EBV阳性的血管免疫母细胞性T细胞淋巴瘤临床病理分析

目的探讨血管免疫母细胞性T细胞淋巴瘤临床病理特点、治疗和预后。方法对1例血管免疫母细胞性T细胞淋巴瘤的临床病理特点、免疫组化、诊断及鉴别诊断要点进行详细分析,并结合相关文献进行复习。结果淋巴结副皮质区肿瘤细胞弥漫浸润,瘤细胞中等大小,胞质淡染或透明,胞核呈圆形或椭圆形,染色质细粉尘样,血管增生,呈分枝状,血管内皮肿胀。免疫组化瘤细胞示:LCA(+)、CD3(+)、CD4(+)、CD(5+)、CD10(+)、CD30(R-S样细胞+)、Bcl-2(+)、CXCL13(+)、CD21(滤泡树突细胞+)、CD35(+)、CD43(+)、EBV(+)、Ki67(约30%阳性)。结论血管免疫母细胞性T细胞淋巴瘤早期难以诊断,需与淋巴结反应性增生、周围T细胞淋巴瘤(非特殊类型)、霍奇金淋巴瘤和滤泡增生样的外周T细胞淋巴瘤相鉴别;诊断依靠组织病理、免疫组化等综合手段;化疗和放疗联合治疗可提高其生存期,但血管免疫母细胞性T细胞淋巴瘤的预后个体差异大,总体预后较差。     

肝血管内大B细胞性淋巴瘤2例临床病理观察

目的探讨肝血管内大B细胞性淋巴瘤的临床病理特征、诊断与鉴别诊断、治疗及预后。方法回顾性分析2例肝血管内大B细胞性淋巴瘤患者的临床资料、组织病理学形态和免疫组化结果。结果光镜下肝窦内和小血管内可见较多具有明显异型性的淋巴样细胞浸润,汇管区可见慢性炎细胞浸润,亦可见少许异型淋巴细胞样细胞,未见明确纤维化。免疫组化示CD20、PAX5弥漫(+),CD3散在少许(+),Ki-67阳性率为70%,AE1/AE3、CD117和CD56均(-);其中例1 CD5弥漫(+)。结论血管内大B细胞性淋巴瘤是一种具有高度侵袭性的结外弥漫性大B细胞性淋巴瘤的亚型,由于该病临床表现多样及不典型性,造成了部分患者的诊断困难,因此,掌握临床病理及免疫组化特征对该病的诊断和鉴别诊断具有重要意义。     

伴有淋巴样间质的微结节型胸腺瘤6例临床病理分析

目的探讨伴有淋巴样间质的微结节型胸腺瘤(MNT)的临床病理特征、免疫组化、诊断与鉴别诊断及其预后。方法回顾性分析6例MNT的临床相关资料及病理特征,对其进行HE及免疫组化Eili Vision法染色,并复习相关文献。结果 6例患者年龄53~77岁,平均年龄61.8岁,男女之比为5∶1。5例为体检时发现,1例以胸闷为主诉检查发现。CT提示均为前纵隔占位。镜检由富于淋巴细胞的间质所分隔的散在或融合成团的上皮细胞结节,淋巴细胞间质中常见含有生发中心的淋巴滤泡结构,背景中可见散在分布的小囊腔;其中1例伴发B2型胸腺瘤,镜下可见丰富的上皮细胞中散在不成熟的淋巴细胞。免疫组化:上皮细胞CK19(+)、EMA(-),上皮细胞巢内可见散在CD3(+)的T淋巴细胞,富于淋巴细胞的间质主要由CD20(+)的B细胞构成淋巴滤泡结构,囊腔被覆上皮CK19和EMA均(+),CD3和CD5(+)的T细胞位于滤泡间区,而CD99(+)的不成熟T细胞主要位于淋巴间质中,上皮细胞团中少见。术后随访,1例不明原因死亡,其余存活,无复发及转移。结论 MNT是一种罕见的胸腺上皮性肿瘤,预后良好。具有特征性病理表现,结合发生部位、影像学资料、组织病理学特点及免疫组化标记可以作出准确诊断。     

原发性鼻腔鼻窦黏膜恶性黑色素瘤4例临床病理分析

目的 探讨原发性鼻腔鼻窦黏膜恶性黑色素瘤(SMMM)的临床病理特点、鉴别诊断、治疗与预后.方法 回顾性分析4例SMMM的临床病理资料,观察其组织形态特征及免疫组化特点,并复习相关文献.结果 患者男性1例,女性3例,平均年龄75.5岁(65～90岁),临床主要表现为鼻塞、出血及鼻腔新生物,镜下瘤细胞形态多样,异型性较明显,以上皮样细胞和小圆细胞为主,伴有局部少量梭形细胞样细胞,仅部分显示黑色素.其中1例可见黏液样变,1例色素性黏膜恶性黑色素瘤局部复发后细胞体积明显增大,色素减少,多数细胞胞质透明,呈气球样变.免疫组化示HMB45、vimentin、S-100均不同程度(+),NSE、LCA、sMA、CD99和desmin(-),局部CK、CD68弱(+)各1例,Ki-67程度不一.结论 鼻腔原发性黑色素瘤少见,恶性程度较高,形态多样,免疫组化对诊断及鉴别较为重要.     

子宫高度富于细胞性平滑肌瘤14例临床病理学特征

目的 探讨子宫高度富于细胞性平滑肌瘤的临床病理特征、免疫组化特点、诊断及鉴别诊断.方法 回顾性复习14例子宫高度富于细胞性平滑肌瘤的临床表现、组织学形态、免疫组化及冷冻诊断.对照组为10例低级别子宫内膜间质肉瘤.结果 患者年龄27～ 65岁,平均年龄44.6岁;11例有月经改变史.镜下见高度致密的富于细胞性平滑肌瘤的瘤细胞,丰富程度类似于子宫内膜间质肿瘤;细胞呈圆形、卵圆形,胞质少;所有病例均可见大的厚壁血管,其中3例见明显的胶原化间质.免疫组化:11例h-caldesmon(+)、desmin (11/14)和SMA (9/14)不同程度(+),3例CD10局灶(+).低级别子宫内膜间质肉瘤镜下形态与高度富于细胞性平滑肌瘤非常相似,但免疫组化h-caldesmon(－),CD10(10/10)、desmin(9/10)和SMA(6/10)(+).结论 子宫高度富于细胞性平滑肌瘤与低级别子宫内膜间质肉瘤的鉴别非常困难,尤其在冷冻切片诊断中;免疫组化染色有助于鉴别诊断.     

卵巢混合性大细胞神经内分泌癌伴黏液性上皮性肿瘤临床病理观察

目的探讨卵巢大细胞神经内分泌癌(LCNEC)伴黏液性上皮性肿瘤的临床病理学特点及鉴别诊断。方法对1例发生于48岁女性患者右侧卵巢的囊实性肿瘤组织行临床病理学和免疫组化观察,并结合相关文献对该类肿瘤进行总结和分析。结果镜下显示该肿瘤95%区域为LCNEC,5%区域为黏液性上皮性肿瘤伴灶性区出现交界性肿瘤的特征。免疫组化示LCNEC细胞Syn、Cg A和CD56(+)。结论卵巢LCNEC合并上皮源性或生殖细胞肿瘤是一种非常罕见的高度恶性混合性肿瘤,预后常较差;其诊断需借助组织形态学和免疫组化。     

CD40、HLA-DR在自身免疫性肝病Kupffer细胞的表达变化

目的研究自身免疫性肝病Kupffer细胞抗原呈递分子的表达变化。方法按照自身免疫性肝炎、原发性胆汁性胆管炎的诊断标准进行分组,脂肪肝作为对照组。入选患者的肝穿石蜡切片进行CD40、HLA-DR免疫组化染色,随机选取10个显微镜高倍视野(×400倍),观察染色情况,并统计CD40+、HLA-DR+细胞数目。结果 CD40在自身免疫性肝病组的阳性细胞数低于对照组。CD40免疫组化阳性产物呈棕褐色或棕黄色颗粒状着色,主要位于细胞膜,少部分位于细胞质。分别计算自身免疫性肝炎、原发性胆汁性胆管炎和脂肪肝3组肝穿标本中的CD40阳性的kupffer细胞数绝对值,结果显示:3组间CD40阳性的kupffer细胞的计数绝对值比较具有统计学意义(P=0.04,P0.05)。AIH与PBC组比较无统计学差异(P=0.428,P0.05);AIH与脂肪肝组比较具有统计学意义(P=0.02,P0.05)。免疫组化中,HLA-DR在自身免疫性肝病组的阳性细胞数低于对照组,HLA-DR免疫组化染色阳性产物呈棕褐色颗粒,主要位于细胞膜。分别统计AIH、PBC和脂肪肝3组的HLA-DR阳性的kupffer细胞计数的绝对值,结果显示3组比较其结果具有统计学意义(P=0.015,P0.05),AIH与脂肪肝组比较具有统计学意义(P=0.017,P0.05)。CD40、HLA-DR结果与肝功能、凝血功能化验检查结果进行相关性分析,结果显示具有相关性。结论自身免疫性肝病患者组的肝穿石蜡切片免疫组化染色显示CD40及HLA-DR表达较脂肪肝组数量减少,且CD40、HLA-DR与自身免疫性肝病的转氨酶变化相关,提示自身免疫性肝病可能存在kupffer细胞抗原呈递功能减弱,是导致自身免疫性肝病肝损害的原因之一。     

直肠炎性肌纤维母细胞性肿瘤临床病理观察

目的 探讨直肠炎性肌纤维母细胞性肿瘤(IMT)的临床病理特征.方法 对1例IMT进行临床、组织学和免疫组化观察,并复习相关文献.结果 患者女性,36岁.临床表现为腹痛伴黏液血便半个月.肠镜检查示距肛门15 cm处可见5 cm×4 cm大小的息肉样肿块,表面高低不平.镜检:肿瘤组织主要由束状交错排列的梭形细胞组成,背景为黏液样基质及大量淋巴细胞、浆细胞浸润.免疫组化:梭形细胞vimentin、SMA和desmin(+),Ki-67阳性指数为20％,而ALK1、CD117、KP1、CD30、CD34、AE1/AE3、myogenin、H-caldesmon和HMB45均为(-).术后随访18个月未复发.结论 炎性肌纤维母细胞性肿瘤罕见于直肠,易被临床医师误诊为恶性肿瘤.免疫组化结果是该肿瘤与其他软组织肿瘤鉴别的重要依据.     

前列腺炎性肌纤维母细胞瘤临床病理观察

目的 探讨前列腺炎性肌纤维母细胞瘤的临床病理学特征和鉴别诊断.方法 对1例前列腺炎性肌纤维母细胞瘤进行临床、组织病理学和免疫组化观察,并复习相关文献.结果 镜下见肿瘤呈多结节状,黏液样背景,瘤细胞以梭形细胞为主,无异型性及核分裂.间质内见大量淋巴细胞、浆细胞浸润,血管丰富.免疫组化:梭形肿瘤细胞vimentin和desmin(+),SMA灶性(+),CD117、CD34和S-100均(-).结论 炎性肌纤维母细胞瘤是一种特殊类型的软组织肿瘤,较罕见于前列腺,应注意与良性纤维组织细胞瘤、孤立性纤维性肿瘤、低度恶性纤维黏液样肉瘤、炎症性恶性纤维组织细胞瘤等鉴别.     

窦组织细胞增生伴巨大淋巴结病累及鼻腔临床病理观察

目的探讨窦组织细胞增生伴巨大淋巴结病(SHML)的临床病理特征。方法对1例颈部、鼻腔同时发生SHML的病例进行临床病理分析及免疫组化研究,并进行文献复习。结果颈部淋巴结SHML临床表现为双侧颈部无痛性巨大型淋巴结肿大,镜下病变淋巴结淋巴窦扩张,淋巴窦内充盈淋巴细胞、浆细胞及大量组织细胞,并可见特征性吞噬淋巴细胞、浆细胞或红细胞的组织细胞。鼻腔SHML的临床表现为鼻塞、鼻出血等。镜下病变主要由梭形细胞组成,伴有大量炎细胞浸润,伸入运动不显著。免疫组化显示颈部淋巴结和鼻腔组织细胞S-100和CD68(+),CD1α(-)。结论 SHML可累及鼻腔,诊断主要依赖病理组织学和免疫组化。     

Pulmonary involvement of autoimmune pancreatitis

Background  A wide variety of systemic lesions have been seen in patients with autoimmune pancreatitis. The pulmonary involvement of autoimmune pancreatitis was analysed to clarify the clinicopathological features of pulmonary lesions in comparison with pulmonary sarcoidosis.
Materials and methods  Nineteen patients had autoimmune pancreatitis and eight had pulmonary sarcoidosis. The symptoms, laboratory data, chest computed tomography, Gallium-67 scintigraphy, pulmonary function testing and bronchoscopy findings, including the histological IgG4-immunostaining and IgG subclasses in the bronchoalveolar lavage in autoimmune pancreatitis, were collected to compare them with pulmonary sarcoidosis.
Results  The serum total protein, IgG and IgG4 levels were found to be significantly elevated in comparison with pulmonary sarcoidosis. In autoimmune pancreatitis, 17 patients showed bilateral hilar lymphadenopathy, while eight showed pulmonary nodules on chest computed tomography. Eighteen of 19 patients on Gallium-67 scintigraphy showed accumulation spots in either the hilar or mediastinal lymph nodes. Six patients with pulmonary nodules demonstrated accumulation spots in the corresponding lesions on chest computed tomography. All eight patients with pulmonary sarcoidosis showed accumulation spots in either the hilar or mediastinal lymph nodes. Bronchoalveolar lavage IgG4 in autoimmune pancreatitis showed a significant increase in comparison with pulmonary sarcoidosis. The histological findings obtained by a transbronchial lung biopsy showed the infiltration of lymphocytes and plasma cells in the thickened interstitum and alveoli with IgG4-positive plasma cell infiltration in patients with autoimmune pancreatitis.
Conclusion  IgG4 in the bronchoalveolar lavage was seen at remarkably increased levels and IgG4-positive plasma cells were identified in the pulmonary lesions of patients with autoimmune pancreatitis.     

Serum IgG4 concentrations in pancreatic and biliary diseases

BACKGROUND: Recently, it has been reported that the serum concentration of IgG4, a minor component of IgG subclasses, is increased in autoimmune pancreatitis. However, data regarding IgG4 concentrations in other pancreatic or biliary diseases have been insufficient. METHODS: Serum IgG4 was measured in 116 patients with pancreatic or biliary diseases (35 autoimmune pancreatitis, 24 chronic pancreatitis except autoimmune pancreatitis, 11 primary sclerosing cholangitis, 23 pancreatic cancer, 3 islet cell tumor, 2 papilla cancer, 15 bile duct cancer, and 3 gallbladder cancer patients). The cut-off concentration of IgG4 was 135 mg/dl. RESULTS: Increased serum IgG4 was observed in 33 of 35 patients with autoimmune pancreatitis, 0 of 24 with chronic pancreatitis, 4 of 11 with primary sclerosing cholangitis, 0 of 23 with pancreatic cancer, 0 of 3 with islet cell tumor, 0 of 2 with duodenal papilla cancer, 0 of 15 with bile duct cancer and 0 of 3 with gallbladder cancer patients. CONCLUSIONS: Serum IgG4 was increased in autoimmune pancreatitis and was within normal limits for other pancreatic or biliary diseases except primary sclerosing cholangitis.     

Contrast-enhanced endoscopic ultrasound in the diagnosis of autoimmune pancreatitis

Autoimmune pancreatitis is a rare condition which can mimic pancreatic carcinoma. We report the cases of 10 patients with autoimmune pancreatitis investigated in two different centers using contrast-enhanced endosonography. In these patients, contrast-enhanced endosonography showed a unique vascularization pattern which makes it easy to discriminate between autoimmune pancreatitis and lesions caused by pancreatic cancer. Lesions caused by autoimmune pancreatitis and the surrounding pancreas typically showed hypervascularization, whereas lesions caused by pancreatic cancer were hypovascularized. This was true for all patients with the exception of one who showed a normal vascularization pattern in comparison with normal patients and no signs of hypovascularization. Final diagnosis was achieved either by transcutaneous biopsy or a combination of endoscopic fine-needle aspiration with IgG4 immunostaining of the sample. All patients were followed up over a period of at least 12 months to rule out pancreatic carcinoma.     

Immunological diseases of the pancreatico-hepatobiliary system: update on etiopathogenesis and cross-sectional imaging findings

Immunological diseases of the hepatobiliary system and the pancreas include a broad spectrum of disorders that manifest characteristic histopathology/serology and variable clinical features and imaging findings. Recent studies have thrown fresh light on the complex role of genetics and autoimmunity in the pathogenesis and natural history of these diverse disorders that include autoimmune hepatitis, primary biliary cirrhosis, primary sclerosing cholangitis, IgG4-related cholangitis, overlap/outlier syndromes, and autoimmune pancreatitis.     

Contrast-enhanced sonography of autoimmune pancreatitis: comparison with pathologic findings.

OBJECTIVE: We evaluated the vascularity of autoimmune pancreatitis lesions on contrast-enhanced harmonic gray scale sonographic images in comparison with the pathologic findings. METHODS: Six patients with autoimmune pancreatitis were examined. All patients held their breath from 20 to 50 seconds after the injection of a contrast agent while the vascularity of the lesion was examined by contrast-enhanced harmonic gray scale sonography (early phase), and lesion enhancement was monitored at about 90 seconds after the injection while the patients held their breath for a few seconds (delayed phase). We then compared the vascularity on the contrast-enhanced harmonic gray scale sonographic images with the pathologic findings (fibrosis and inflammation) in all lesions. The vascularity of 3 of the 6 lesions was also evaluated by contrast-enhanced harmonic gray scale sonography before and after treatment with corticosteroids. RESULTS: The autoimmune pancreatitis lesions exhibited mild (n = 1), moderate (n = 3), or marked (n = 2) enhancement throughout almost the entire lesions in both the early and delayed phases. The grade of lesion vascularity on the contrast-enhanced harmonic gray scale sonographic images correlated with the pathologic grade of inflammation and inversely correlated with the grade of fibrosis associated with autoimmune pancreatitis. The vascularity of all 3 lesions had decreased on the contrast-enhanced harmonic gray scale sonographic images after steroid therapy. CONCLUSIONS: Contrast-enhanced harmonic gray scale sonography may be useful for evaluating the vascularity of autoimmune pancreatitis lesions and the therapeutic efficacy of steroid therapy.     

CT双期扫描对节段性自身免疫性胰腺炎与胰腺癌及胰内分泌肿瘤的鉴别诊断价值

目的：研究节段性自身免疫性胰腺炎（Autoimmune pancreatitis,AIP）CT双期扫描的强化模式与胰腺癌及内分泌肿瘤（Pancreatic endocrine tumor,PECT）的差异,提高AIP的诊断水平。方法：收集9例节段性AIP患者、25例胰腺癌及16例PECT患者的CT平扫和双期动态增强资料以及25例性别、年龄与AIP相似的正常对照组,分析双期扫描3种病变的影像特点以及与正常胰腺的密度差异;测量4组的双期扫描平均CT值,分别比较AIP组与胰腺癌、PECT组的平均CT值差异,同时与正常对照组比较。结果：动脉期,在图像上,与同病例正常胰腺比较,9例AIP患者的病变胰腺均表现为相对低密度,25例胰腺癌患者呈明显低密度,16例PECT患者均呈高密度;AIP组、胰腺癌组、PECT组的平均CT值分别为72.7HU、49.2HU、115.6HU,正常组的平均CT值为93.4HU。门脉期,在图像上,与同病例正常胰腺比较,9例AIP患者的病变胰腺均为等或偏高密度,25例胰腺癌患者的病变胰腺均为明显低密度;16例PECT患者呈偏高密度;AIP组、胰腺癌组、PECT组的平均CT值分别为86.6HU、57.6HU、104.2HU,正常组的平均CT值为83.9HU。结论：AIP与胰腺癌、PECT的强化模式不同,不同时相,病变与正常胰腺的对比度不同,CT双期扫描在节段性AIP与胰腺癌、PECT鉴别诊断中有重要价值,通过CT双期扫描可作出AIP的倾向性诊断。     

Steroid-responsive pancreatitides

Autoimmune pancreatitis has received considerable attention, especially due to the marked effect of corticosteroid therapy on its clinical course. Knowledge, especially regarding type 1 autoimmune pancreatitis, has significantly increased over the last decades, and despite significant differences in pathophysiology and outcomes, both type 1 and 2 autoimmune pancreatitis are still considered different types of the same disease. Some have proposed a different nomenclature reflecting these differences. Although the term steroid-responsive pancreatitides may be interpreted as synonymous to type 1 and 2 autoimmune pancreatitis, these are not the only pancreatic conditions that show a response to steroid therapy. Acute pancreatitis caused by vasculitis and connective tissue diseases and acute pancreatitis secondary to checkpoint inhibitors or programmed cell death receptor antibody-mediated blockage cancer therapy may also benefit from steroid treatment. This review presents current concepts on these disorders, aiming to increase awareness, analyze similarities and differences, and propose a new nomenclature that reflects their specific particularities, clustering them under the term “steroid-responsive pancreatitides”.     

Immunopositive and immunonegative forms of chronic pancreatitis

The data are provided on the immune status in 108 patients with chronic pancreatitis. The authors revealed the immune responses toward pancreatic tissue, deoxyribonucleic acid and deoxyribonuclease. Based on the revealed autoimmune responses to the pancreatic tissue, two patterns of chronic pancreatitis were distinguished: the immunopositive and immunonegative. It is suggested that immunologic mechanisms are involved into the pathogenesis of the immunopositive pattern of chronic pancreatitis.     

自身免疫性胰腺炎误诊胰腺癌1例报告并文献复习

目的:探讨自身免疫性胰腺炎(AIP)的临床特点。方法:总结1例AIP患者临床表现、实验室、影像学、病理学检查及治疗情况。结果:患者,男,55岁,以上腹部不适疼痛伴黄疸2个月就医,误诊胰腺癌。经血清IgG 1 840 mg/dl、内镜逆行胰胆管造影(胰管头部线样不规则狭窄)、CT(胰腺体积增大,胰头明显)、MRI(胰腺弥漫异常改变)、病理学(胰腺间质纤维增生伴淋巴细胞浸润)证实为AIP,激素治疗反应好。结论:AIP临床表现缺乏特异性,影像学、血清学和胰腺病理学是诊断AIP的重要手段。     

自身免疫性胰腺炎32例分析

目的探讨自身免疫性胰腺炎(AIP)临床诊治经验。方法对32例AIP患者诊治的临床资料进行回顾性分析。结果 32例AIP中B超、CT诊断为胰胆癌肿28例,占87.5%,其中24例行剖腹探查术,占75.0%,4例仅行B超引导下穿刺活检未行手术。结论 AIP是一种特殊类型的慢性胰腺炎,其影像学特征与胰胆癌相似,易被误诊。临床医师应加强对本病的认识。