Diffuse large B-cell lymphoma with concurrent gastric adenocarcinoma: case report and literature review

The simultaneous occurrence of diffuse large B-cell lymphoma (DLBCL) and gastric carcinoma is rare. The present case report describes a 61-year-old man with DLBCL at the ileocaecal junction with several metastatic lymph nodes and concurrent gastric intramucosal adenocarcinoma. Both tumours, together with the enlarged lymph nodes, were successfully removed by surgery. At 1 month postoperatively, the patient received chemotherapy consisting of rituximab, cyclophosphamide, vindesine, epirubicin hydrochloride and dexamethasone; he responded well to treatment. Reports published in the literature between January 2006 and March 2011 of other cases of DLBCL combined with concurrent non-haematological malignancies in immunocompetent patients were reviewed. The identification of common factors is important for clarification of the mechanisms of lymphomagenesis and carcinogenesis, as well as the creation of preventive and therapeutic strategies. Such cases highlight the need routinely to perform preoperative imaging studies to exclude other synchronous tumours and, if possible, to biopsy any such masses in order to offer timely and appropriate therapy.     

Localized bullous pemphigoid in a patient with B-cell lymphoma

Bullous pemphigoid (BP) is an immunobullous disease characterized by circulating IgG antibodies directed towards cutaneous basement zone antigens. We report the case of a patient who had BP localized to a stoma site. At initial examination, a nodule was noted on the temple, which proved to be a large cell lymphoma, B-cell phenotype. On Western immunoblot, the patient's serum showed circulating IgG antibodies reactive with the 230 kDa BP antigen and the 97 kDa linear IgA bullous dermatosis antigen. The co-incident onset of the two diseases suggest that this may represent a case of paraneoplastic BP.     

Malignant triton tumor in a patient with neurofibromatosis type 1

We report a case of neurofibromatosis type 1 (NF1) complicated by a malignant triton tumor (MTT), with an emphasis on B-mode sonographic (US) and contrast-enhanced US (CEUS) findings. To the best of our knowledge, this is the first report describing CEUS findings of MTT. The mass was poorly demarcated and composed of an internal echogenic area and an outer hypoechoic zone. CEUS findings showed the outer zone to be strongly enhanced, and the internal area was very poor in blood flow because of necrotic tissues.     

A case of B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma and Burkitt lymphoma in a Korean child

B-cell lymphoma, unclassifiable, with features intermediate between diffuse large B-cell lymphoma (DLBCL) and Burkitt lymphoma (BL) (intermediate DLBCL/BL), is a heterogeneous group with some features resembling DLBCL and others resembling BL. Here, we report a case of intermediate DLBCL/BL in a Korean child. A 2-yr-old male was admitted for evaluation and management of left hip pain. Immunohistochemistry of a biopsy of the femur neck revealed tumor cells positive for CD20, CD10, BCL2, BCL6, and Ki67. A bone marrow (BM) aspirate smear revealed that 49.3% of all nucleated cells were abnormal lymphoid cells, composed of large- and medium-sized cells. Immunophenotyping of the neoplastic cells revealed positivity for CD19, CD10, CD20, and sIg lambda and negativity for CD34, Tdt, and myeloperoxidase (MPO). Cytogenetic and FISH analyses showed a complex karyotype, including t(8;14)(q24.1;q32) and IGH-MYC fusion. Intensive chemotherapy was initiated, including prednisone, vincristine, L-asparaginase, daunorubicin, and central nervous system prophylaxis with intrathecal methotrexate (MTX) and cytarabine. One month after the initial diagnosis, BM examination revealed the persistent of abnormal lymphoid cells; cerebrospinal fluid cytology, including cytospin, showed atypical lymphoid cells. The patient was treated again with cyclophosphamide, vincristine, prednisone, adriamycin, MTX, and intrathecal MTX and cytarabine. The patient died of sepsis 5 months after the second round of chemotherapy.     

Primary ileal plasmacytoma arising in mixed low- and high-grade B-cell lymphoma of mucosa-associated lymphoid tissue type

We report an extremely rare case of primary ileal plasmacytoma accompanied by mixed low- and high-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type. The radiographic and macroscopic features of the tumor were characterized by two constricting lesions in the ileum. Histologic examination of the resected specimen showed that one constrictive lesion was plasmacytoma and the other MALT lymphoma was low and high grade. The plasmacytoma seemed to have differentiated from the MALT lymphoma.     

Sonographic findings in a patient with neurofibromatosis type 1 and a gastrointestinal stromal tumor

Gastrointestinal stromal tumors (GIST) have been suggested to be the most common neurofibromatosis 1‐associated gastrointestinal tumors. This case report describes and compares US and CT findings of both abdominal neurofibromas and a gastrointestinal stromal tumor. On US, the GIST appeared as a well‐defined inhomogeneous lesion with a target‐like pattern similar to CT. The neurofibromas appeared as well‐demarcated round nodules with a relatively homogeneous hypoechoic internal structure and were accompanied by subtle posterior acoustic enhancement. US and CT were able to differentiate between neurofibromatomas and GIST in this neurofibromatosis 1 patient; however, a biopsy of the suspicious mass was performed to clarify the diagnosis. © 2010 Wiley Periodicals, Inc. J Clin Ultrasound 2010     

1例右颈部血管及腮腺破裂Ⅰ型神经纤维瘤并血管病变病人的术后护理

神经纤维瘤(neurofibromatosis,NF)是一种渐进性、可侵害全身各系统的常染色体显性遗传性疾病[1]。NF分为4种类型,Ⅰ型神经纤维瘤(NF1)最多见,也被称作Yon Reek1inghausen病,约占NF的90%,临床上主要表现为皮肤牛奶咖啡色斑和多发神经纤维瘤[2]。NF1并发血管病变发生率约为     

1例右颈部血管及腮腺破裂Ⅰ型神经纤维瘤并血管病变病人的术后护理

神经纤维瘤（neurofibromatosis,NF）是一种渐进性、可侵害全身各系统的常染色体显性遗传性疾病[1]。NF分为4种类型,Ⅰ型神经纤维瘤（NF1）最多见,也被称作Yon Reek1inghausen病,约占NF的90%,临床上主要表现为皮肤牛奶咖啡色斑和多发神经纤维瘤[2]。     

肠道复合性弥漫性大B细胞淋巴瘤及周围T细胞淋巴瘤临床病理观察

目的 探讨罕见的肠道复合性淋巴瘤的临床病理特征.方法 采用形态学、免疫组化及分子遗传学方法对1例肠道复合性弥漫性大B细胞淋巴瘤及周围T细胞淋巴瘤进行临床病理分析并复习文献.结果 本例肠道复合性B与T细胞淋巴瘤在形态上有弥漫性大B细胞淋巴瘤和非特殊性周围T细胞淋巴瘤的两种区域,免疫组化ClY20和CD3(+),分子遗传学上同时有IgH和TCR基因重排.结论 肠道复合性淋巴瘤罕见,诊断需结合形态、免疫表型和分子遗传学特征.     

一例Ⅰ型神经纤维瘤病患者的NF1突变检测及家系分析

目的 对1例临床拟诊为Ⅰ型神经纤维瘤病(neurofibromatosis type 1,NF1)患者进行致病基因突变研究. 方法 提取先证者及其家系成员外周全血基因组DNA,通过目标捕获二代测序技术(targeted next-generation sequencing,TNGS)对先证者Ⅰ型神经纤维瘤蛋白基因(neurofibromin 1,NF1)的全部编码区外显子及其侧翼序列进行高通量测序检测可疑突变,并用Sanger测序法进一步验证;对其家系成员NF1相同突变位点进行Sanger测序检测. 结果 基因检测发现先证者NF1第45号外显子1个已知致病突变c.6790_6791insA(p.Tyr2264Ter),有类似临床表现的父亲和姐姐检出相同突变,表型正常的母亲和妻子未检测到此突变,其4岁儿子也检测出该突变. 结论 NF1的c.6790_6791insA(p.Tyr2264Ter)突变存在与该家系NF1的发病密切相关.     

原发性皮肤弥漫性大B细胞性淋巴瘤(腿型)17例临床病理分析

目的探讨原发性皮肤弥漫性大B细胞性淋巴瘤(腿型)(PCDLBCLLT)的临床病理特点。方法回顾性分析17例PCDLBCLLT的临床资料、组织学形态和免疫组化标记。结果 17例PCDLBCLLT的发病年龄为31～86岁,平均64.4岁;其中男性9例,女性8例,男女之比为1.1∶1;主要发生于腿部和躯干部。组织学表现为弥漫分布的肿瘤细胞,以中心母细胞和免疫母细胞为主,核分裂象易见,肿瘤组织不累及表皮。免疫组化:肿瘤细胞表达B细胞相关抗原,bcl-2、MUM1、FOX-P1和bcl-6(+),Ki-67增殖指数为60%～90%。结论 PCDLBCLLT是一种独特类型的大B细胞性淋巴瘤,预后较差。     

弥漫大B细胞淋巴瘤治疗进展

非霍奇金淋巴瘤(non-Hodgkin lymphoma NHL)是恶性淋巴瘤的一大类型,在中国所占比例明显高于霍奇金淋巴瘤.弥漫大B细胞淋巴瘤(diffuse large B-cell lymphoma,DLBCL)是非霍奇金淋巴瘤中最为常见的一种类型,在临床、免疫表型及分子遗传学等方面具有高度异质性,其治疗方案也呈多样化.近10年来,DLBCL的诊断及治疗取得了重要进展,现就其规范化治疗方案及最新治疗研究进行阐述.