原发性肺结外鼻型NK/T细胞淋巴瘤一例临床分析

目的报道国内第一例肺部原发性结外鼻型NK/T淋巴瘤,分析原发性肺鼻型NK/T淋巴瘤的临床特点和诊断治疗。方法通过1例病理证实的原发性肺结外鼻型淋巴瘤的病例分析,结合文献,对原发性肺NK/T淋巴瘤的免疫表型、发病机制、临床特点、影像学表现、诊断、治疗及预后进行分析。结果NK/T细胞淋巴瘤是一种新近认识的恶性淋巴瘤,原发于肺部的比较罕见,具有特殊的免疫表型与临床病理特点,EB病毒在其发病中扮演重要角色。此瘤恶性程度很高,疾病进展快,治疗效果及预后差。结论原发性肺结外鼻型NK/T淋巴瘤临床罕见,诊断难度大,预后差,目前缺少有效的治疗方法。     

以急性呼吸窘迫症为表现的原发性肺NK/T细胞淋巴瘤1例临床分析

目的报道1例以急性呼吸窘迫症为表现的原发性肺NK/T细胞淋巴瘤,分析NK/T细胞淋巴瘤的临床特点和诊断治疗。方法通过1例骨髓病理证实的原发性NK/T淋巴瘤的病例分析,结合文献,对原发性NK/T淋巴瘤的发病机制、临床特点、诊断、治疗及预后进行分析。结果 NK/T细胞淋巴瘤原发于肺部且以急性呼吸窘迫为表现比较罕见,具有特殊的免疫表型和临床症状特点,此病恶性程度高,疾病进展快,治疗效果及预后差。结论原发性肺NK/T淋巴瘤临床罕见,诊断难度大,预后差,目前缺少有效治疗方法。     

肺黏膜相关淋巴组织淋巴瘤的免疫表型和诊断标准

肺黏膜相关淋巴组织(MALT)淋巴瘤是非常少见的一种肺的原发性低度恶性淋巴瘤，其病因不清，瘤细胞表达B细胞抗原，在病理学上符合其他部位MALT型淋巴瘤的一般特点，诊断需结合组织病理学、免疫学检测和临床特点。施行局部切除、化疗、低剂量的放疗或综合治疗，预后良好。     

肺黏膜相关淋巴组织淋巴瘤的免疫表型和诊断标准

肺黏膜相关淋巴组织 (MALT)淋巴瘤是非常少见的一种肺的原发性低度恶性淋巴瘤 ,其病因不清 ,瘤细胞表达B细胞抗原 ,在病理学上符合其他部位MALT型淋巴瘤的一般特点 ,诊断需结合组织病理学、免疫学检测和临床特点。施行局部切除、化疗、低剂量的放疗或综合治疗 ,预后良好。     

原发性肺淋巴瘤22例临床与病理学相关特征分析

目的探讨并分析原发性肺淋巴瘤患者临床表现、病理学特征。方法回顾经病理组织诊断为原发性肺淋巴瘤的患者22例相关资料,分析其临床表现、影像学表现、病理检查结果以及随访相关资料。结果 22例原发性肺淋巴瘤患者临床表现主要为:20例咳嗽(90.91%)、18例(81.82%)发热、15例(68.18%)胸部疼痛、12例(54.55%)体重减轻;CT影像学表现主要为实变影、肿块影、结节影;病理检查可见B淋巴细胞大量浸润,B淋巴细胞相关抗原阳性表达;大部分患者预后较好,少数患者预后较差。结论原发性肺淋巴瘤临床表现以及影像学表现无特异性,其诊断主要依靠病理检查,T细胞淋巴瘤患者恶性程度高,预后差,其余患者预后较好。     

肺原发性支气管相关淋巴组织淋巴瘤临床分析

目的 探讨肺原发性支气管相关淋巴组织（BALT）淋巴瘤的临床诊断及治疗,提高对BALT淋巴瘤的认识.方法 分析肺原发性BALT淋巴瘤的临床特点、影像学表现、病理特征、临床诊断,并复习文献.结果 患者以反复咳嗽、咳痰伴胸闷、气促为其特点;胸部CT检查显示：双肺多发大片实变影,右侧胸腔积液;CT引导下经皮肺穿刺活检病理检查结果：黏膜相关淋巴组织结外边缘带小B淋巴细胞淋巴瘤,免疫表型：CD20、BCL-2、ki-67、PAX-5均阳性;CD3、CD5、CD10、CD23、CD43、cyclin-D1、MPO等表达均阴性.结论 BALT淋巴瘤临床罕见,属于低度恶性B细胞淋巴瘤,依靠组织细胞学病理、免疫表型与临床、影像学相结合可明确诊断.     

原发性骨恶性淋巴瘤4例临床分析并文献复习

目的：探讨原发性骨恶性淋巴瘤(PBL)的特点、治疗及预后。方法：4例PBL均经手术取活检，病理明确诊断，给予放、化疗治疗。结果：4例患者对化疗和放疗敏感，达到完全缓解。结论：PBL多为B细胞性非霍奇金淋巴瘤，可侵犯骨的任何部位，治疗主要是化疗结合局部放疗，是一种预后较好、有可能被治愈的疾病。     

肺原发性霍奇金淋巴瘤临床病理分析

目的 探讨肺原发性霍奇金淋巴瘤的诊断与鉴别诊断.方法 回顾性分析3例肺霍奇金淋巴瘤病例并复习相关文献.结果 3例患者均为男性,以咳嗽症状为主,肿瘤较大,平均直径为5 cm,可伴有肺门及纵隔淋巴结肿大,镜下均可见典型的RS细胞,背景细胞内有较明显嗜酸粒细胞浸润,并见干尸细胞,免疫组化CD15和CD30阳性.化疗、骨髓移植可使病情缓解,长期生存.结论 肺原发性霍奇金淋巴瘤病理学及免疫表型具有一定的特征,恰当的治疗预后良好.     

原发性肝黏膜相关淋巴组织淋巴瘤临床病理特征

目的：探讨原发性肝黏膜相关淋巴组织(MALT)淋巴瘤的临床病理特征及治疗方法。方法：对1例原发性肝MALT淋巴瘤的发病因素、临床表现、病理特征及治疗结果进行分析。结果：腹部B超检查发现肝左叶低回声区。腹部MRI检查示肝脏恶性占位性病变，行肝左叶切除术，病理检查示正常肝小叶结构破坏，被弥漫分布的肿瘤细胞替代，瘤细胞以单核样细胞为主，伴有淋巴上皮病变形成，免疫组化示，CD45、CD20、CD79a阳性，CD5、CD10、ALK、TdT阴性，HCV、HBV和EBV阴性。结论：原发性肝MALT淋巴瘤属低度恶性B细胞淋巴瘤，有独特的临床、组织病理学和免疫表型特点。治疗上多采取手术局部切除治疗，部分需加用联合化疗。     

北京协和医院对18年间病理诊断肺淋巴瘤患者的回顾分析

目的回顾性总结分析肺淋巴瘤的分类、分型特征及临床特点。方法收集北京协和医院1999年-2016年近18年期间病理诊断为肺淋巴瘤的全部病例,通过光镜、免疫组织化学染色及基因重排方法进行病理分析。结果本组共有肺淋巴瘤病例142例,男女比例1︰1.33,平均年龄48岁。123例(86.6%)诊断为非霍奇金淋巴瘤,19例(13.4%)诊断为霍奇金淋巴瘤。101例(69.2%)诊断为B细胞性淋巴瘤,其中45.6%(46例)为黏膜相关淋巴组织淋巴瘤或边缘区淋巴瘤,18.8%(19例)为弥漫大B细胞性淋巴瘤,11.9%(12例)为淋巴瘤样肉芽肿病。22例诊断为T细胞性淋巴瘤,其中27.3%为外周T细胞淋巴瘤(6例),13.6%(3例)为NK/T细胞淋巴瘤,9.1%(2例)为间变大细胞淋巴瘤。121例患者临床资料完善,其中64例(52.9%)为原发性肺淋巴瘤,男女比例1︰1.37,平均年龄50岁;57例(47.1%)为继发性肺淋巴瘤,男女比例1︰1.19,平均年龄45岁。在原发性肺淋巴瘤中,96.9%为非霍奇金淋巴瘤,3.1%为霍奇金淋巴瘤;在继发性肺淋巴瘤中,78.9%为非霍奇金淋巴瘤,21.1%为霍奇金淋巴瘤。65例手术切除或胸腔镜下肺活检病例全部进行了病理分类及分型,77例穿刺活检病例中有22例(28.6%)进一步分型困难。结论肺淋巴瘤中最常见类型依次为黏膜相关淋巴组织淋巴瘤、霍奇金淋巴瘤、弥漫大B细胞性淋巴瘤和淋巴瘤样肉芽肿病,其中黏膜相关淋巴组织淋巴瘤和淋巴瘤样肉芽肿病是主要的原发性肺淋巴瘤类型,而弥漫大B细胞性淋巴瘤和霍奇金淋巴瘤是主要的继发性肺淋巴瘤类型。开胸手术或胸腔镜下肺活检的病例易于明确病理分型,而穿刺活检病例中有小部分病例进一步分型困难。     

Three cases of primary pulmonary malignant lymphoma]

Primary pulmonary malignant lymphoma is a rare disease that is thought to belong to a category of malignant lymphomas arising from mucosa- or bronchus-associated lymphoid tissue (MALT or BALT). We encountered 3 cases of primary pulmonary malignant lymphoma, Case 1: In a 51-year-old male, an abnormal shadow was detected in chest radiography in the right S9 after an operation for thyroid carcinoma. A right lower lobectomy was performed. The diagnosis was malignant lymphoma (marginal zone B-cell lymphoma). Immunohistochemical staining for IgM gave a positive result. Case 2: Multiple nodular shadows were noted in both lungs of a 55-year-old man after a bout of pneumonia. Video-assisted thoracoscopic surgery was performed, and the diagnosis was malignant lymphoma (marginal zone B-cell lymphoma). Gene analysis revealed rearrangement of a heavy chain gene. Case 3: An abnormal shadow was seen in the chest radiograph of a 60-year-old man. He was treated by right upper and middle lobectomy. The diagnosis was Hodgkin's disease, nodular sclerosing type. Chemotherapy was given after surgery and the patient is now alive without recurrence. As the pulmonary malignant lymphoma was difficult to diagnosepreoperatively, it was necessary to resect the mass for diagnostic purposes. The prognosis of a resected solitary lesion in the lobe was good. Therefore lobectomy was performed as the treatment of choice. Systemic chemotherapy is performed for the diffuse type of pulmonary lymphoma.     

Practice guidelines for the management of extranodal non-Hodgkin's lymphomas of adult non-immunodeficient patients. Part I: primary lung and mediastinal lymphomas. A project of the Italian Society of Hematology, the Italian Society of Experimental Hematology and the Italian Group for Bone Marrow Transplantation

Extranodal non-Hodgkin's lymphomas constitute 20-25% of overall non-Hodgkin's lymphomas cases and can be managed with very different therapeutic strategies. Therefore, the Italian Society of Hematology and the two affiliate societies (the Italian Society of Experimental Hematology and the Italian Group of Bone Marrow Transplantation) appointed a panel of experts to produce clinical practice-guidelines for the management of these conditions. Primary lung and mediastinal lymphomas were the objective of this part of the project. The panel of experts produced the following key recommendations that were graded according to the strength of evidence and clinical judgement. The first-line therapy for non-MALT primary lung non-Hodgkin's lymphomas should include anthracycline-based chemotherapy with CHOP or CHOP-like, MACOP-B or MACOP-B-like regimens (grade D). Rituximab association with chemotherapy needs to be evaluated within approved clinical trials. Second-line therapy with high-dose chemotherapy and autologous stem cell transplantation is recommended (grade B). In patients with MALT primary lung non-Hodgkin's lymphomas, the recommended first-line therapy should include chlorambucil, CHOP, CHOP-like or fludarabine-containing regimens (grade B). Radiotherapy is to be reserved for patients with a unique, small lesion in a poorly mobile site and with contraindication to surgery (grade D). Rituximab should be administered only within approved clinical trials. For treatment of primary mediastinal large B-cell lymphomas, the recommended first-line therapy is a chemotherapy and radiotherapy association (grade B). An anthracycline-based chemotherapy with CHOP, MACOP-B or VACOP-B is recommended (grade B). Rituximab combination with chemotherapy is highly suggested but only for patients enrolled into approved clinical trials. Patients with an inadequate early response should be candidates for early intensification with high-dose chemotherapy (grade C). Patients with refractory or relapsed disease should undergo rescue programs including intensive, non-cross-resistant debulking treatment followed, in chemosensitive patients, by high-dose chemotherapy and autologous stem cell transplantation (grade B).     

Monoclonal B Cells in Peripheral Blood in Non-Hodgkin's Lymphoma. Correlation with Clinical Features and DNA Content

Peripheral blood from 69 patients with non-Hodgkin's lymphoma was examined with respect to B and T cell markers. Evidence for monoclonal B cell was found in 29 cases, 8 of ‘high grade’ and 21 of ‘low grade’ malignancy according to the Kiel classification. 17 out of the 29 patients had a normal lymphocyte count. Using conventional staging methods 4 cases of the 29 were in stages II and III, all others in stage IV. The proportion of S-phase cells in peripheral blood, determined by flow cytometry, was found to be elevated in cases with a monoclonal cell population. It is concluded that surface marker analysis of blood cells may be valuable as a diagnostic tool, as an indicator of prognosis and perhaps for the staging procedure of malignant lymphomas.     

肺原发性间变性大细胞淋巴瘤1例及文献复习

目的 探讨肺原发性间变性大细胞淋巴瘤(anaplastic large cell lymphoma,ALCL)临床病理特点、免疫组化特点、鉴别诊断、治疗和诊断技术,以提高对肺原发性ALCL的认识。方法 通过本病例及复习相关的文献资料,回顾性分析病例的临床特征、组织细胞形态学和免疫组化特点。结果 肺原发性ALCL的临床特...     

乳腺原发恶性淋巴瘤的诊断及治疗方法探讨

目的探讨乳腺原发恶性淋巴瘤(PBL)的临床特点及诊断、治疗方法。方法对经病理检查证实的11例女性PBL患者的临床表现、检查及治疗方法、预后等进行分析。结果11例均经手术或穿刺活检取标本，组织学及免疫组织化学检查证实为非霍奇金氏淋巴瘤(NHL)，B细胞来源10例、T细胞来源1例，弥漫性大B细胞型占54．5％(6／11)。10例分别接受乳腺肿瘤根治术或改良根治术或肿块切除术。均辅以化疗和放射治疗。首程治疗后7例获得局部控制。全组无病生存期4～49个月(中位生存期12个月)，总生存期15～110个月(中位30个月)。结论PBL的确诊依靠病理组织学和免疫组织化学检查。治疗应包括局部手术、放射治疗和化疗的综合治疗。     

Telomerase activation in normal B lymphocytes and non-Hodgkin's lymphomas

Activation of telomerase seems to be a prerequisite for immortalization and is found in permanent cell lines and most malignant tumors. Normal somatic cells are generally telomerase negative, except for bone marrow stem cells. Weak activity is also present in peripheral blood cells. In the present study strong telomerase activity was demonstrated in vivo in normal mature cells of the immune system, as well as in malignant lymphomas. Benign lymph nodes had lower telomerase activity than benign tonsils, which exhibited intermediate to high activity comparable with findings in malignant lymphomas. In benign tonsils the activity seemed to be restricted to germinal center B cells. In benign lymphoid tissues telomerase activity correlated with B-cell numbers and cell proliferation, but this was not observed in the lymphoma group. High- grade lymphomas exhibited higher levels of telomerase compared with low- grade cases. The data showed that in vivo activation of telomerase is a characteristic feature of germinal center B cells. Different signals for activation of telomerase are likely to exist, one of them being immune stimulation. The data suggest that telomerase activity in malignant lymphomas can be explained by an "induction and retention" model, ie, transformation occurs in a normal, mature B cell with reactivated telomerase, which is retained in the neoplastic clone.     

原发性肺平滑肌肉瘤8例诊治分析并文献复习

目的探讨原发性肺平滑肌肉瘤的临床、病理特点,诊断和治疗,提高其诊治效果。方法总结分析我院收治的8例原发性肺平滑肌肉瘤患者的诊治及预后情况。结果无手术死亡,8例患者均恢复出院,术后随访死亡2例,其余仍健在。结论原发性肺平滑肌肉瘤是一种少见的肺部恶性肿瘤,早期无特殊症状,极易误诊。手术切除是治疗原发性肺平滑肌肉瘤的有效方法,肿瘤的大小、局部受侵程度、癌细胞分化程度与预后明显相关。     

Efficacy and safety of pegylated liposomal doxorubicin in primary cutaneous B‐cell lymphomas and comparison with the commonly used therapies

Objectives: The therapy of advanced, relapsed or refractory primary cutaneous lymphomas is often unsatisfactory. Recent data indicate a favourable pharmacokynetic, pharmacodynamic and toxicity profile of pegylated liposomal doxorubicin (Peg‐Doxo) in primary cutaneous T‐cell lymphomas, while in primary cutaneous B‐cell lymphomas (PCBCLs), the drug efficacy has never been assessed so far. Methods: We performed a prospective phase II pilot clinical trial of Peg‐Doxo monotherapy (20 mg/m²) in PCBCLs. One patient had a marginal zone B‐cell lymphoma and four were affected by diffuse large B‐cell lymphoma‐leg type, all with widespread nodular lesions. Results: All the patients achieved a complete response (CR = 100%) in a short period of time (median 3 months), even when pretreated with radio‐chemotherapy. Two experienced a relapse. At follow‐up, one patient died for progressive disease; four are in CR after 5, 52, 63 and 69 months. As concerning the toxicity profile, the treatment was well‐tolerated, no one decreased or delayed the dose. The haematological toxicity was mild with only one case of grade III neutropenia; a patient showed a grade I neurotoxicity. Dermatological toxicity, in particular the palmar–plantar erythrodysesthesia, did not occurred, probably because of both the low dosages of Peg‐Doxo monotherapy and the oral prophylaxis with pyridoxine. Conclusions: In spite of the small number of patients, it emerges that monochemotherapy with Peg‐Doxo has a significantly high clinical activity and a good safety profile in PCBCLs, even in aggressive forms, compared with other therapeutic regimens, which are completely reviewed. It suggests the need of further investigations in this field.     

Bronchopulmonale Manifestationen von malignen hämatologischen Erkrankungen

Malignant hematological disorders, e.g. malignant lymphomas (Hodgkin’s and non-Hodgkin’s lymphomas), multiple myeloma, acute and chronic leukemias may show pulmonary, bronchial and/or pleural manifestations. They may occur as primary disease manifestations, but are more frequently seen during the further course of the disease. The clinical symptoms and radiological abnormalities are manifold, ranging from isolated or multiple asymptomatic pulmonary nodules to diffuse alveolar or interstitial infiltrates and mucosal or pleural infiltrates. Diffuse alveolar hemorrhage may occur. A broad differential diagnosis is therefore required. It includes infectious diseases, drug-induced lung injury and independent secondary disorders. In addition to clinical laboratory and radiological investigations, the diagnosis is based on endoscopic and bioptic methods, primarily bronchoscopy with bronchoalveolar lavage and biopsies with histological, cytological and immunohistochemical methods and flow cytometry. These tools have a favorable risk-benefit relationship. An early and consistent diagnosis is essential for the patient’s prognosis.     

Primary esophageal lymphoma in a patient with AIDS

Primary esophageal lymphomas are extremely rare. We report a primary esophageal T cell lymphoma of a diffuse large cell phenotype B in a patient with AIDS. Also we reviewed other published cases. The diagnosis of this complication should be considered in HIV seropositive patients with progressive displagia and endoscopic findings of masses, polyps or ulcerations and, specially in those unresponsive to antifungal or antiviral therapy. Biopsy and histopathologic studies are needed to confirm the diagnosis.