肺透明细胞癌1例并文献复习

目的 分析肺透明细胞癌的临床特征、影像学表现及病理诊断要点.方法 报告1例经肺活检病理确诊的肺透明细胞癌病例,并结合文献资料对该病的临床特征、影像学表现及病理诊断要点进行分析.结果 本例患者为女性,45岁,以反复咳嗽2个月为首发症状,胸部CT示两肺多发结节灶合并肺门及纵隔淋巴结肿大,胸腔镜下肺活检病理确诊为原发性肺透明细胞癌.近20年国内外文献报道肺透明细胞癌病例仅27例,其中男性18例,女性9例,平均年龄(55±11)岁.临床表现主要为咳嗽(16/27)、胸痛(12/27)、咯血(10/27).影像学表现以周围型单发结节或团块影多见(22/27),病灶直径0.5～11 cm.该病罕见,属大细胞癌的变异型;确诊多依赖开胸肺组织病理活检,免疫组织化学染色有助于鉴别.结论 肺透明细胞癌的临床表现无特异性;影像学表现以肺部单发结节或团块影多见,亦可为两肺多发和弥漫型;确诊依赖肺组织病理活检,免疫组织化学染色有助于鉴别.     

累及呼吸系统的Sweet’s综合征17例临床分析

目的探讨呼吸系统受累的Sweet’S综合征的临床表现、实验室检查、影像学特点和诊疗方法，以提高临床医师对该病的认识。方法检索Google、Pubmed、Ovid、万方等数据库，得到1986年以来的17例呼吸系统受累，血液、痰液、肺泡灌洗液细菌、真菌培养阴性的Sweet’S综合征，分析其临床资料。结果男性8例，女性9例，发病年龄26~82岁．，平均（53．5±15．o）岁。6例（35．3％）首发部位在肺或气道，9例（52．9％）首发于皮肤，2例（11．8％）皮肤和肺几乎同时受累。血常规检查白细胞升高13例（76．4％），中性粒细胞比例〉70％ll例（64．7％）。2例受累气道黏膜活检病理变化与皮肤改变相似，5例肺组织病理发现大量中性粒细胞浸润，3例呈机化性肺炎。15例肺受累病例，胸部影像示单侧或双侧肺索条状、斑片状致密影或磨玻璃影，其中1例纵隔内隆突前见肿大淋巴结，1例伴有胸腔积液。在13例做过骨髓检查患者中，7例骨髓增生异常，1例白血病。所有病例抗生素治疗无效，糖皮质激素均有显效。4例应用激素虽有好转，但仍死于急性呼吸衰竭、多器官衰竭、全血细胞减少致出血或感染致死。结论呼吸系统受累可以先于、同时或后发于皮肤损害，且常伴造血功能异常。诊断有赖于临床表现、病理检查或激素治疗反应。早期诊断、早期全身应用糖皮质激素治疗可有效缓解病情。     

巨细胞间质性肺炎一例及文献复习

目的提高对巨细胞间质性肺炎（GIP）的临床、胸部影像学和病理改变的认识。方法对1例经开胸肺活检证实的GIP患者的职业史、临床表现、胸部X线、CT和病理资料并结合有关文献进行回顾性分析。结果GIP主要的临床表现有咳嗽和活动后呼吸困难；肺功能检查表现为限制性通气功能障碍；胸部X线和HRCT表现为两肺磨玻璃样影、实变影、弥漫性的小结节影、网状影和牵拉性支气管扩张。GIP主要病理表现有脱屑性间质性肺炎样反应，即在肺泡腔内有巨噬细胞和大量的多核巨细胞聚集，位于肺泡腔内的多核巨细胞内可见被吞噬的炎性细胞，是GIP的特点。GIP是硬金属尘肺的特征性病理改变。结论GIP是非常罕见的慢性间质性肺炎，无特异性的临床表现，影像学表现类似于特发性间质性肺炎。在GIP诊断中，仔细收集患者的职业史非常重要。     

隐源性机化性肺炎的临床病理特征和影像学表现

目的探讨隐源性机化性肺炎(COP)的临床病理特征和影像学表现。方法分析5例隐源性机化性肺炎病例的临床特点、影像学表现、肺活检的病理特征,并复习相关文献。结果 COP常见的临床表现为咳嗽、进行性呼吸困难和吸气末肺部爆裂音。肺活检病理检查显示肺泡管、肺泡腔内见肉芽组织栓。胸部CT表现为含支气管充气征的实变阴影,伴或不伴磨玻璃影。患者对糖皮质激素治疗有显著疗效。结论临床表现结合影像学特点可提示COP临床诊断,肺活检是诊断COP有效的检查方法。     

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。     

淋巴细胞间质性肺炎7例临床、CT影像学及病理特征分析

［摘要］　目的　分析淋巴细胞间质性肺炎（LIP）的临床、CT影像学及病理特征。方法　回顾性分析7例经病理确诊的LIP患者的临床、影像资料,所有病例均进行胸部薄层CT检查。结果　7例LIP患者均为女性,4例表现为弥漫分布的斑片状磨玻璃影,其中3例伴有网格状影、小结节影;3例表现为局灶分布的斑片状磨玻璃影,其中1例局部夹杂小结节影。5例有多发薄壁含气肺囊肿,4例伴有纵隔淋巴结肿大。病理活检组织HE染色主要表现为肺间质内弥漫性淋巴细胞浸润,免疫组织化学染色主要提示CD3细胞和多克隆CD20细胞的增加。结论　LIP为罕见病,好发生于女性干燥综合征患者,当肺部高分辨率CT呈斑片状磨玻璃影并多发薄壁含气肺囊肿改变,病理表现为肺间质弥漫性淋巴细胞浸润时,高度提示LIP。     

隐源性机化性肺炎18例临床分析

目的 分析我院经活检确认的18例隐源性机化性肺炎患者,总结其在临床、治疗及预后方面的特点.方法 收集我院2008年1月至2012年10月间经肺活检证实的18例隐源性机化性肺炎患者的所有资料,回顾性分析本组患者的临床资料、易患因素、影像学表现、病理、实验室检查等的特点.结果 18例患者平均年龄（59±11）岁.本组18例患者临床症状主要为咳嗽、咳痰、胸闷、气短等呼吸系统症状,少数有盗汗、乏力、消瘦等全身症状.影像学表现多种多样,可有斑片影、实变影、磨玻璃样变影等,最初发病多以斑片影为主.肺功能表现为轻度限制性通气功能障碍,弥散功能受损.14例（77.8％）患者治愈,4例（22.2％）患者复发,对糖皮质激素效果较好.结论 隐源性机化性肺炎容易误诊为肺部感染,诊断需要综合临床-影像-病理三方面分析,病理具有早期指导治疗作用,但是本病预后良好.     

38例结节病临床分析

目的 探讨结节病的临床特点及诊治方法.方法 分析经组织病理学确诊的38例结节病患者的临床资料.结果 平均发病年龄54.6岁,女性多于男性;临床表现多样,包括呼吸系统症状和肺外表现;胸部影像学检查是早期诊断的重要手段;确诊依赖组织病理学检查,纵隔镜淋巴结活检及外科电视胸腔镜下肺活检阳性率最高.结论 结节病临床表现多样而且无特异性,尽可能行组织病理学检查明确诊断,糖皮质激素治疗有一定疗效.     

侵袭性肺曲霉病1例并文献复习

目的提高对侵袭性肺曲霉病的临床、胸部影像学、病原学和病理改变的认识。方法对1例经病理证实的侵袭性肺曲霉病患者的职业、临床表现、胸部X线、CT、气管镜和病理资料并结合文献进行回顾性分析。结果侵袭性肺曲霉病的临床表现缺乏特异性，典型的影像学检查显示胸膜下单发或多发结节状或斑片状阴影，并呈动态变化。GM试验用于诊断侵袭性肺曲霉病具快速灵敏的特点；侵袭性肺曲霉病的病理可见由大小规范，呈两分叉（Y形）的分隔菌丝引起的特征性的血管侵害，也可以表现为局限性肉芽肿或广泛化脓性肺炎。结论侵袭性肺曲霉病的临床表现和影像学改变非常复杂，在诊断中需要综合患者的各种临床资料（包括临床症状、放射学、血清学检查和病理）。     

以中枢性尿崩为首发表现的朗格汉斯细胞组织细胞增生症的临诊应对

报道3例以中枢性尿崩为首发表现的朗格汉斯细胞组织细胞增生症(LCH)患者,总结其临床表现、实验室检查、影像学检查、病理结果、诊断过程和治疗反应。3例患者早期均以中枢性尿崩症起病,垂体磁共振成像(MRI)均表现为垂体柄增粗,垂体后叶正常高信号消失。2例患者表现为孤立性下丘脑-垂体病变,1例表现为垂体和甲状腺多系统受累。病理结果显示典型的朗格汉斯细胞,免疫组织化学示S-100、CD1a、Langerin阳性。LCH临床表现呈现明显的异质性,容易误诊和漏诊。确诊依赖病理结果,孤立性下丘脑-垂体病变活检难度较大,建议积极筛查其他器官增加活检概率。LCH导致的神经垂体损害通常需要终生激素替代治疗。     

Primary anaplastic large cell lymphoma of the lung. Report of two cases and literature review

To our knowledge, only eleven cases of primary anaplastic large cell lymphoma (ALCL) of the lung have previously been reported. We describe here another two cases of primary pulmonary ALCL that developed in two Chinese women. Both patients presented with insidious symptoms related to pulmonary mass or nodule lesions. A comprehensive workup failed to show disease outside the chest. CD30-positive ALCL was demonstrated by histopathological studies of the lung tissue. Both patients were treated with 6 cycles of CHOP (cyclophosphamide, doxorubicin, vincristine and prednisone) chemotherapy and had complete remission. Primary pulmonary lymphoma is a great challenge for pneumologists since the clinical presentations and radiological findings are nonspecific. Appropriate invasive biopsy is necessary for early diagnosis.     

A case of anaplastic large cell lymphoma associated with Epstein-Barr virus infection, representing clinicopathological features of malignant histiocytosis

An 82-year-old woman was admitted with fever and anorexia. Aggravated pancytopenia and liver dysfunction suggested the presence of disseminated intravascular coagulation. The serum ferritin level increased to 9,100 ng/ml. Bone marrow aspiration showed an increase of histiocytes with phagocytosis and a diagnosis of hemophagocytic syndrome was made. Symptomatic therapy was performed because of her deteriorated general condition. She died of multiple organ failure, 32 days after admission. Autopsy revealed swollen lymph nodes with proliferation of large neoplastic cells containing rich cytoplasm and pleomorphic and multi-segmented large nuclei. The immunophenotype of the neoplastic cells was LCA-, CD3-, CD5-, CD 20-, CD79a-, UCHL1-, MT1-, CD15-, p80-. Neoplastic cells were positive for CD30, mainly in Golgi apparati, and also positive for EBV-encoded small nonpolyadenylated RNAs (EBER). This case was diagnosed as anaplastic large cell lymphoma (ALCL) associated with hemophagocytic syndrome. It was estimated that Epstein-Barr virus had played an important role in the development of ALCL in the present case.     

Ki-1 (CD30)-positive anaplastic large cell lymphoma, sarcomatoid variant accompanied by spontaneously regressing lymphadenopathy

Although it has been reported that primary Ki-1 (CD30)-positive anaplastic large cell lymphoma (ALCL) of the skin may undergo spontaneous regression, it is rare for ALCL without cutaneous involvement to have spontaneously regressing lymphadenopathy. We report a case of sarcomatoid variant of ALCL accompanied by spontaneously regressing lymphadenopathy. The patient had gastric and pulmonary involvement of ALCL in addition to systemic lymphadenopathy, but with no cutaneous involvement. The lymphadenopathy spontaneously improved gradually during a period of one month without any treatment. At the same time, multiple small nodules in both lung fields decreased on chest computed tomography and multiple elevated gastric tumors with dimples were endoscopically recognized to have improved. He has since been treated with combination chemotherapy because of recurrence of the lymphadenopathy.     

Hemophagocytic syndrome as a presenting sign of transformation of smoldering to acute adult T-cell leukemia/lymphoma: efficacy of anti-retroviral and interferon therapy

A 55-year-old Caribbean woman with a 6-year history of smoldering adult T-cell leukemia/lymphoma presented with clinical and biological symptoms of hemophagocytic syndrome. An extensive search for infectious diseases was negative. A lymph node biopsy showing large T-cell lymphoma (CD4-, CD25+) and findings of high LDH count and severe lymphocytosis led to the diagnosis of acute adult T-cell leukemia/lymphoma. Anti-retroviral therapy combining zidovudine, lamivudine, and interferon-alpha was started, resulting in rapid control of both hemophagocytic syndrome and symptoms of acute adult T-cell leukemia/lymphoma. Thus, we propose that adult T-cell leukemia/lymphoma must be added to the spectrum of etiologies of hemophagocytic syndrome.     

Sialosylated lewis x expression in CD30-positive anaplastic large-cell lymphomas

Summary The expression of sialosylated Lewis ^x (SLEX), a ligand for endothelial leukocyte adhesion molecule 1 in malignant lymphomas, was immunohistochemically examined, using the monoclonal antibody, CSLEX1, which specifically reacts with SLEX. It was expressed in 6 out of 64 non-Hodgkin's lymphomas, which consisted of 1 nasal large-cell lymphoma and 5 of 8 (62%) Ki-1-positive anaplastic large-cell lymphomas (ALCL). One nasal lymphoma positive for SLEX co-expressed a T cell marker, cluster of differentiation (CD) 5, and natural killer (NK) cell markers such as CD56 and CD16, indicating that SLEX⁺ nasal lymphoma cells are possibly malignant counterparts of SLEX⁺ NK cells. SLEX did not react with 30 B cell lymphomas or most Hodgkin's disease lymphomas, though it did with one lymphocyte predominance type. Although SLEX⁺ ALCL exhibit T cell markers in some cases, some ALCL expressing SLEX may represent histiocytic differentiation of the neoplastic cells. The lymphoma cells of ALCL were preferentially positive for SLEX, in contrast to Hodgkin's disease cells, and thus CSLEX1 in conjunction, with CD30 and CD15 should be of use for analyzing and making differential diagnoses of routine paraffin-embedded sections of ALCL.Abbreviations SLEX sialosylated Lewis ^x - ALCL anaplastic large-cell lymphomas     

NPM/ALK gene fusion transcripts identify a distinct subgroup of null type Ki-1 positive anaplastic large cell lymphomas

The chromosomal aberration t(2;5) resulting in the juxtaposition of NPM and ALK genes is a well-known feature of several Ki-1⁺ anaplastic large cell lymphomas (ALCL) of the T-cell type. However, conflicting results have been reported concerning the presence of this gene rearrangement in other ALCL and Hodgkin’s disease (HD), respectively. We performed NPM/ALK RT-PCR on 14 cases of ALCL expressing distinct myelomonocytic markers, e.g. CD11c, CD13, CD14 or CD68, but neither T-cell nor B-cell associated antigens (null cell phenotype). The specific translocation was found exclusively in six childhood tumours previously diagnosed as malignant histiocytosis (MH), whereas all adult lymphomas (three ALCL without characteristics of MH, three secondary ALCL following HD) and two paediatric cases of secondary ALCL following HD did not show NPM/ALK gene fusion products. By Southern blotting, the status of T-cell receptor (TCR) and immunoglobulin heavy chain genes (IgH) were investigated; two patients with initially diagnosed MH had the TCRδ-chain gene rearranged (Dδ2–Dδ3 and Vδ1–Jδ1, respectively). IgH rearrangements were detected in only one patient with secondary ALCL. Our data indicate a high association of previously diagnosed MH and NPM/ALK gene rearrangements. In one case, this specific translocation was demonstrated at an early stage of development; in another, a mature TCRδ-chain gene rearrangement was detected. These data support the hypothesis of a lymphoid origin of this subgroup of Ki-1 positive ALCL previously diagnosed as MH.     

Anaplastic large-cell lymphoma of null-cell type with multiple bone involvement

 A 21-year-old man who had anaplastic large cell lymphoma (ALCL) of the null-cell type with multiple bone involvement is reported. On admission, he had symptoms of incomplete paraplegia and urinary and rectal incontinence. Workup studies for staging revealed para-aortic lymph node swellings and multiple bone involvement including skull, ribs, left iliac bone, and thoracic/lumbar spine. Because paraplegia was rapidly progressive, a decompression operation was performed. The biopsy specimen obtained from the lumbar spine revealed sheetlike proliferation of anaplastic large cells. These cells were positive for CD30 (Ki-1), EMA, vimentin, and p80^NPM/ALK, and negative for CD3, CD20 (L26), and CD45 (LCA). Epstein-Barr virus-encoded small RNAs were not detectable in these cells. Thus, the patient was diagnosed as having ALCL of the null-cell type. He was treated with several courses of combination chemotherapy, and finally with total body irradiation plus high-dose chemotherapy supported by peripheral blood stem cell transplantation. However, soon after the treatment, the lymphoma cells massively infiltrated his bone marrow. He died of lymphoma 8 months after admission. Received: January 30, 1998 / Accepted: August 5, 1998     

NPM/ALK gene fusion transcripts identify a distinct subgroup of null type Ki-1 positive anaplastic large cell lymphomas

The chromosomal aberration t(2;5) resulting in the juxtaposition of NPM and ALK genes is a well-known feature of several Ki-1⁺ anaplastic large cell lymphomas (ALCL) of the T-cell type. However, conflicting results have been reported concerning the presence of this gene rearrangement in other ALCL and Hodgkin's disease (HD), respectively. We performed NPM/ALK RT-PCR on 14 cases of ALCL expressing distinct myelomonocytic markers, e.g. CD11c, CD13, CD14 or CD68, but neither T-cell nor B-cell associated antigens (null cell phenotype). The specific translocation was found exclusively in six childhood tumours previously diagnosed as malignant histiocytosis (MH), whereas all adult lymphomas (three ALCL without characteristics of MH, three secondary ALCL following HD) and two paediatric cases of secondary ALCL following HD did not show NPM/ALK gene fusion products. By Southern blotting, the status of T-cell receptor (TCR) and immunoglobulin heavy chain genes (IgH) were investigated; two patients with initially diagnosed MH had the TCRδ-chain gene rearranged (Dδ2–Dδ3 and Vδ1–Jδ1, respectively). IgH rearrangements were detected in only one patient with secondary ALCL.   Our data indicate a high association of previously diagnosed MH and NPM/ALK gene rearrangements. In one case, this specific translocation was demonstrated at an early stage of development; in another, a mature TCRδ-chain gene rearrangement was detected. These data support the hypothesis of a lymphoid origin of this subgroup of Ki-1 positive ALCL previously diagnosed as MH.     

Antitumor activity of anti-CD30 immunotoxin (Ber-H2/saporin) in vitro and in severe combined immunodeficiency disease mice xenografted with human CD30+ anaplastic large-cell lymphoma

To develop a novel adjunctive therapy for CD30 (Ki-1)+ anaplastic large- cell lymphoma (ALCL), we investigated in preclinical studies the antitumor activity of an immunotoxin (IT) constructed by coupling the plant ribosome-inactivating protein saporin (SO6) to the monoclonal antibody (MoAb) Ber-H2 that is directed against the CD30 molecule, a new member of the tumor necrosis factor receptor (TNFR) super-family. The activity of Ber-H2/SO6 IT was tested both in vitro against the CD30+ ALCL-derived cell line JB6 and in vivo using our severe combined immunodeficiency disease (SCID) mouse model of human xenografted CD30+ ALCL. In vitro, the Ber-H2/SO6 IT was selectively and highly toxic to the JB6 cell line [50% inhibiting concentration (IC50), 3.23 x 10(-12) mol/L as SO6]. In vivo, a 3-day treatment with nontoxic doses of Ber- H2/SO6 (50% of LD50) induced lasting complete remissions (CR) in 80% of mice when started 24 hours after tumor transplantation. In contrast, injection of the IT at later stages of tumor growth (mice bearing subcutaneous tumors of 40- to 60-mm3 volume), induced CR in only 6 of 21 (approximately 30%) mice and significantly delayed tumor growth rate (P < .01). This finding suggests that maximum effect of the anti-CD30 IT is observed when tumor cell burden is small. Persistent tumors from IT-treated mice consisted of CD30+ cells, thus excluding the possibility that selection of CD30-negative mutant clones during IT therapy was responsible for resistance to treatment. We conclude that Ber-H2/SO6 IT is an effective agent against CD30+ ALCL growing in SCID mice, suggesting its possible role as adjuvant therapy in patients with CD30+ ALCL refractory to standard treatments.     

The tyrosine kinase NPM-ALK, associated with anaplastic large cell lymphoma, binds the intracellular domain of the surface receptor CD30 but is not activated by CD30 stimulation

The heterogenous group of anaplastic large cell lymphomas (ALCLs) is characterized by expression of the Ki-1/CD30 antigen, a member of the tumor necrosis factor receptor superfamily. About 40 to 50% of cases diagnosed as ALCL contain a specific chromosomal rearrangement, t(2;5)(p23;q35), resulting in expression of the chimeric tyrosine kinase NPM-ALK. As NPM-ALK-positive lymphomas define a distinct subtype within the group of ALCL, the chimeric protein might be responsible for certain pathogenetic and clinicopathologic characteristics. To better elucidate the function of NPM-ALK, we investigated a possible mechanism for regulation of its activity. We demonstrate that NPM-ALK specifically binds to the intracellular domain of the cytokine receptor CD30. In vitro binding assays revealed that the ALK portion of NPM-ALK mediates interaction of the two proteins. Stimulation of the CD30 receptor by cross-linking with immobilized anti-CD30 antibody results in complete growth inhibition of Karpas 299, an NPM-ALK-positive ALCL cell line, but does not alter proliferation of HDLM-2, a Hodgkin's lymphoma-derived cell line lacking t(2;5). Western blot analysis of coimmunoprecipitated CD30 and NPM-ALK proteins from stimulated Karpas 299 cells showed that the interaction of the proteins is not modified by stimulation. Activation of CD30 neither enhanced NPM-ALK activity measured by autophosphorylation of the chimeric tyrosine kinase nor phosphorylation of phospholipase C-gamma, an NPM-ALK substrate. We conclude that NPM-ALK is not stimulated by CD30 activation, but exists as a constitutively hyperactivated protein. Interaction with CD30 may extend the subcellular localization of NPM-ALK to the microenvironment of membrane-associated proteins.