Detection of Epstein Barr virus in an hepatic leiomyomatous neoplasm in an adult human immunodeficiency virus 1-infected patient

We report the first case of a human immunodeficiency virus (HIV)-related primary hepatic leiomyoma in an adult patient. The diagnosis was made at autopsy and confirmed by immunohistochemistry. Epstein Barr virus (EBV) was identified in tumour cells by in situ hybridization. Review of the literature revealed 13 cases of visceral myogenic tumours occuring in acquired immunodeficiency syndrome children, and only 2 cases in adults. One was a spinal epidural leiomyoma, the other multiple smooth muscle tumours of the colon and adrenal gland. This is the first report of EBV in smooth muscle neoplastic cells in an HIV-infected adult patient.     

Ga67显像评估纵隔大B细胞淋巴瘤治疗预后的研究

目的:本研究旨在评价纵隔大B细胞淋巴瘤Ga67显像预测疗效的临床价值。方法:39例病人中女24例,男15例,平均年龄(48±5.6)岁,临床病史及组织病理学诊断为纵隔大B细胞淋巴瘤。分别在化疗前、化疗结束后1个月和放疗结束后3个月进行常规的Ga67 SPECT和CT显像,图像分析采用常规的定性方法。结果:Ga67显像结果:在39例病人治疗结束后,有32例病人肿瘤Ga67显像结果为阴性,提示治疗完全有效,随访证实阴性预测率为94%(30/32);7例病人治疗后Ga67显像阳性,表示治疗部分有效或治疗无效。随访证实阳性预测率为86%(6/7),提示Ga67显像阴性的病人预后很好,而治疗后Ga67显像阳性预后很差。CT扫描结果:39例病人进行了治疗前后的胸、腹和盆腔CT扫描,其阴性预测率为83%;而阳性预测率只有25%。结果提示与Ga67显像相比,CT预测疗效的结果难于区分治疗有效、部分有效或无效。结论:Ga67显像在评价纵隔大B细胞淋巴瘤病人治疗反应中明显优于CT扫描。     

肠道T细胞淋巴瘤临床分析

本文总结报告了3例肠道T细胞淋巴瘤(intestinal T-cell lymphoma，ITCL)的诊治资料，并结合文献复习，发现IT—CL多见于中年男性，以腹痛、血便、发热、体质量下降为主要症状，治疗效果差，预后不良。病理改变以肠道溃疡形成为特点，溃疡形态呈多形性、多灶性、不规则，镜下瘤细胞明显异型、弥漫性浸润，中至大细胞多见。肿瘤细胞呈T细胞表型。ITCL临床少见，缺乏特异性临床表现，极易误诊。故临床医师应重视对ITCL临床病理特征、免疫表型和基因型的研究，注意识别，促其早期诊治。     

弥漫性大B细胞淋巴瘤中CD40L的表达及意义

目的探讨弥漫性大B细胞淋巴瘤（DLBCL）组织中CD40L表达与DLBCL预后间的关系及意义。方法免疫组织化学法检测27例弥漫性大B细胞淋巴瘤、20例淋巴结反应性增生组织中CD40L的表达。结果（1）DLBCL中CD40L过度阳性率（25．93％）显著低于淋巴结反应性增生（63．64％），P〈0．05。（2）CD40L在Ⅲ、Ⅳ期DLBCL过度阳性率（14．29％）低于Ⅰ、Ⅱ期（38．46％），P〈0．05。CD40L过度阳性率在有结外浸润DLBCL（11．76％）低于无有结外浸润DLBCL（40％），P〈0．05。（3）DLBCL患者CD40L的过度阳性率与远处转移、临床分期均显著相关，P〈0．05。结论（1）CD40L过度阳性率与结外器官浸润及临床分期密切相关，其可能作为判断DLBCL侵袭性及预后的指标。（2）DLBCL中CD40L表达的减少可能是影响其发病的因素之一。     

Immunohistochemical and molecular genetic analysis of a case of T-cell-rich B-cell lymphoma presenting in bone marrow

Summary We report the case of a patient who, over a 20-month period, developed overt B-cell non-Hodgkin's lymphoma in a T-cell rich background initially indistinguishable from reactive lymphoid aggregates in bone marrow. This morphological pattern of B cell lymphoma has been termed T-cell-rich B-cell lymphoma and, to our knowledge, has not previously been reported with a primary bone marrow presentation. Of additional interest, the patient's initial presentation was with myelodysplasia which appeared morphologically to resolve as the lymphomatous population emerged. Subsequently, however, the patient developed overt acute myeloid leukaemia (AML) and demised. This patient appeared to have two distinct neoplastic processes occurring in his bone marrow. The relationship between the two remains a matter of speculation.     

Adolescence Mycosis Fungoides: An Unusual Presentation with Hypopigmentation

Mycosis fungoides (M.F.) is a rare cutaneous malignancy of childhood and adolescence. The disease commonly presents with the classic sequence of erythematous patches and plaques. Hypopigmentation as the presenting symptom is distinctly rare. This is the 9th case report of M.F. presenting with hypopigmentation in a patient under 20 years of age. The disease was only partially controlled with PUVA therapy, necessitating nitrogen mustard treatment.     

Phenotypic and Genotypic Lineage Switch of a Lymphoma with Shared Chromosome Translocation and T-Cell Receptor γ Gene Rearrangement

A case of non-Hodgkin's lymphoma showed a phenotypic and genotypic cell lineage switch twice during nine years of his clinical history; first, T-cell type, pleomorphic small cell lymphoma developed, followed by B-cell type, diffuse centroblastic/centrocytic lymphoma, and finally T-zone lymphoma without follicles again developed, from which AST-1 cultured cell line was established. Karyotype analysis demonstrated a shared abnormal chromosome, der(1)t(1;?)(p36;?), among the first relapsed B-cell tumor, the second relapsed T-cell tumor and AST-1 cell line. Furthermore, T-cell receptor (TCR) γ gene rearrangement bands of the same size were observed in the first relapsed B-cell tumor and the second relapsed T-cell tumor as well as AST-1 cell line. These results suggested that both relapsed tumors of different cell lineages are derived from a common malignant clone, presumably a committed lymphoid stem cell. A unique translocation, t(2;14)(q37;q11.2), which may involve TCR δ/α gene complex, was observed in the second relapsed tumor and AST-1 cells. To attempt to isolate the breakpoint of this translocation, the configuration of TCR δ/α gene complex was studied. The result showed that two rearrangements of TCR α gene detected with J_α probes were the products of the normal TCR rearrangement process, and were not involved in the translocation at this region. This patient, together with the AST-1 cell line, provided us a unique opportunity to study the development and clonal evolution of malignant lymphoma.     

Primary breast lymphoma.

Primary breast lymphoma (PBL) is a rare tumor of the breast. Three cases of PBL are being described. All three cases achieved remission following biopsy, chemotherapy, and/or radiotherapy. The literature is extensively reviewed with emphasis on true incidence of PBL and survival rates.     

API2对黏膜相关淋巴瘤凋亡及凋亡相关蛋白的影响

目的：了解黏膜相关淋巴瘤的凋亡水平及其所涉及到的信号传导通路。方法：收集33例胃肠黏膜相关淋巴样组织(MALT)淋巴瘤病例，通过TUNEL技术原位检测MALT淋巴瘤肿瘤细胞的凋亡水平，通过逆转录聚合酶链反应(RT-PCR)和免疫组织化学染色检测肿瘤的凋亡抑制蛋白2编码基因(API2)和Caspase3 mRNA及蛋白，对API2和Caspase3水平作半定量和定量分析。根据凋亡检测结果将全部病例分为两组，即凋亡细胞数每50个高倍视野小于或等于2的病例组和大于2的病例组，比较2组的API2和Caspase3 mRNA及蛋白。结果：半数以上的MALT淋巴瘤中存在凋亡抑制；凋亡抑制的MALT淋巴瘤病例中API2 mRNA及蛋白水平明显高于没有明显凋亡抑制的病例，但Caspase3 mRNA及蛋白水平在两组病例中表达没有明显的差异。结论：MALT淋巴瘤中存在明显的凋亡抑制，其发生与凋亡抑制因子API2的表达上调有关，但API2对凋亡的调节与Caspase3有关的信号传导通路无明显相关。     

Treatment options for primary splenic low‐grade non‐Hodgkin's lymphomas

The purpose of this comparative study was to evaluate the response of primary splenic low‐grade non‐Hodgkin's lymphomas (NHL) to chemotherapy, splenectomy, and chemotherapy combined with splenectomy in order to elaborate the optimum treatment modality. A total of 104 patients (age range: 15–82 years) with primary low‐grade B‐cell NHL of the spleen were comprised by our study. Stage IV disease was determined in 102 (98.1%) cases. Regarding the treatment modality, splenectomy was performed in 14 patients, early splenectomy and single‐agent chemotherapy in 15, early splenectomy and combined chemotherapy in 19, single‐agent chemotherapy in 23, and combined chemotherapy in 33. In the above‐mentioned order, complete remission rate was following: none, 40.0, 31.6, 21.8, and 18.2%. Partial remissions were achieved in 85.7, 46.7, 57.9, 30.4, and 69.7% of cases, respectively. The median remission duration turned out to be longer (74.5 months) in the group of patients with complete remissions attained by means of splenectomy and combined chemotherapy. Local relapses in the spleen developed in 19 (72.7%) patients treated with combined chemotherapy and in 9 (90.0%), who had undergone single‐agent chemotherapy. The 5‐year overall survival was 54.4% after splenectomy, 39.4% after single‐agent chemotherapy, and 37.1% after combined chemotherapy, being significantly higher (P < 0.05) after splenectomy and single‐agent chemotherapy (67.2%), and splenectomy followed by combined chemotherapy (64.7%). Early splenectomy combined with chemotherapy is the optimum treatment option for primary low‐grade NHL of the spleen because of the superiority in complete remission rate, remission duration, and in overall survival rate. Splenectomy leads to somatic compensation of patients, makes impossible local relapsing in the spleen, prevents continuous dissemination from the primary tumor site, and mostly corrects cytopenias, creating better conditions for chemotherapy.