肾黏液样小管状和梭形细胞癌临床病理观察

目的:探讨肾黏液样小管状和梭形细胞癌(mucinous tubular and spindle cell carcinoma,MTSCC)的临床病理特征、诊断、鉴别诊断及组织起源。方法:对3例肾黏液样小管状和梭形细胞癌进行临床病理学特征及免疫组化分析。结果:2例患者女性,年龄分别49岁和56岁,1例患者男性,51岁。3例患者均无临床特异症状。体检发现肾占位性病变。巨检:肿瘤大小3.5cm×3cm×2.5cm-7cm×6cm×5cm,切面灰白、浅褐色,质地均匀。镜下见瘤细胞主要呈两种结构:由单层立方细胞排列呈大小不一管状结构、缎带样及类似于平滑肌瘤的梭形细胞结构,立方状细胞及梭形细胞均形态温和。背景中见大量黏液,并可见灶状泡沫细胞、淋巴细胞浸润。免疫组化:CK、AE1/AE3、Vim、EMA阳性表达;CD10、SMA、HMB45、SYN均阴性表达。结论:MTSCC是一种少见的低级别恶性肿瘤,预后较好,可能来源于远端肾单位。     

急性混合细胞白血病合并母细胞性浆细胞样树突细胞肿瘤一例并文献复习

目的 提高对急性混合细胞白血病(MAL)合并母细胞性浆细胞样树突细胞肿瘤(BPDCN)的认识.方法 通过报道1例MAL合并BPDCN患者并复习相关文献,分析BPDCN的临床特点、治疗方案及预后.结果 患者为中年女性,临床表现脾大、淋巴结肿大,骨髓涂片可见肿瘤细胞呈母细胞形态,形态单一,核不规则,染色质细,可见数个小核仁,胞质量少,无颗粒状且嗜碱性,骨髓免疫分型可见30.79 %的细胞CD56-、CD4-、CD123st+、HLA-DRst+、BDCA2+,为异常浆细胞样树突细胞,根据相关评分系统,表达CD123及BDCA2,总分为3分,可诊断为BPDCN.患者有长期服用硫唑嘌呤以及血小板减少的病史,染色体核型检测发现存在7号染色体缺失,提示该患者有可能存在骨髓增生异常综合征病史,骨髓细胞免疫分型提示同时存在髓系幼稚细胞及异常单核细胞的表达,急性粒-单核细胞白血病诊断明确.结论 MAL合并BPDCN的诊断需综合临床表现、形态学、免疫学、分子遗传学等,应争取对疾病尽早诊断及治疗.     

发热病人外周血及骨髓异形细胞的识别

[目的]探讨发热病人外周血及骨髓中异形细胞的临床意义.[方法]以瑞氏染色和二步法非生物素免疫组化染色相结合识别发热病人外周血及骨髓中的异形细胞并确定细胞性质.[结果]26例发热病人中,19例在外周血出现4%～48%、22例在骨髓出现3%～30%异形细胞.按瑞氏染色主要分为四类:异形淋巴样细胞(8例)、幼稚单核样细胞(6例)、组织细胞样细胞(9例)、淋巴瘤样细胞(3例).经免疫组化染色标记异形淋巴样、组织细胞样、淋巴瘤样异形细胞分别表达T淋巴细胞、组织细胞及淋巴细胞标志,而幼稚单核样细胞多为淋巴细胞、少数为组织细胞标志.异形淋巴样细胞和幼稚单核样细胞在病毒感染性疾病和系统性红斑狼疮患者为CD8 T细胞,在组织细胞性坏死性淋巴结炎多为CD4 T细胞.非霍奇金淋巴瘤患者骨髓可无浸润而组织细胞增生,伴噬血现象.恶性组织细胞病患者可见异形淋巴样细胞、幼稚单核样细胞,实质上为异形恶性组织细胞.[结论]瑞氏染色和免疫组化染色标识异形细胞对发热病人具有鉴别诊断意义.     

5例食管基底细胞样鳞状细胞癌组织化学及免疫组化观察

目的：探讨食管基底细胞样鳞状细胞癌(BSCC)的组织化学、免疫组化特征及在病理诊断中的作用方法：收集5例食管BSCC分别作HE、组织化学及免疫组化染色，同时收集5例典型鳞状细胞癌(SCC)作对照结果：BSCC主要由密集的小细胞组成，排列成实体小叶状及假腺样(筛网状)，并见粉刺样坏死及基底膜样物质沉积(PAS )，4例BSCC均见不同分化程度的鳞状细胞癌成分．1例见局灶性鳞状细胞分化免疫组化基底细胞样成分Ker(AE1／AE3)、Ker14、PCNA、LM及p53均阳性，Ker194例基底细胞样成分及2例伴随鳞癌成分同时阳性：Syn仅1例BSCC阳性；5例SCC Ker(AE1／AE3)均阳性．3例Ker14弱阳性，2例Ker19弱阳性5例BSCC和BCC其S-100、CgA、NSE及SMA均阴性。结论：BSCC是鳞状细胞癌独特的组织学类型，进行组织化学及免疫组化染色有助于BSCC诊断及鉴别诊断。     

miR-128在急性淋巴细胞白血病中的表达

目的 探讨miR-128在急性淋巴细胞白血病(ALL)中的表达及其意义.方法 采用实时荧光定量聚合酶链反应法对62例ALL患者和20例骨髓正常患者骨髓单个核细胞miR-128的相对表达量进行检测,并与患者的临床资料进行相关性分析.结果 miR-128在ALL患者中的相对表达量高于健康对照组(P＜0.05),但其在急性B淋巴细胞白血病(B-ALL)及急性T淋巴细胞白血病(T-ALL)中的表达水平差异无统计学意义(P＞0.05),miR-128的表达与是否存在bcr-abl融合基因及其mRNA的表达水平均无相关性(均P＞ 0.05).结论 miR-128在ALL患者中表达上调,可作为临床诊断ALL的潜在分子标志物,bcr-abl融合基因的表达对miR-128在ALL中的表达无明显影响.     

扁桃体上皮样滤泡树突细胞肉瘤临床病理学分析

目的:探讨扁桃体上皮样滤泡树突细胞肉瘤(follicular dendritic cell sarcoma,FDC sarcoma)的临床病理学特点、免疫表型、超微结构以及鉴别诊断.方法:对1例发生于扁桃体的上皮样FDC肉瘤进行组织学观察、免疫组化标记及电镜观察.结果:患者女性,55岁.咽部不适感2年,近期症状加重而就诊.术中见左扁桃体肿大伴有糜烂.临床考虑为恶性淋巴瘤.镜下见瘤细胞形态较一致,由大上皮样细胞组成,未见梭形瘤细胞.瘤细胞境界不清,丰富的嗜酸性胞质,空泡状核,核仁明显.瘤细胞间散在小淋巴细胞浸润.免疫组化标记显示瘤细胞弥漫性表达vimentin、CD21、CD23、CXCL13和D2 - 40.电镜观察于瘤细胞胞浆内可见粗面内质网和线粒体,少量由桥粒连接的细胞突,明显的椭圆形核及核内的大核仁.肿块切除后随访4个月无复发及转移.结论:扁桃体上皮样亚型的滤泡树突细胞肉瘤非常罕见.诊断时应与多种具有上皮样形态的肿瘤鉴别.     

食管基底细胞样鳞癌的诊断与治疗

目的 观察食管基底细胞样鳞癌的临床和病理组织学特征,探讨其诊断、鉴别诊断及治疗方法.方法 对23例食管基底细胞样鳞癌患者的临床和病理资料进行回顾性分析.结果 食管基底细胞样鳞癌发病年龄较大,临床表现和影像学特点与鳞癌相似,以溃疡型多见;治疗首选手术,术后应结合放化疗.本组23例食管基底细胞样鳞癌患者的1、2和3年生存率分别为60.9%、21.7%和0.结论 食管基底细胞样鳞癌具有恶性程度高、进展快、转移早和预后差等特点,临床上应采用根治性手术并结合术后放化疗的治疗方案.     

肝脾T细胞淋巴瘤临床特征分析(附4例)

目的:探讨肝脾T细胞淋巴瘤（hepatosplenic T-cell lymphoma,HSTCL）的临床表现、病理学特征、免疫表型特点。方法:回顾性分析2014年9月-2021年12月我院收治的4例肝脾T细胞淋巴瘤（HSTCL）患者临床资料。结果:4例患者中男性3例,女性1例,诊断时平均年龄为55岁,所有患者均有脾大,其中3例有骨髓受累,4例患者均经脾脏病理活检和/或骨髓免疫分型明确诊断为HSTCL,其中2例为γδ型,1例为αβ型,1例不确定亚型,4例均合并TCR基因重排,1例合并7号染色体异常,4例患者从有临床症状到确诊时间平均时间为10.3月。结论:肝脾T细胞淋巴瘤在老年患者中有一定比例,临床易误诊、漏诊,脾脏病理联合脾脏制备单个核细胞行免疫分型可能提高敏感性,骨髓受累时免疫分型为明确诊断的重要指标;该型淋巴瘤呈侵袭性进展,预后差。     

细胞芯片法在急性白血病骨髓样本免疫分型中的应用

目的:应用急性白血病免疫分型诊断的细胞芯片对急性白血病患者的骨髓样本进行免疫分型,从而快速诊断白血病的类型.方法:将细胞悬液滴于细胞芯片,那些有相应CD抗原的细胞只能与相应的单克隆抗体的点结合,进行Wright's染色,并对65例临床白血病骨髓样本进行分型并验证结果.结果:本实验所得的免疫分型结果与流式细胞仪的白血病免疫分型结果一致.结论:本实验使用的细胞芯片可作为临床白血病免疫学分型的诊断依据.     

B细胞型幼淋巴细胞白血病1例并文献复习

目的:提高对B细胞型幼淋巴细胞白血病（B-PLL）的诊治认识。方法:回顾分析了我院1例B-PLL患者的临床表现、骨髓细胞形态学、免疫分型、不良基因检测等临床资料以及治疗过程,并结合文献进行学习讨论。结果:B-PLL患者初诊时白细胞总数较高,脾肿大;骨髓形态学呈体积中等的幼稚淋巴细胞,核圆、核仁清晰、核染色质浓密、核浆比例低的特点;成熟B细胞免疫表型,免疫球蛋白轻链限制性表达,排除其他淋巴增殖性疾病,诊断为B细胞型幼淋巴细胞白血病。该患者TP53缺失,给予R-CHOP方案治疗,治疗反应差。结论:形态学检查和免疫表型是诊断B-PLL的重要手段,同时需整合临床表现、细胞遗传学等结果综合判断,TP53基因缺失或突变会导致这类患者传统化疗的预后不良。     

Gaucher disease and multiple myeloma

Gaucher disease (GD) is the most frequent lysosomal storage disease and corresponds to an inherited deficiency of glucocerebrosidase. Due to excessive accumulation of glucocerebroside in bone marrow, both cytopenia and bone lesions may occur. The incidence of malignant disorders has been evoked in non-neuronopathic type I GD. More particularly, many case reports have been published that describe the association between GD and multiple myeloma (MM). Here, we first deal with diagnosis criteria that allow to distinguish between bona fide Gaucher celles and the so-called pseudo or pseudo-pseudo Gaucher cells. We then analyse relevant case reports and recent articles that provide convincing data regarding GD and MM association and suggest physiopathological links between the two disorders.     

Adult Gaucher disease in association with primary malignant bone tumors

BACKGROUND: Malignant neoplastic disorders are more common in patients with Gaucher disease (GD) than in the general population. Very few cases of primary malignant bone tumors in association with GD have been reported to date. Thus, the recommendations for an adequate therapy are often based on limited professional experience. To their knowledge, the authors report the first case of a leiomyosarcoma of the bone in a patient with GD and report another patient with GD and an anaplastic large cell non-Hodgkin lymphoma with localized osseous manifestation. A review of the literature is included. METHODS: The clinical, radiologic, and histologic data of the authors' two patients who had GD and primary malignant bone tumor are presented. Epidemiologic data, clinical data, and treatment results from published reports of 18 patients who had GD and various malignancies and the authors' 2 patients were compared and evaluated. RESULTS: Radiographic examination showed a destructive osteolytic lesion in a case of a leiomyosarcoma of the bone and in a case of anaplastic, large-cell, non-Hodgkin lymphoma. In both cases, the bone marrow architecture was partially effaced by sheets of large histiocytic cells with striated or fibrillary cytoplasm. In both patients, chemotherapy was performed. Whereas the patient who had the leiomyosarcoma showed poor recovery of the bone marrow that necessitated withdrawal of aggressive chemotherapy, the patient who had non-Hodgkin lymphoma and enzyme therapy tolerated the chemotherapy well. In spite of local control after preoperative radiotherapy and hemipelvectomy, the first patient developed lung metastases and finally died. The second patient was continuously free of disease at a follow-up examination 32 months after chemotherapy and radiotherapy. In a total of 20 patients who had malignant disorders and GD, the numbers of males and females were equivalent, and the mean age was 55 years. Approximately 33% presented with a cancer originating in the bone. In 16 patients, follow-up data were available. Of these, after a mean follow-up of 36 months (range, few days-108 mos), only 2 patients were continuously free of disease, and one patient was alive with disease. The other patients had died of disease or hemorrhagic complications of GD. CONCLUSIONS: Because there is a relatively high incidence of malignant disorders of the bone, the study suggested that malignant disorders have to be included in the differential diagnosis of painful lytic lesions in patients who have GD. Evaluation of the expense and value of enzyme therapy to patients who have GD should be undertaken with regard to the incidence of malignant disorders and patient survival.     

Gaucher disease type 1: Unexpected diagnosis in a 75-year old patient presenting with splenomegaly

We present a 75-year old Korean female patient harboring novel hemizygous variant mutation in glucosidase beta acid (GBA) gene, who was diagnosed with splenic marginal zone cell lymphoma and Gaucher disease (GD) concurrently. Our case is significant in that (1) it delivers the message that GD can occur at any age regardless of ethnicity and (2) we report a novel variant of pathogenic GBA mutation, and the fact that the patient harbored hemizygous mutation.     

CIK、DC-CIK细胞对神经母细胞瘤细胞杀伤作用的研究

目的：研究细胞因子诱导的杀伤细胞（ CIK）与树突状细胞（ DC）共培养后对神经母细胞瘤（ neuro-blastoma,NB）细胞株的杀伤作用。方法：取健康人和肿瘤患者外周血单个核细胞（ PBMC）,加入不同的细胞因子分别诱导出DC和CIK细胞,用流式细胞术测定诱导培养前后DC和CIK细胞的表型,MTT法测定不同组CIK细胞对NB细胞株的杀伤活性。结果：流式细胞仪检测健康人PBMC培养后CD3＋CD56＋淋巴细胞百分比以及对NB细胞株的杀伤活性均显著高于肿瘤患者（ P〈0.05）。此外,与单纯CIK细胞相比,DC-CIK细胞具有更强的杀伤NB细胞株的活性（ P〈0.05）。结论：DC-CIK细胞是一种细胞毒作用高于单纯CIK细胞的免疫活性细胞。健康人和肿瘤患者的PBMC经诱导培养获得的CIK细胞有显著差别,为临床进一步提高CIK细胞的治疗效果提供了实验依据。     

造血细胞与内皮细胞的相互关系及其研究现状

 造血细胞和内皮细胞的相互关系已越来越为人们所重视,成为目前研究十分活跃的领域。造血细胞与内皮细胞表达某些共同的表面标记和基因,且两者能相互促进发育,内皮细胞在诱导造血细胞归巢方面也有一定作用,研究两者关系有较高的科研及临床应用价值,文章介绍了造血细胞和内皮细胞相互关系的研究现状。     

Chemoresistant colorectal cancer cells and cancer stem cells mediate growth and survival of bystander cells

Background:

Recent studies suggest that cancer stem cells (CSCs) mediate chemoresistance, but interestingly, only a small percentage of cells in a resistant tumour are CSCs; this suggests that non-CSCs survive by other means. We hypothesised that chemoresistant colorectal cancer (CRC) cells generate soluble factors that enhance survival of chemonaive tumour cells.

Methods:

Chemoresistant CRC cells were generated by serial passage in oxaliplatin (Ox cells). Conditioned media (CM) was collected from parental and oxaliplatin-resistant (OxR) cells. CRC cells were treated with CM and growth and survival were assessed. Tumour growth rates were determined in nude mice after cells were treated with CM. Mass spectrometry (MS) identified proteins in CM. Reverse phase protein microarray assays determined signalling effects of CM in parental cells.

Results:

Oxaliplatin-resistant CM increased survival of chemo-naive cells. CSC CM also increased growth of parental cells. Parental and OxR mixed tumours grew larger than tumours composed of parental or OxR cells alone. Mass spectrometry detected unique survival-promoting factors in OxR CM compared with parental CM. Cells treated with OxR CM demonstrated early phosphorylation of EGFR and MEK1, with later upregulation of total Akt .We identified progranulin as a potential mediator of chemoresistance.

Conclusion:

Chemoresistant tumour cells and CSCs may promote resistance through soluble factors that mediate survival in otherwise chemosensitive tumour cells.     

Contribution of stem cells and differentiated cells to epidermal tumours

The outer covering of the skin--the epidermis--is subject to sustained environmental assaults. As a result, many cells acquire potentially oncogenic mutations. Most cells are lost through differentiation, and only long-term epidermal residents, such as stem cells, accumulate the number of genetic hits that are necessary for tumour development. So, what genetic and environmental factors determine whether a mutant stem cell forms a tumour and what type of tumour will develop?     

Immunogenic reactivity of CTLs induced by electrofusion cells of human dendritic cells and gastric cancer cells

In tumor immunotherapy, there were several reports of attempts to induce anti-tumor immunity by fusion hybrid cells generated with dendritic and tumor cells. One of them reported that vaccination of hybrid cells resulted in a remarkable reduction of tumor cells in a lab mouse experiment. In our study, fusion cells were generated successfully with human matured dendritic and human gastric cancer cells by electrofusion technique and employed to induce CTLs. The evaluated fusion rate was 47.8% by FACS analysis. We tried to induce CTLs by co culture of effector and stimulator cells in the presence of IL-2, IL-7 and IL-12 for 4 weeks. Although it was not statistically significant in tumor cytotoxic assay, effector cells induced by the fusion cells as stimulator cells showed a few cytotoxic responses in an immunological tumor specific manner. Our data suggest that fusion hybrid cells may facilitate stimulation and expansion of tumor-specific T cells, but further investigation is required for clinical application of fusion cells in adoptive immunotherapy.