Peripheral T-cell lymphoma in childhood. A clinicopathological study of six cases

A review of the pathological material from 42 children with non-Hodgkin's lymphoma seen over a 44 month period revealed 10 large cell tumours. Of these, six were classified as peripheral T-cell lymphoma, an entity rarely reported in childhood. Three patients were boys and three girls (median age 9.5 years), and extranodal presentation was a feature of two patients. Five had high-grade tumours; of these, three were classified as large cell anaplastic, Ki-1 positive and two as pleomorphic large cell. The remaining patient had a low-grade tumour of angioimmunoblastic type. T-cell subsets were examined in three cases and showed the following phenotypes: CD4-, CD8-; CD4+, CD8-; CD4-, CD8+. Three of the patients with high-grade tumours died, with a mean survival of 22 weeks. The remaining patients are alive and clinically disease-free for between 10 and 24 months after treatment.     

36例原发胃肠道淋巴瘤的诊断和治疗

目的：探讨提高原发胃肠道淋巴瘤术前诊断率及治疗效果的方法。方法：对36例原发胃肠道淋巴瘤患者的临床表现、体征及辅助检查进行分析，通过COX回归等统计方法对其预后因素及生存率等进行分析。结果：该病临床表现无特异性；诊断主要依靠内窥镜病理活检；手术方式、综合治疗完成情况、临床分期为与预后有关的影响因素。结论：提高对该病临床表现及内镜、造影检查特点的认识，采用正确的活检方法是提高术前诊断率的关键；以手术及化疗为主的综合治疗措施是提高疗效的关键。     

中西医结合治疗恶性淋巴瘤超声造影成像特点分析

目的 观察中西医结合治疗恶性淋巴瘤前后超声造影灌注的动态变化。     

原发性乳腺恶性淋巴瘤的诊断及外科治疗探讨

目的探讨原发性乳腺恶性淋巴瘤的诊断与治疗. 方法回顾性分析我院1992～2004年5例原发性乳腺恶性淋巴瘤的临床资料.5例均为术后组织学病理诊断.4例行乳腺癌改良根治术,1例行乳房单纯切除术.5例均行化疗及放疗. 结果乳腺癌改良根治术手术时间60～120 min,平均80 min;乳房单纯切除术加乳腔镜腋窝淋巴结清扫术手术时间75 min.术中出血量150～250 ml,平均200 ml.术后病理组织诊断均为弥漫性非霍奇金氏淋巴瘤,为B细胞来源.且均有腋窝淋巴结转移.全组病例随访6个月～12年,平均4.5年.2例II期,均死于肿瘤多器官转移;3例I期,肿瘤无复发. 结论原发性乳腺恶性淋巴瘤无明显特异性表现,术前诊断困难.钼靶X线照相,对诊断有帮助. 治疗可选择手术、放疗、化疗或单纯给予化疗和放疗.     

Hypophyseal Non-Hodgkin's Lymphoma presenting with clinical panhypopituitarism successfully treated with chemotherapy

Summary A patient is described with a testicular Non-Hodgkin's Lymphoma (NHL) presenting with panhypopituitarism caused by a hypophyseal localization. A⁶⁷Gallium scintigraphy showed avid uptake in the hypophyseal region. Obviously⁶⁷Gallium could reach the tumor, by the intravenous route, which was the reason to treat the patient with intravenous chemotherapy. A complete remission was induced, which seems to be lasting (+ 25 months). As far as we know this is the first report of panhypopituitarism caused by a hypophyseal NHL in the hypophysis and successfully treated by intravenous chemotherapy.     

Immunoglobulin heavy chain Diversity genes rearrangement pattern indicates that MALT-type gastric lymphoma B cells have undergone an antigen selection process

Gastric MALT lymphoma usually develops from chronic gastritis, the vast majority of which (>90%) is associated with Helicobacter pylori infection. We sequenced the third complementarity determining region (CDR3) of immunoglobulin heavy chain genes in 19 gastric MALT lymphoma clones to determine the pattern of variable (V), diversity (D) and joining (J) gene utilization during immunoglobulin gene rearrangement.
DNA was extracted from paraffin-embedded sections and the rearranged CDR3 regions were amplified using a semi-nested polymerase chain reaction (with primers complementary to the conserved framework-three segment of the variable region [FR3A] and J regions). The DNA used for cloning and sequencing was obtained after purification of monoclonal bands excised from polyacrylamide gels. The N-D-N region specific to each clone was compared with known germline D sequences.
Similarly to that observed in normal and leukaemic B cells, our series of gastric MALT lymphomas showed apparent preferential utilization of genes from the DXP family. In two cases no similarity between the CDR3 nucleotide sequences of the neoplastic clones and the known germline D sequences could be found. In 10/19 analysed alleles the lymphoma B-cell clones appeared to contain two D gene segments (D-D recombination), a rare occurrence in normal individuals but one which has been described as a significant event in the determination of idiotype expression and antigen-binding affinity. Remarkably, despite the use of different D and J segments, the resultant amino acid sequences matched in two patients, suggesting the presence of a common selecting antigen.
The observed pattern of D gene rearrangement suggests that MALT lymphoma B-cell clones have undergone antigen selection, which seems to indicate that the antigen stimulation plays a pivotal role in the development of the lymphoma.     

Hemophagocytic syndrome and T-cell lymphoma after kidney transplantation: a case report

Lymphoma in immunocompromised transplant patients is a feared cause of morbidity and mortality. Superimposed on the lymphoma and the transplantation immunosuppression is a rare condition: hemophagocytic syndrome (HS). HS is characterized by fever, hepatosplenomegaly and lymphadenopathy, skin rashes, jaundice, coagulopathy, and phagocytosis of blood elements with pancytopenia. Here we describe a rare but fatal case of a kidney transplant patient who developed T-cell lymphoma and HS, without evidence of EBV replication. A short review of the diagnosis, treatment, and prognosis of HS is given. Received: 4 March 1997 Received after revision: 6 June 1997 Accepted: 30 June 1997     

43例鼻咽,鼻腔恶性淋巴瘤的临床病理及免疫表型研究

本文对遵义地区４３例鼻咽、鼻腔恶淋巴瘤的临床病理及免疫学表型进行了研究，结果显示：全部病例均为弥漫型，无１例滤泡型，组织学类型以多表细胞性淋巴瘤为主，共３３例（包括小细胞型１３例，中多形１５例，大多形５例），占７６．７％。用ＵＣＨＬ－１、ＣＤ２０和Ｍａｃ３８７等多单克隆抗体进行免疫表型研究。显示Ｔ细胞淋巴瘤３７例（８６％），Ｂ细胞淋巴瘤４例（９．３％），无１例组织细胞性淋巴瘤。鼻咽部Ｔ．Ｂ淋巴瘤比     

59例老年非何杰金氏淋巴瘤病理分析

本文报道老年非何杰金氏淋巴瘤(NHL)59例并与同期非老年患者138例相比。老年患者占同期NHL总数的29.9％。59例中有结内型43例,结外型16例;低度恶性与中度恶性各12例,高度恶性35例。与非老年组比较,老年组的就诊原因、病变部位、恶性程度、细胞类型和预后等方面无何差别,提示年龄对上述特征无明显影响。     

皮肤T细胞淋巴瘤患者的人类T淋巴细胞白血病病毒Ⅰ型感染

目的　研究皮肤T细胞淋巴瘤 (CTCL)患者中是否存在人类T淋巴细胞白血病病毒Ⅰ型 (HTLV Ⅰ )的感染。方法　检测CTCL患者血清中的HTLV Ⅰ抗体以及患者外周血细胞和活检标本中的前病毒DNA。结果　PCR法检测CTCL患者外周血样 5 7例 ,其中HTLV Ⅰ前病毒DNA阳性患者 6例 ;ELISA法检测CTCL患者 5 4例 ,其中 1例为可疑阳性 ;Western印迹法检测CTCL患者 18例 ,其中HTLV Ⅰ抗体阳性者 4例 ,此 4例患者前病毒DNAPCR结果也为阳性。结论　皮肤T细胞淋巴瘤患者存在HTLV Ⅰ病毒感染 ,CTCL与HTLV Ⅰ有一定的关系。HTLV Ⅰ病毒感染可能是CTCL发病的诱因之一。