人类免疫缺陷病毒阴性的阴道残端浆母细胞淋巴瘤一例并文献复习

目的:浆母细胞淋巴瘤(PBL)为罕见的、侵袭性极强的B细胞淋巴瘤,好发于HIV阳性患者的口腔,本文报道了目前国内外首例HIV阴性的原发于阴道残端的PBL,并通过文献复习总结了PBL的临床病理学特征、诊断及鉴别诊断、治疗及预后。方法:回顾分析该病例的临床病理学资料,并结合国内外相关文献进行讨论。结果:该患者诊断明确,HIV阴性,以阴道残端起病,免疫组化示MUM-1阳性,不表达CD20、CD79a和PAX-5,Ki-67阳性率73%。EBER原位杂交阴性。分子学诊断结果示:样品免疫球蛋白基因发生克隆性重排,未检到TCR基因克隆性重排。CHOP样方案疗效不佳,部分缓解后疾病快速进展。该患者从确诊至死亡时间为10.3个月。结论:PBL罕见,病情进展迅速,预后差,生存期短,在进行阴道残端肿瘤的诊断及鉴别诊断时,阴道残端PBL应纳入鉴别范围。     

原发性乳腺淋巴瘤研究进展

原发性乳腺淋巴瘤(primary breast lymphoma,PBL)是一种罕见的结外型淋巴瘤,最常见的病理类型为弥漫大B细胞淋巴瘤,近年来隆胸相关的间变大细胞淋巴瘤亦被界定为新的临床类型。其与乳腺癌相比,临床表现和影像学检查均无特异性,需依靠病理活检及免疫组化明确诊断,预后较差。目前PBL尚无标准治疗方案,但趋向于以环磷酰胺+阿霉素+长春新碱+泼尼松(cyclophosphamide+doxorubicin+vincristine+prednisolone,CHOP)方案化疗为主的综合治疗方案,避免采用扩大的手术治疗。     

原发性胃T细胞淋巴瘤的临床病理分析

目的分析探讨原发于胃的外周T细胞淋巴瘤(Primary gastric T cell lymphoma,PTCL)的临床病理特征、免疫表型及分子生物学特点。方法回顾分析我院自2009~2012年收治的经免疫组化证实的PTCL患者4例。结合临床表现、形态学特征、发病机制和免疫表型,对其进行分析,并对鉴别诊断、治疗和预后的情况进行讨论。结果原发于胃的PTCL临床表现为腹胀、腹痛、腹部包块;镜下见形态较单一的肿瘤性淋巴细胞弥漫分布,穿插于肌间;免疫表型肿瘤细胞均表达T细胞标记,部分病例细胞毒标记(TIA-1)可阳性。治疗手段以手术切除辅以联合化疗为主,预后较差。结论 PTCL原发于胃非常罕见,这是一类异质性很强的肿瘤,必须结合临床、病理组织特征和免疫组织化学特征进行诊断和鉴别诊断。     

骨髓增生异常综合征合并非霍奇金淋巴瘤一例病例报道并文献复习

目的:探讨骨髓增生异常综合征合并非霍奇金淋巴瘤的发病机制、诊断及治疗,并判断其预后情况。方法:回顾我院收治的一例骨髓增生异常综合征(MDS)患者同时诊断间变大细胞淋巴瘤(ALCL)患者的临床资料,总结其诊断及治疗经过,对其预后进行评价。结果:该患者诊断间变大细胞淋巴瘤后,经化疗对症治疗病情无显著改善,且患者ALK阳性,考虑预后较差,建议造血干细胞移植。结论:骨髓增生异常综合征合并非霍奇金淋巴瘤的病例在国内外十分罕见,治疗方法目前尚需进一步研究。     

细胞蜡块诊断淋巴瘤18例

目的探讨18例浆膜腔积液中细胞学诊断淋巴瘤的临床病理学特征、免疫表型、诊断及鉴别诊断、治疗及预后。方法收集武汉大学人民医院2016年1月—2018年12月间由浆膜腔积液诊断为淋巴瘤患者18例,通过常规液基涂片、制作细胞蜡块、HE染色及免疫细胞化学染色,个别病例采用基因重排检测或流式细胞学检查,并部分对照其细胞学诊断和活检组织病理诊断的符合率。结果 18例均行免疫细胞化学检测,结果均为非霍奇金淋巴瘤。其中无淋巴瘤病史的有12例:包括4例弥漫性大B细胞淋巴瘤;1例淋巴浆细胞样淋巴瘤;1例浆细胞瘤;2例T淋巴母细胞淋巴瘤;1例NK/T细胞淋巴瘤;1例外周T细胞淋巴瘤,非特殊类型;2例T细胞淋巴瘤。其余6例均有淋巴瘤病史,其中4例为弥漫性大B细胞淋巴瘤,2例为血管免疫母细胞性T细胞淋巴瘤。结论浆膜腔积液是淋巴瘤的较常见并发症,细胞病理学诊断淋巴瘤难度虽相对较大,但通过制备细胞蜡块,在镜下观察肿瘤细胞成分单一,表现为大量散在分布的不成熟淋巴样细胞,细胞之间无黏附性,同时结合免疫细胞化学染色等相关检测方法,可明确诊断,值得推广。     

一种检测HIV携带者血液中抗原的改良方法

<正> 序言 AIDS系人免疫缺陷性病毒(HIV)所致。其诊断常采用HIV分离、逆转录酶活性测定和抗HIV抗体测定等三种方法。虽然抗体测定法是有用的,但在血清阳转之前,病毒或者抗原也许存在于抗体缺乏者体内,所以,抗体应答并非直接反映抗原物质。近来,HIV抗原试剂盒已有改进,使其能测定感染者在血清阳转之前血清中的抗原及HIV携带者的抗原物质。要了解HIV携带者的临床症状及预后,监测病毒及其抗原物质是有利的。然而,重     

非人类免疫缺陷病毒感染马尔尼菲青霉病9例临床特征分析及文献复习

为探讨和总结非人类免疫缺陷病毒(human immunodeficiency virus,HIV)感染者发生马尔尼菲青霉病的临床特点,回顾性研究复旦大学附属华山医院感染科2007年1月-2017年8月收治的9例及同期发表文献中的马尔尼菲青霉病病例,分析其临床表现、实验室检查、治疗及转归。9例马尔尼菲青霉病患者的HIV检测均为阴性,5例CD4^＋ T细胞计数正常。非HIV感染马尔尼菲青霉病的起病较缓,临床表现与经典马尔尼菲青霉病类似,真菌血症较少见,病理特点以肉芽肿改变及化脓性炎症为主,诊断方法仍以培养为主(8/9),1例通过病理及二代测序技术诊断。目前,非HIV感染马尔尼菲青霉病发病率有升高趋势,部分发生于免疫正常人群,需引起临床医师的重视。     

非HIV感染/艾滋病患者人肺孢子菌肺炎的临床和预后研究

目的研究非HIV感染者发生人肺孢子菌肺炎(PCP)的临床特点,感染的危险因素,治疗和预后。方法回顾性病例分析。结果在15个月内共诊断非HIV感染的PCP16例。患者的平均年龄为(51.9±23)岁。16例患者中,13例有免疫缺陷的基础疾病,其中结缔组织病者11例、非何杰金淋巴瘤者(NHL)1例、Good综合征者1例。在合并结缔组织病的患者中,所有PCP都发生在接受糖皮质激素治疗的过程中。16例PCP在诊断时都存在呼吸衰竭,其中11例需要气管插管,其余5例接受了无创机械通气治疗。平均急性生理和慢性病评分(APACHE II)为16±5。外周血淋巴细胞计数平均(955±635)/μl。9例患者有CD4+淋巴细胞计数结果,其中6例在诊断时CD4+淋巴细胞<250/μl。LDH平均(551.9±292.6)U/L。16例患者中14例在诊断后接受了TMP-SMZ治疗,除了2例患者外,其他患者同时还接受了糖皮质激素(相当于强的松≥60mg/d)辅助治疗。单因素分析显示有4种因素(高APACHEII评分、合并ALI/ARDS、延迟诊断、合并院内感染)是预后不良的危险因素。结论在免疫缺陷患者中,PCP是一种不太常见,但往往是致命的疾病。临床上的及时诊断和治疗对改善预后是非常重要的。     

Epstein-Barr病毒相关淋巴瘤的研究进展

Epstein-Barr病毒（Epstein-Barr virus,EBV）是第1个被发现与人类肿瘤发生相关的病毒,且越来越多的数据表明其感染与某些淋巴瘤的发生发展、治疗及预后密切相关。近年来免疫治疗手段发展迅速,但EBV阳性淋巴瘤的治疗目前主要仍以放化疗为基础,结合抗病毒药物。因此,加强EBV及其相关淋巴瘤的研究,寻找有效预防或治疗EBV感染的方法,将有望改善EBV阳性淋巴瘤患者的预后。     

肝脏假性淋巴瘤的临床病理学研究及分析

目的:探讨肝脏假性淋巴瘤的临床病理特征、免疫表型及鉴别诊断。方法:在光学显微镜下对肝脏假性淋巴瘤进行组织学形态观察,并借助免疫组化进一步对其形态进行分析。由于原发于肝脏的假性淋巴瘤极为罕见,故本文将报道一例发生于肝脏的假性淋巴瘤,结合文献探讨其临床病理特点,以提高诊断及鉴别诊断水平。结果:大体上为切面可见灰白结节,结节切面灰白色,质地中等,与周围分界清,周围肝组织灰红质软。显微镜下组织学表现为肝周边淋巴结淋巴组织增生,细胞大小较一致,核圆形,细胞无异型,未见明显核分裂,其中见嗜酸性白细胞浸润及少数异型细胞。免疫组化显示肿瘤细胞表达CD3、CD20及CD30。结论:肝脏假性淋巴瘤为罕见的、良性淋巴组织增生性病变,形态学特征、免疫组化染色在肝假性淋巴瘤诊断中具有重要价值。在临床病理实践中,必须首先与常见的发生于该部位恶性肿瘤如霍奇金淋巴瘤肿瘤等鉴别。     

Unusual presentation of plasmablastic lymphoma involving ovarian mature cystic teratoma: a case report

Background

Plasmablastic lymphoma (PBL) is relatively new clinical entity described as a distinct subtype of diffuse large B-cell lymphoma (DLBCL). It is characterized by its aggressive nature and proliferation of large neoplastic cells resembling immunoblasts including cells with more obvious plasmacytic differentiation. In this case report, we describe an unexpected finding of PBL associated with a mature cystic teratoma of the ovary in a young immune competent woman.

Case presentation

A 19-year old woman was admitted to the hospital with generalized lymphadenopathy, a pelvic tumor mass measuring 35?×?30 cm and a 4 cm lump in her right breast. She underwent a right salpingo-oophorectomy, lymphadenectomy, splenectomy, omentectomy, and a right breast lumpectomy. On macroscopic examination the right ovary was replaced by a thick-walled multilocular cystic tumor. Upon incision, the cysts were filled with thick, greasy sebaceous material and hair and there were several solid nodules within the cyst walls. Histological examination revealed a mature cystic teratoma and malignant non-Hodgkin lymphoma (NHL) within the solid nodules. Tumor tissue from the right breast, spleen and lymph nodes, all had the same histological, NHL morphology. After extensive immunostaining, a diagnosis of PBL was made. Following surgery, the patient was treated with different chemotherapy regimens, without any significant regression of the disease, and died of multiple organ failure.

Conclusions

Primary NHL of the ovary is relatively rare occurrence while secondary involvement by lymphoma is much more common. PBL is a rare lymphoma, primarily reported in the jaw and oral mucosa, but also documented in extra-oral sites. To the best of our knowledge, this is the first case described in a mature ovarian cystic teratoma. Although the patient was HIV-negative and immune competent, she had progressive disease and died despite aggressive chemotherapy 11 months after the initial diagnosis.

     

Primary malignant non-Hodgkin's lymphoma of the breast: a study of seven cases and literature review

ABSTRACT: INTRODUCTION: Primary breast lymphoma is an uncommon disease with poor clinical outcome. Breast lymphomas present less than 0.5% of malignant breast neoplasms and 2.2% of extranodal lymphomas. This study investigated the clinicopathological features and optimal treatment of PBL. Case presentations Clinical records of seven Moroccan PBL patients, treated at the National Institute of Oncology, Rabat, Morocco, from 2002 to 2010, were reviewed. Six of the patients were women and one a man, with ages ranging from 32 to 76. Five patients had stage IE and two stage IIE. All of the patients were classified with DLBCL. Of seven patients, one received a mastectomy and three excision of the breast lesion. Axillary dissection was performed in three patients. Two patients received chemotherapy followed by radiotherapy, while four received chemotherapy alone. Complete remission (CR) following primary treatment for all patients with PBL except in two cases was obtained. In one patient, recurrence occurred. CONCLUSIONS: There is no consensus on the question of how to best treat PBL: Mastectomy offers no benefit in the treatment of PBL. The combined therapy approach, with chemotherapy and radiotherapy, is the most successful treatment. PBL is poorly represented in rituximab-containing trials in DLBCL patients; there is not much experience with this agent in breast DLBCL. Because of the high incidence of central nervous system (CNS) involvement in PBL patients, many authors strongly believe that patients with aggressive forms of PBL should receive CNS infiltration prophylaxis.     

PBL教学模式在内科临床教学的应用探讨

内科是临床科室的重要组成部分,内科教学是为培养医务工作者的重要步骤,是医学生由学生向医生角色转变的桥梁。传统临床教学以知识灌输为主,学生学习积极性较低,教学质量较低。上世纪60、70年代问题导向(PBL)的教学方法被提出,为教学改革提供了一个全新的思路。本文将PBL教学方法应用于内科临床教学工作中,结合本学科的特点,以学生为中心,以问题为向导,充分发挥学生的学习积极性,提高教学质量,促进学生主动学习能力提高,帮助医学生建立完整的内科诊疗过程思维,进而达到临床内科教学目标。     

TEP/TDM au FDG et bilan initial des lymphomes : du diagnostic au pronostic

Lymphoma represents a very heterogeneous pathologic disease group and its initial assessment, treatment strategy and prognosis is closely related within each histological subtype. The diagnosis of lymphoma disease is often due to the development of a tumour mass at a lymph node area, or within an organ with or without systemic symptoms. However, all these signs remain non specific. Biopsy is the only reliable diagnostic tool, which can be carried out surgically or guided by the morphological and functional imaging. Once the histological diagnosis is determined by the WHO classification, the staging procedure aim is to provide the prognosis to assist the therapeutic decision. Morphologic imaging primarily based on CT remains the reference in exploring most cases. But in the last decade, the introduction of functional imaging using FDG-PET/CT has dramatically changed the therapeutic management of lymphoma. The additional clinical value provided by initial FDG-PET/CT could lead to an alteration of the therapeutic strategy, as well as to the optimization of radiation therapy or to the establishment of prognostic score and lead to improved lymphoma patient survival in the future.     

Clinical presentation and diagnosis of toxoplasmic encephalitis in Japan

Distinguishing life-threatening toxoplasmic encephalitis (TE) from brain lymphoma in patients with acquired immunodeficiency syndrome (AIDS) may be difficult. Empiric anti-toxoplasmosis treatment is often initiated because of the reluctance in performing brain biopsies, which may delay the diagnosis and treatment of brain lymphoma in Japan. In this study, we retrospectively examined the clinical characteristics of 13 AIDS patients with TE in Japan, including magnetic resonance imaging and thallium 201 (201TI) single photon emission computed tomography (SPECT) findings, cerebral spinal fluid analysis, serology, and polymerase chain reaction (PCR) results. All patients improved on anti-toxoplasmosis treatment. Of the 11 patients who underwent serological testing, 6 (55%) had a positive serological result. Of the 7 patients who underwent PCR testing, 3 (42.9%) had a positive PCR result. Nine of 11 patients with TE (81.8%) had multiple lesions. Analysis of the sites of TE lesions did not reveal a difference in site predilection between TE and brain lymphoma. Uptake was negative in all 9 patients who underwent 201Tl SPECT. The study findings suggest that toxoplasma serostatus and PCR may be used to discriminate TE from brain lymphoma. No focal accumulation of 201TI is strongly suggestive of TE in patients with AIDS in Japan.     

Primary cardiac lymphoma initially diagnosed by routine cytology. Case report and literature review

A case of primary cardiac lymphoma initially diagnosed by routine cytologic examination of pericardial fluid is presented. In a 64-year-old woman woman who originally presented with chest pain and heart block, the initial clinical impression was ischemic heart disease. However, coronary angiography failed to reveal significant disease. An echocardiogram demonstrated pericardial fluid, which was drained. A small amount was sent for cytologic examination, and the diagnosis of malignant lymphoma, large cell type, was made. Subsequent radiologic examinations revealed an intracardiac mass involving the atrioventricular canal; surgical biopsy confirmed the diagnosis of a large cell lymphoma. While primary malignant lymphoma of the heart is rare, this case highlights the efficacy of routine cytologic examination of an effusion fluid (often drained therapeutically) in establishing the correct diagnosis.     

Specific cytolysis of fresh tumor cells by an autologous killer T cell line derived from an adult T cell leukemia/lymphoma patient

Cytotoxic T cells (Tc) derived from one patient with adult T cell leukemia/lymphoma (ATL) killed fresh autologous lymphoma cells in vitro. The Tc were induced from peripheral blood leukocytes (PBL) of this patient during remission by multiple in vitro stimulations with an autologous ATLV-bearing cell line (ILT) that was previously established by cloning of PBL in the presence of interleukin 2 (IL 2). PBL from eight other ATL patients were stimulated in the same manner, and responder cells from a patient in remission also showed cytotoxicity specific for ATL virus (ATLV)-bearing cells. Fresh lymphoma cells were obtained in relapse and were used as target cells for the autologous Tc induced. They became susceptible to the Tc within 4 hr of in vitro incubation, and their susceptibility increased with incubation time for at least 12 hr. ATLV antigens on the cell surface of these lymphoma cells, however, were not detected by radioimmunoassay during these incubation periods, but were detectable after 16 hr of incubation. In addition, cytotoxicity against lymphoma cells was completely inhibited by autologous ILT cells used as "cold" target competitor cells. These findings indicate that the target antigen of the Tc was expressed on both autologous ILT cells and lymphoma cells, and it may be different from ATLV antigens detected by serologic methods. In addition, the data suggested allogeneic restriction of the Tc in that the preferentially killed allogeneic ATLV-bearing cells share several HLA antigens.     

T cell-lymphoma in the eyelid of a 9-year-old English Setter

Background

Eyelid tumours are frequently found in dogs, most of these being benign. In case of an ulcerating eyelid tumour, malignancy must be considered. We report a unique case of a low-grade peripheral T-cell lymphoma in the eyelid of a 9-year-old English Setter.

Case presentation

A 9-year-old Setter presented with a 6-month history of an eyelid ulcer. A malignant eyelid neoplasm was suspected, and the lesion was surgically excised. No other treatment was applied, and 19 months after excision the dog was still well. Histopathology revealed a diffuse lymphocytic infiltrate in the eyelid skin. Ulceration of the epithelium was seen, and the underlying tumour was composed of round and poorly demarcated pleomorphic tumour cells. The cytoplasm was pale and the nuclei heterogeneous. Numerous mitoses were present. The tumour cells stained strongly for CD3. The final diagnosis was a peripheral T-cell lymphoma not otherwise specified (NOS).

Conclusions

This is the first described case of a solitary T-cell lymphoma NOS in the haired eyelid skin in a dog. Lymphoma should be considered in case of a persistent eyelid ulcer and a biopsy should be performed. T-cell lymphoma is generally an aggressive disease; however, indolent cases are well known, and as this case shows, complete excision of a solitary T-cell lymphoma can be curable. Canine cutaneous epitheliotropic T-cell lymphoma is an important differential diagnosis, which must be recognized as the prognosis is very poor and systemic treatment is mandatory. The sub-classification of canine lymphoma is not complete, and further studies are needed to identify lymphoma subgroups and provide treatment guidelines.