);乳酸脱氢酶(LDH)升高者占46.7%(14/30);间变性大细胞淋巴瘤激酶(ALK)+18例(60.0%),其发病年龄小于ALK-者(u=3.92,P=0.001).单因素分析显示ALKˉ及LDH升高是重要的预后不良因素.结论 S-ALCL患者发病年龄较轻,预后较好.但ALK-、LDH升高者预后不良.治疗以联合化疗为主,对于有不良预后因素的患者,大剂量治疗可能获益.     

儿童侵袭性成熟B细胞淋巴瘤的临床病理学及分子遗传学特点

目的 分析总结中国儿童各类型侵袭性成熟B细胞淋巴瘤的临床病理学及分子遗传学特点,为其诊断的标准化提供依据.方法 收集97例儿童侵袭性成熟B细胞淋巴瘤石蜡包埋组织标本,包括伯基特淋巴瘤(BL)81例、弥漫大B细胞淋巴瘤(DLBCL)8例、介于BL和DLBCL间的不能分类的B细胞淋巴瘤(BL/DLBCL)8例,利用免疫组织化学技术和间期荧光原位杂交(FISH)技术检测其免疫表型和分子遗传学特征.结果 BL的bcl-2和MUM1的阳性率分别为3%(2/66)和17%(12/71),DLBCL分别为50%(4/8)和63%(5/8),BL/DLBCL分别为50%(4/8)和63%(5/8).BL、DLBCL和BL/DLBCL的Ki-67平均值分别为(93±4.4)%、(83±14.3)%和(80±11.5)%.BL、DLBCL和BL/DLBCL的c-myc基因易位的比例分别为98%(79/81)、38%(3/8)和50%(4/8).38%(3/8)的DLBCL和25%(2/8)的BL/DLBCL存在bcl-6基因的多拷贝,BL与DLBCL之间、BL与BL/DLBCL之间bcl-2、MUM1和Ki-67平均值的差异及c-myc基因易位和bcl-6基因多拷贝的差异均有统计学意义(均P＜0.05).结论 儿童侵袭性成熟B细胞淋巴瘤的诊断和分型需要综合分析形态学、免疫表型和分子遗传学特征.儿童BL/DLBCL可能是DLBCL的一个亚型.CD10+、bcl-6+、bcl-2-、Ki-67＞90%、伴有IGH/c-myc重排、不伴有bcl-2和bcl-6重排时,支持BL的诊断;bcl-2+、Ki-67为50%～90%,同时伴有bcl-6基因的多拷贝时,支持DLBCL或BL/DLBCL的诊断.     

原发结内外周T细胞淋巴瘤19例临床特征及预后分析

目的 分析原发结内外周T细胞淋巴瘤(PTCL)的临床特点、治疗和预后.方法 回顾性分析19例原发结内PTCL患者的临床资料、治疗反应以及预后因素.结果 19例患者中位发病年龄54岁,男女比例2.17∶1,其中94.7%(18/19)为Ⅲ～Ⅳ期,84.2%(16/19)有B症状,84.2%(16/19)有结外器官受累,57.9%(11/19)有骨髓浸润.化疗完全缓解(CR)率36.8%(7/19),2年总生存(OS)率47.4%,2年无进展生存(PFS)率25%.预后分析显示,结外侵犯数量(EN)≥2个、美国东部肿瘤协作组(ECOG)体能状态评分≥2分、国际预后指数(IPI)评分＞2分以及β2-微球蛋白(βrMG)升高为不良预后因素.结论 原发结内PTCL是一类高度侵袭的异质性T细胞淋巴瘤,化疗效果差,多项因素提示不良预后.     

Combined thoracoscopy and mediastinoscopy for the evaluation of mediastinal lymph node metastasis in left upper lobe lung cancer

Cervical mediastinoscopy has an important but limited role in the evaluation of mediastinal adenopathy of the aorticopulmonary window in patients with left lung cancer. Thoracoscopy is another valuable diagnostic procedure in the assessment of mediastinal adenopathy. Combined thoracoscopic and mediastinoscopic evaluation may be more accurate for assessing mediastinal lymph node metastasis in left lung cancer than either procedure alone.     

Metastasis of an adenocarcinoma of unknown origin to mediastinal lymph nodes, and transient regression

A 67-year-old woman was admitted to our hospital because of fever. Chest roentgenogram showed an enlargement of mediastinal lymph nodes. Despite thorough examination, no definite diagnosis could be made. The mediastinal lymph nodes got smaller over the next 3 weeks and a chest roentgenogram taken 4 months later showed no mediastinal lymphadenopathy. The mediastinal lymphadenopathy and fever recurred 5 months later. She underwent thoracotomy and the mediastinal lymph nodes were excised. Microscopic examination of pretracheal lymph node specimens showed invasion of poorly differentiated adenocarcinoma associated with abundant tumor-infiltrating lymphocytes. The other lymph nodes showed sarcoid reaction. Although she has been followed for one year and 11 months, no primary site of the cancer has been found. Metastasis of cancer of unknown origin to mediastinal lymph nodes is extremely rare. It is also interesting that the lymph node swelling diminished spontaneously. The tumor-infiltrating lymphocytes and sarcoid reactions may have been immunological responses to the cancer and may have caused the transient regression.     

Primary mediastinal tumors: part II. Tumors of the middle and posterior mediastinum

Lymphoma, mediastinal cysts, and neurogenic neoplasms are the most common primary middle and posterior mediastinal tumors. Lymphoma may involve the anterior, middle and/or posterior mediastinum, frequently as lymphadenopathy or as a discrete mass. Foregut cysts are common congenital mediastinal cysts and frequently arise in the middle mediastinum. Pericardial cysts are rare. Schwannoma and neurofibroma are benign peripheral nerve neoplasms, represent the most common mediastinal neurogenic tumors, and rarely degenerate into malignant tumors of nerve sheath origin. Sympathetic ganglia tumors include benign ganglioneuroma and malignant ganglioneuroblastoma and neuroblastoma. Lateral thoracic meningocele is a rare cause of a posterior mediastinal mass.     

An early comparison between endoscopic ultrasound-guided fine-needle aspiration and mediastinoscopy for diagnosis of mediastinal malignancy

Precise mediastinal lymph node staging is essential in non-small cell lung cancer for proper evaluation and treatment. In addition to CT, mediastinoscopy is routinely used for staging and diagnosis of mediastinal malignancy. Recently, endoscopic ultrasound (EUS) combined with fine-needle aspiration (FNA) biopsy has been used to evaluate mediastinal disease. The purpose of this study was to assess and compare mediastinoscopy with EUS/FNA in the evaluation of mediastinal masses. From August 1995 to July 1997, 21 patients with suspected mediastinal malignancy underwent cervical mediastinoscopy with biopsy. During this same period, seven patients with suspected mediastinal malignancy were evaluated using EUS/FNA. All patients were retrospectively studied. Both mediastinoscopy and EUS/FNA were highly sensitive in diagnosing mediastinal malignancy (100% and 86%, respectively). Specificity and positive predictive value were 100 per cent for both procedures. Mediastinoscopy and EUS/FNA are highly accurate methods of staging mediastinal malignancy. Mediastinoscopy provides better access to the upper and anterior mediastinum, whereas EUS/FNA can safely be used to biopsy subcarinal and posterior mediastinal masses. Mediastinoscopy and EUS/FNA target different areas of the mediastinum and may be complimentary in the evaluation of mediastinal malignancy and staging of bronchogenic carcinoma.     

Incidence of iron-deficiency anaemia and depleted iron stores among nine-month-old infants in Vancouver, Canada

The authors analysed a group of 80 patients who were operated in 1985-1990 on account of non-small cell carcinoma of the lungs in stage IIIA with affection of the ipsilateral mediastinal nodes. The patients were not treated by neoadjuvant chemotherapy and systematic dissection of the mediastinal nodes was not performed. The results of five-year survival in the group of patients with affection of the mediastinal nodes (N2) were compared with those in the group of patients without affection of the mediastinal and hilar nodes (NO). Patients with affected N2 nodes who survived five years were significantly fewer than patients with negative mediastinal and hilar nodes. The probability of five-year survival in N2 was 15.8%, in patients with NO 28.0%. From data in the literature it is known that neoadjuvant chemotherapy and subsequent complete resection of the lung with the tumour combined with dissection of the mediastinal nodes may improve long-term survival after surgery. The authors assume that introduction of the described methods in their department will improve postoperative results.     

Mediastinal lipomatosis in simple obesity

A case of superior mediastinal widening due to unusual amounts of fat accumulations is reported in a patient with simple obesity, not associated with iatrogenic or primary Cushing's syndrome. The radiographic features included a smooth bilateral widening of the superior mediastinum, relative lucency, no definable mass in the lateral view, and no pressure effects on the trachea. Such mediastinal lipomatosis is a benign condition and may be related to general obesity. although rare, it should be considered in the differential diagnosis of mediastinal masses, particularly in obese individuals.     

Mediastinal lipomatosis

Mediastinal lipomatosis is a benign condition characterized by a large amount of mature adipose tissue within the mediastinum. It widens the mediastinum and may simulate mass lesions, thus leading to diagnostic errors. We describe a new case of a huge mediastinal lipomatosis and review 36 cases previously reported in the literature. Computed tomography has an important role in the diagnosis of this disease. To avoid invasive and unnecessary procedures, this diagnosis should be considered in any patient with Cushing's syndrome.     

Hydrocortisone and 11-desoxycortisone modify acetylcholine receptor channel gating

Over the years several methods for evaluating mediastinal involvement in Hodgkin's disease have been applied to chest radiographs and conflicting results have been reported. In a retrospective study of 104 patients we evaluated interobserver variability in assessing mediastinal involvement and investigated various cut-off points for mediastinal size as to their ability to identify patients with high- and low-risk for recurrence. For mediastinal involvement the concordance rate for two reviewing radiologists was 94% (98/104) and compared with prior assessment by outside radiologists the concordance rates were 90% (94/104) and 88% (92/104), respectively. A good correlation between the reviewing radiologists was found for the quantitative evaluation of mediastinal diameter and thoracic ratios. ROC curves and relative risk figures were used to investigate the various cut-off points for mediastinal width and for the ratios of the maximal mediastinal diameter to the chest diameter at Th 5-6 (M1) and to the chest diameter at the widest thoracic level (M2). Neither the ROC curve analysis nor the use of relative risk figures revealed a cut-off point clearly more accurate in predicting recurrence. In conclusion, our results do not suggest that interobserver variability in mediastinal assessment, differences in the method of mediastinal measurement, or the cut-off points applied to mediastinal width can explain the discrepancies in the reported data on the prognostic value of mediastinal width in Hodgkin's disease, but rather factors such as patient selection and differences in treatment given may be responsible.     

Anterior mediastinal lymphadenopathy in sarcoidosis

Several lymph node chains in the anterior mediastinum lie between the sternum and the trachea. On a lateral chest radiograph they form two distinct groups separated by the superior vena cava. One group projects anterior to the trachea and is formed by overlapping nodes of the anterior paratracheal and the left anterior mediastinal (prearterial) chains. The other is in front of the superior vena cava, composed of the right anterior mediastinal (prevenous) and horizontal chains. The position of these chains prevents them, even when enlarged, from being seen on posteroanterior chest radiographs or tomography: frequently they may not be appreciated on lateral chest films. Therefore lateral chest tomography is the best method to evaluate anterior mediastinal lymphadenopathy. With this technique, anterior mediastinal lymphadenopathy in sarcoidosis should be found more frequently than indicated in the literature.